pediatrics

In the NICU, mL/kg/day for continuous infusion =

(Rate per hour x 24)/weight = ml/kg/day

A 20-month-old, previously healthy girl is preparing to go on a 5-week trip to Lagos, Nigeria, as part of an organized church group trip. Her mother is concerned about their risk of acquiring poliomyelitis in Nigeria. The child was born in the United States and received inactivated polio vaccination at 2, 4, and 15 months of age. Her mother was also born in the United States and received her complete polio vaccination series as a child. The girl's physical examination findings are normal. Of the following, the MOST appropriate management to minimize the risk of infection of both the child and mother is to administer inactivated polio vaccine in

*1 dose to the mother and none to the child* - In the United States, a total of 4 doses of inactivated -polio vaccine at ages 2, 4, and 6 to 18 months and 4 to 6 years is currently recommended for all infants and children. - Before traveling to polio-endemic areas, travelers should ensure that they have received the recommended age-appropriate polio immunization series. - Adults who received a complete polio immunization series in childhood and intend to travel to and stay for more than 4 weeks in a polio-affected country should receive a single lifetime booster dose of IPV before departure.

Age? - chest up in prone position - holds hands together - reciprocal smiling: responds to adult voice with smiling - coos/social smile (vowel-like noises)

*2 mos* - head bobs when held in sitting position - hands unfisted 50% of time - retains rattle if placed in hand - opens mouth at site of breast or bottle - recognizes mother - follows large highly contrasting objects - alerts to voice/sound

You are discussing a clinical case with a group of medical students. The patient has a history of arthritis that started in the ankles, moved to the knees, and then shifted to the wrists. On physical examination, there is a fever (40°C) and a friction rub. Echocardiography reveals mitral valve prolapse and left ventricular dysfunction consistent with carditis. The laboratory results show an elevated erythrocyte sedimentation rate. The medical students ask how long before onset of symptoms does the infection associated with this diagnosis typically occur. Of the following, the MOST accurate response to the students' question is

*3 weeks* - Acute rheumatic fever presents approximately 3 weeks after a group A Streptococcus infection. - Diagnosis of acute rheumatic fever (ARF) is based on the Jones criteria, which require evidence of a group A streptococcal infection and either 2 major criteria or 1 major and 2 minor criteria. - Major Jones criteria for ARF are polyarthritis, carditis, Sydenham chorea, subcutaneous nodules, and erythema marginatum. - Minor Jones criteria for ARF are fever, arthralgia, prolonged PR interval on electrocardiograph, and elevated acute-phase reactants.

During a health supervision visit, a 14-year-old adolescent boy with known primary hypertension is found to have a blood pressure of 146/90 mm Hg. The cuff size was confirmed as appropriate. His body mass index is at the 85th percentile. His history and physical examination findings are otherwise unremarkable, and previous evaluations for end-organ damage have been negative. Bilateral radial and femoral pulses are normal and without delay. The adolescent has been participating in high school football and weight training without complaint. Of the following, the BEST recommendation for this adolescent regarding football participation is to

continue aerobic and low-intensity conditioning only - Children and adolescents with elevated blood pressure (previously known as prehypertension) or stage 1 hypertension can participate in unrestricted athletic activity as long as there is no left ventricular hypertrophy or other end-organ damage. - Children and adolescents with diagnosed hypertension should achieve blood pressure control below stage 2 levels before clearance to play a competitive sport.

After you introduce yourself during a routine health supervision visit, your male patient is able to clearly tell you his first and last name, but not his phone number. You offer him some crayons and paper while you visit with his parents. They don't report any health concerns. He likes to dress himself but needs some help with tying his shoes. He can bush his teeth fairly well and is toiler trained. He tells you he likes to play with blocks and can build a house. His mother adds that he will play simple board games that involve matching colors, but gets angered by games that involve a lot of rules, especially if others win. He is very inquisitive and asks a lot of "why" questions. He is a well-developed, nourished child who is able to hop on 1 foot 7 times, but is not able to skip. He tells you he is good at riding his tricycle and catching a ball. You note that he is able to copy a square, but not a triangle. This is most typical for what aged child?

*48 months* 4 years old... - clearly states first and last name - speaks in paragraphs using past and present tense - 100% intelligible to fam and strangers - identifies 5 or 6 colors - asks many "why" questions - dresses self but not yet able to tie shoelaces - brushes teeth and uses toilet independently - hops on 1 foot several times - builds house with blocks - copies cross and square - holds crayon well - cuts scissors on line

In the delivery room, a newborn is noted to be jaundiced with a diffuse purpuric rash and hepatosplenomegaly. The mother immigrated from Mexico four weeks prior to delivery. She saw a physician twice during pregnancy but had no ultrasounds. The infant's weight is 2500 grams and he is estimated to be full term based on Ballard scoring. Head ultrasound reveals periventricular calcifications and a complete blood count is notable for a platelet count of 50,000/mcL. Which of the following is the most likely diagnosis?

*Congenital CMV infection* - Approximately 10% of infants with congenital cytomegalovirus infection (CMV) have clinical findings at birth, which can include hepatosplenomegaly, hyperbilirubinemia, thrombocytopenia, purpura ("blueberry muffin" rash), microcephaly, intracranial periventricular calcifications, sensorineural hearing loss, and retinitis. - The intracranial calcifications in CMV infection are periventricular versus the diffuse cerebral calcifications seen in toxoplasmosis. - The treatment for symptomatic congenital CMV infection is oral valganciclovir, which has been shown to improve neurodevelopmental and audiologic outcomes at 2 years of age.

Which of the following is the most common cause of kidney failure requiring renal transplant in children?

*Congenital anomalies of the kidney and urinary tract* - Treatment with dialysis or kidney transplant should be considered for any child with chronic stage 4 kidney disease with glomerular filtration rate is < 30 mL/min per 1.73 m². - End-stage renal disease is defined as chronic kidney disease stage 5, or glomerular filtration rate < 15 mL/min per 1.73 m². The most common reason for transplant in children, accounting for about 30% of cases, is kidney failure resulting from congenital anomalies of the kidney and urinary tract. In children, kidney transplant is the treatment of choice, as mortality is much higher in children undergoing dialysis. Some children undergo preemptive transplantation, in which affected patients receive a transplant prior to attempting dialysis. - This happens most commonly when there is a living donor, usually a relative of the patient, available. Living donor transplant is associated with improved outcomes when compared to deceased donor transplant.

A 10-year-old girl is admitted post-small bowel resection as the result of Crohn disease. She has been doing well and is ready to start enteral feeding. Initiation of feedings typically begins with which method?

*Continuous feeding via nasogastric tube* - Children in whom enteral feeds are initiated typically receive continuous feeding via nasogastric tube or gastrostomy tube. This facilitates optimum absorption and continued intestinal adaptation.

A 9-year-old girl presents to clinic with her parents. They are concerned that over the past year she has been having more difficulty at school. They describe her as a good student that mostly gets As. This school year, she has been progressively fixated on doing every assignment perfectly. Several months ago, she had a report due and ended up not turning it in because it was not perfect to her, which caused her to get a zero on the assignment. Since then, she has had difficulty with most assignments and will often break down at home and cry while working on them. For the past several weeks, she has been resistant to going to school in the morning. Her teachers note that she has been more emotional lately but deny behavioral issues at school. She has generally been active outside of school and enjoys playing soccer, though her parents note that this too has become more of a chore, and she often complains of headaches or stomach aches, which come on just prior to practice or games. She admits to often feeling worried but cannot pinpoint a specific reason. She denies bullying at school. At home, she has become more irritable and argumentative over the past several months. They note that over the past year, she has had progressive difficulty falling asleep. She has no significant past medical history and takes no medications. She is quiet, avoids eye contact, and participates minimally in the visit. Which of the following is the most likely diagnosis?

*GAD* - Characterized by excessive anxiety and worry, which occurs most days for at least six months. - For children, one of the following should be present in order to make the diagnosis: restlessness, easy fatigue, difficulty concentrating, irritability, disturbed sleep, or muscle tension.

A 2-day-old, well-appearing boy presents to the emergency department with fussiness. He is found to have a serum calcium of 6.5 mg/dL, and ionized calcium of 0.9 mmoL/L, both confirmed on repeat measurements. He also has a harsh systolic murmur and absent thymus on chest X-ray suggestive of DiGeorge syndrome. Which of the following history or exam findings is consistent with this patient's hypocalcemia?

*Increased startle response* - The serum calcium not bound to albumin is physiologically active and is measured as ionized calcium. Low levels of ionized calcium indicates low serum calcium. This normally triggers parathyroid hormone secretion, leading to calcium mobilization from bone, increased reabsorption of calcium in the kidneys, and conversion of 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D, which promotes additional calcium and phosphorus absorption from both the gastrointestinal tract and kidneys. - Symptoms of hypocalcemia are usually neuromuscular and cardiac in nature and vary from asymptomatic, irritability, and increased startle responses to tetany and seizures. Hypocalcemia can also lead to a prolonged QT interval. Intravenous calcium is indicated in patients with a prolonged QT, tetany, seizures, carpopedal spasm, or a serum calcium < 7.5 mg/dL, regardless of symptoms.

A 16-year-old boy presents to the ED with fever. His fever began yesterday, and his illness quickly escalated to involve vomiting, headache, sore throat, and fatigue. Four days ago, he had surgery for a deviated septum. Vital signs show T of 40.2℃, HR 122 beats/min, RR 24 breaths/min, BP 84/56 mm Hg. On physical exam, he appears ill and confused. He has a diffuse red macular rash on his neck and chest, as well as hyperemia of his oral mucous membranes. What is the most likely pathophysiology of this patient's presentation?

*Local exotoxin release leading to T cell activation and cytokine production* - Local exotoxin release leading to T cell activation and cytokine production is the main mechanism by which damage is mediated in staphylococcal toxic shock syndrome. - Toxic shock syndrome should be suspected in cases of rapid onset of severe illness, usually over one to two days. - Clinical criteria include high fever (> 38.9℃), hypotension, diffuse macular erythroderma, and multisystem organ involvement. Desquamation of the skin will occur one to two weeks later. - Other scenarios in which toxic shock syndrome should be considered include surgical and postpartum wound infections, septorhinoplasty, osteomyelitis, and burns, especially when there is rapid onset of illness.

A 16-year-old girl presents with a change in mental status over the past several days. She says that the voice in the television and her bedroom are telling her to take her mother's medications. She has had an upper respiratory tract infection and mild headache recently but no head trauma or known ingestions. Her mother says she has not slept for the past two days. She has no previous psychiatric history, though her mother has anxiety and depression. When asked where she is, she says that she is in France trying to escape the Nazis. Her neurologic exam is normal. What is the next best step in managing this patient?

*Lumbar puncture for anti-N-Methyl-D-aspartate (NMDA) receptor antibodies* Medical conditions that may present with primary psychiatric symptoms include thyrotoxicosis, Wilson's disease, and anti-N-Methyl-D-aspartate (NMDA) receptor encephalitis. Patients with anti-NMDA receptor encephalitis often demonstrate primary psychiatric symptoms, including hallucinations, impaired memory, and sleep problems. They may have had a recent viral illness. The diagnosis of anti-NMDA receptor encephalitis requires a lumbar puncture for anti-NMDA receptor antibodies. The pathophysiology of anti-NMDA receptor encephalitis is complex but likely includes antibody-mediated synaptic dysfunction. Treatment includes medications such as corticosteroids, immune globulin, rituximab, or plasmapheresis. Prognosis is generally good, though there is a mortality rate (5.2% in one adult study).

Most commonly used illegal substance in the United States and worldwide

*Marijuana* - Derived from the cannabis plant, marijuana's primary active component is delta-9-tetrahydrocannabinol (THC). - Selective cannabis plant breeding has increased the THC content of marijuana, making marijuana more potent today than in the past. - Marijuana's street names include pot, grass, dope, ganja, MJ, and hemp. - Marijuana's effects occur within seconds of smoking and 30 to 90 min after ingestion. - The peak is 15 to 30 min after smoking and 2 to 3 hours after ingestion. - Effects last up to 4 hours after smoking and up to 12 hours after ingestion.

A 3-year-old boy is referred to the hospital for lymphadenitis. He has had an enlarged but nontender submandibular lymph node for the past two months. He completed full courses of amoxicillin, clindamycin, and azithromycin. The node has continued to slowly enlarge but remains nontender. The overlying skin appears purple and thinned. Which of the following is most likely to reveal the reason for treatment failure?

*Mycobacterial culture of the lymph node* - The child's findings are typical of nontuberculous mycobacterial lymphadenitis. - Lymphadenitis is the most common manifestation of nontuberculous mycobacteria in children. It most commonly affects immunocompetent children between the ages of 1 and 5 years. Nontuberculous mycobacteria classically cause a painless, gradually enlarging unilateral lymph node. The most commonly affected nodes are those of the submandibular region. - The overlying skin has a characteristic purple hue, and it eventually begins to thin to a tissue or parchment paper consistency. The child may also develop draining sinus tracts from the lymph node to the skin. Fever and other signs of systemic infection are typically absent. The diagnostic test and therapy of choice for nontuberculous mycobacterial lymphadenitis is an excisional biopsy. The biopsy specimen should be sent for an acid-fast stain, though a negative stain does not preclude the possibility of growth of nontuberculous mycobacterial on culture. The biopsy specimen can be sent for histopathology, mycobacterial polymerase chain reaction, and mycobacterial culture of the lymph node.

In prepubertal children and those who are not sexually active, what are the most common causes of epididymitis?

*Mycoplasma and viruses* - In sexually active males, the most common etiologies of epididymitis are Chlamydia and Neisseria gonorrheae, followed by Escherichia coli and viruses.

An obese Hispanic female presents with fatigue, polyuria, polyphagia, and polydipsia. Her vital signs are normal. Her BMI is 37 kg/m2. The exam is normal except for morbid obesity. Fasting blood sugars are 135 mg/dL and 137 mg/dL on separate days. Which study would determine if the patient has type 1 or type 2 diabetes mellitus?

*No test is needed* - The patient has type 2 diabetes mellitus as evidenced by history and physical exam. - No further study is needed. - A C-peptide level can be done if it would have an affect on the patient's therapy. - In type 1 diabetes, the C-peptide is low. In type 2 it should be normal, but a glucose load will cause an increase.

A 17-year-old boy presents to the emergency department with dizziness, nausea, and headache after falling off a trampoline this evening. He thinks he lost consciousness for a few seconds, but otherwise was awake shortly after the incident. He vomited once after the fall but has not since then. There are no focal findings on neurologic examination, and he is ambulatory in the emergency department. What are the most appropriate disposition recommendations to give this patient?

*Outpatient cognitive and physical rest for 24 hours, with gradual return to physical activity* - Concussion typically results in short-lived impairment of neurologic function after a direct blow to the head or face that spontaneously resolves. Clinical signs and symptoms of a concussion reflect a functional disturbance rather than structural injury to the brain. - No abnormalities are seen on neuroimaging studies. The incidence of concussion is highest among boys playing collision sports, such as rugby, football, hockey, and lacrosse. - Children with a concussion may manifest headaches, confusion or disorientation, difficulties with memory, inattentiveness, slow speech, dizziness, gait abnormalities, vomiting, and emotional lability. Patients may or may not have experienced loss of consciousness. - Management of concussion includes removal from competitive sports, restriction of physical activity, and neurocognitive rest. Recent observational studies have suggested that strict physical rest for more than a few days after a concussion may prolong symptoms. Therefore, the current recommendation is brief cognitive rest for 24 to 48 hours after concussion, followed by a gradual return to noncontact physical activity. - Patients with worsening or persistent symptoms for greater than 21 days should be referred for specialty evaluation.

An 18-year-old patient of yours presents for his sports clearance physical. He is excited to report that he will be playing on his college's varsity wrestling team this upcoming academic year. As part of your evaluation, you screen him for use of performance-enhancing drugs. What is the mechanism of action(s) of anabolic-androgenic steroids as performance-enhancing drugs?

*Promotion of anabolic growth of muscle and prevention of catabolic breakdown of muscle* - These substances are synthetic hormones that are like testosterone and exert androgenic effects, despite being designed to have a greater anabolic to androgenic effect. They include oral 17-alpha-alkylated androgens and parenteral 19-nortestosterone derivatives. - They act via multiple mechanisms, including increasing nitrogen concentration in muscle to promote anabolic growth of muscle. In addition, they prevent muscle breakdown by inhibiting the binding of catabolic glucocorticoids to muscle. Thus, they both promote anabolic growth of muscle and prevent catabolic breakdown of muscle. - As anabolic-androgenic steroids also have an androgenic effect, they are thought to increase levels of aggression, thereby causing athletes to push themselves harder in training. - There are also several adverse effects of anabolic-androgenic steroids including, but not limited to, dyslipidemia (especially with oral use of 17-alpha-alkylated androgen), reproductive changes in both men and women, gynecomastia, infection, and hepatic side effects.

A 3-year-old boy is brought to the office by his mother for a health supervision visit. He has no significant medical history. She reports that he has been having trouble sleeping for the past 2 months. It has been difficult to get him to bed at night; he does not want her to leave his room and repeatedly tells her that he needs 1 more story or another good night kiss. He has also been waking up with nightmares. In the office, the boy stays close to his mother instead of exploring the examination room. His mother reports that his father moved out of their home 2 months ago. She asks what she should do to improve his bedtime behavior pattern. Of the following, the BEST recommendation is for this mother to

continue to keep the same bedtime routine through this adjustment period - Children are exposed to many changes when their parents separate and they can have difficulty adapting. It is helpful to keep schedules and routines as constant as possible during this time of adjustment. For this young boy, maintaining his preseparation bedtime routine will help him return to his previous stable sleep pattern. Allowing him to sleep in his mother's bed or having his mother stay with him until he falls asleep will produce a sleep association that will need to be addressed in the future. Varying the bedtime and bedtime routine will prolong the time to normalization of his behaviors. Outcomes for children of divorce are worse in children who have inconsistent schedules.

Which one of the following best describes the goal of "bystander" training programs?

*Promotion of self-efficacy to intervene in situations involving potential or actual victimization of others* - Education to recognize and the promotion of self-efficacy to intervene in situations involving potential or actual victimization of others are the main goals of "bystander" training programs. These programs had primarily targeted sexual victimization on college campuses but have now expanded to other populations, including high school students and members of the military, and to other types of victimization such as bullying. - Examples of programs include "Green Dot," "Bringing in the Bystander," and "Coaching Boys into Men." - Common components of bystander-focused prevention include heightening awareness of what constitutes interpersonal violence, instilling a sense of responsibility to intervene, disseminating education and training so that the real and perceived norm is that peers and authorities will support intervention by the bystander, self-safety considerations, and building skills and confidence to intervene. Examples of bystander interventions include distracting would-be perpetrators, taking direct action (e.g., physically or verbally intervening), and delegating actions to others (e.g., calling 911, escorting an intoxicated person home safely). - Although research is ongoing, studies have demonstrated promising reductions in sexual victimization, sexual harassment, stalking, and psychological dating violence among college students and male athletes. A particularly substantial reduction in alcohol- and drug-facilitated sexual violence has been observed in both high school students and college freshman.

A 17-year-old female is seen in the emergency department with a third-degree burn on her foot that occurred 8 days ago. She now has an eschar covering most of the wound. She is febrile and has an elevated white blood cell count. What is the next step in the management?

*Remove burned skin and apply sodium mafenide* - Silver sulfadiazine remains popular for the treatment of burn wounds. The agent is water-soluble and makes changing of dressings easier. However, it does induce allergies. - Mafenide is an alternate agent and is excellent for eschar-containing tissues. It is the treatment of choice for more severe burns, but it can cause metabolic acidosis. - Acticoat has antimicrobial properties because of silver and can be maintained in the wound for up to 4 or 5 days. Because of infrequent wound changes, most patients prefer this agent. - Despite topical antibiotic use, fungal infections of burn wounds continue to increase. Fungal infections are associated with large burns and often cause sepsis. Fungal infections are most common in the elderly and the young.

A 12-year-old girl presents to the emergency department (ED) with chest pain, shortness of breath, and inability to sleep for 24 hours. She cannot lie flat on the stretcher as her chest pain becomes unbearable. She states that these symptoms have been present intermittently for approximately 2 months, but have worsened over the last 48 hours. One month ago, she had several days of right knee and left wrist swelling. She had been able to manage her pain with ibuprofen. On physical examination, the girl's temperature is 37.5°C, heart rate is 130 beats/minute, respiratory rate is 28 breaths/minute, blood pressure is 120/70 mm Hg, and oxygen saturation is 98% in room air. Her breath sounds are diminished at the left base. Her cardiac examination is significant for muffled heart sounds and a soft friction rub, with no murmur or gallop. Her liver is palpable 1 cm below the right costal margin. The remainder of her examination is unremarkable. On chest radiography, there is an enlarged cardiac shadow, which obscures the left lung base. An echocardiogram shows a large pericardial effusion. Results of laboratory studies are shown: Laboratory Test Patient Result White blood cells 3,000/mL (3 x 109/L) Hemoglobin 9 g/dL (90 g/L) Hematocrit 27% Platelets 390 x 103/mL (390 x 109/L) Sodium 141 mEq/L (141 mmol/L) Potassium 4.2 mEq/L (4.2 mmol/L) Chloride 104 mEq/L (104 mmol/L) Bicarbonate 23 mEq/L (23 mmol/L) Blood urea nitrogen 22 mg/dL (7.8 mmol/L) Creatinine 1.4 mg/dL (124 mmol/L) Erythrocyte sedimentation rate 85 mm/h The only remarkable finding on urine dipstick test was 3+ protein. Of the following, the MOST likely cause of this girl's pericardial effusion is

*SLE* The clinical picture for the girl in the vignette is consistent with systemic lupus erythematosus (SLE). She presents with symptoms of positional chest pain and arthritis, which improved with ibuprofen. On physical examination, she is afebrile and has signs of a left pulmonary process, as well as a friction rub, suggesting a pericardial effusion that is confirmed on echocardiography. Her laboratory tests show protein in her urine, an elevated creatinine, and evidence of an inflammatory process as well as chronic disease, such as would be seen in SLE. The first presentation for SLE may be with a pericardial effusion. Nonsteroidal anti-inflammatory medication, such as ibuprofen or aspirin, is the usual initial medical intervention. Patients with hemodynamically significant effusions will require pericardiocentesis.

Phrynoderma, reddish-brown papules usually found around the elbows and knees, are associated with which specific vitamin deficiency?

*Vitamine A* - This condition is generally found in early childhood and is a result of malnutrition. - Extreme xerosis is another cutaneous finding in avitaminosis A. - Avitaminosis A is the most common cause of childhood blindness. - Phrynoderma commonly is associated with deficiency of vitamin A, B, and E. It is also associated with essential fatty acid deficiency. Phrynoderma is asymptomatic or mildly symptomatic follicular hyperkeratosis, where follicular papules with central keratotic plugs that block the follicle openings. New patches may be hypopigmented. Patches arise on the back of the elbows and the front of the knees and can spread to the upper forearms, and thighs. Occasionally patches appear on the abdomen, back, and buttocks. The face is rarely affected, and hands and feet are spared.

A 2-year-old African American boy is brought to the emergency department by his parents because of excessive fatigue and "funny breathing." He had been well until 2 days ago when he was seen by his pediatrician for cellulitis on his thumb, for which he was prescribed trimethoprim-sulfamethoxazole. He received the first dose just over 24 hours ago. He has not been taking any other medications, vitamins, or supplements, and he has had normal growth and development. He is at the 55th percentile for height and the 60th percentile for weight. He has a heart rate of 122 beats/min, blood pressure of 100/75 mm Hg, temperature of 37°C, respiratory rate of 30 breaths/min, and oxygen saturation of 99% on room air. He appears pale and fatigued, but his examination findings are otherwise unremarkable. Laboratory data are shown: Laboratory Test Result White blood cell count 6,700 /µL (6.7 × 109/L) Hemoglobin 5.1 g/dL (51 g/L) Platelet count 640 × 103 /µL (640 × 109/L) Reticulocytes 37% A peripheral blood smear is remarkable for the presence of "blister" cells and polychromatic macrocytes. Of the following, this child's disease is MOST likely caused by an

*X-linked red cell enzymopathy* - Glucose-6-phosphate dehydrogenase deficiency is an X-linked condition resulting in an ineffective response to intracellular oxidative stress. Erythrocytes are particularly sensitive and undergo hemolysis. - Although there are many variants of glucose-6-phosphate dehydrogenase deficiency, 2 variants are the most prevalent in the United States, with the A- variant affecting 10% of African American males. - Patients with glucose-6-phosphate deficiency should avoid specific medications and foods that induce increased oxidative stress.

What is the test of choice for diagnosis of hepatopulmoanry syndrome?

contrast echocardiography - contrast echocardiography with agitated saline is the test of choice for diagnosing hepatopulmonary syndrome. Typically, the bubbles produced by agitation should be lodged in the pulmonary capillaries. However, due to dilatation of these capillaries (leading to relative intrapulmonary shunt and hypoxemia), the bubbles are able to pass through and can be visualized in the left side of the heart within 3-6 cardiac cycles

Vent initiates the breath

control breath

A 20-month-old boy is brought to the emergency department because of difficulty breathing. He was in his state of usual health until 3 days ago, when he developed a fever (up to 39.4°C). Since then, other symptoms have developed, including decreased oral intake, irritability, and vomiting. He has had 1 wet diaper in the last 24 hours. His mother brought him in because of progressive lethargy, cough, and difficulty breathing. Vital signs show a temperature of 38.5°C, heart rate of 190 beats/min, respiratory rate of 50 breaths/min, blood pressure of 85/65 mm Hg, and SpO2 is 90% on room air. Physical examination reveals a generally lethargic child in moderate respiratory distress. Conjunctivae and oral mucous membranes are erythematous and dry. There is no cervical lymphadenopathy. He is tachypneic with use of accessory muscles and grunting. There is wheezing and scattered rales on auscultation. Cardiovascular examination reveals tachycardia, normal S1 and S2 with no rubs, gallops, or murmurs. His extremities are pale and cool, with capillary refill time approximately 4 seconds. Abdomen is soft with palpable spleen and liver edge 2 cm below the costal margin. He receives a 20 mL/kg intravenous fluid bolus of 0.9% saline. Subsequently, his respiratory distress worsens and his pallor increases. Repeat physical examination reveals development of jugular venous distention, and a palpable liver edge of now 4 cm below the costal margin. Of the following, the MOST likely diagnosis is

*cardiogenic shock* - The child in this vignette is in shock, as evidenced by lethargy, respiratory distress, tachycardia, and decreased urine output, all of which indicate decreased organ perfusion. The type of shock is likely cardiogenic, as demonstrated by tachycardia, poor skin perfusion, hepatosplenomegaly, rales, jugular venous distention, and worsening condition upon fluid challenge. - Shock is a condition in which oxygen delivery is insufficient to meet the metabolic demands of vital organs. It is a life-threatening state that must be distinguished from other less serious conditions so that treatment can be initiated without delay. Oxygen delivery is the product of cardiac output and oxygen content. It can also be represented by the following formula: - Oxygen delivery = Stroke volume × Heart rate × Oxygen content - As suggested by this equation, an early compensatory mechanism to increase oxygen delivery in shock, especially in children, is tachycardia. Elevated circulating catecholamines serve to increase stroke volume, heart rate, and blood pressure, as well as preferential diversion of cardiac output away from skin, renal, and splanchnic vascular beds toward the heart and brain. The resultant increased systemic vascular resistance leads to maintained, or even increased, blood pressure, resulting in compensated shock. The clinician should not be reassured by a normal or elevated blood pressure when considering shock. Despite representing a compensatory mechanism, catecholamines and increased systemic vascular resistance can be especially deleterious in cardiogenic shock because of increased afterload, diastolic dysfunction, increased myocardial oxygen demands, and impaired coronary artery filling. - Cardiogenic shock further characterizes the insufficiency of oxygen delivery as a failure of the heart as a pump. This can be caused by impaired contractility, arrhythmias, outflow tract obstruction, or impaired filling as seen in diastolic dysfunction, restrictive cardiomyopathy, lesions of reduced cavity size, or tamponade physiology. Impaired contractility is most commonly seen in myocarditis, primary neuromuscular disorders, or metabolic derangements. Single ventricle congenital heart disease with ductal-dependent systemic blood flow, such as hypoplastic left heart syndrome, critical aortic stenosis, and interrupted aortic arch can cause shock if right-to-left shunting across the ductus arteriosus is impaired. Ductal-dependent pulmonary blood flow lesions, such as tricuspid atresia or pulmonary atresia can cause severe cyanosis if left-to-right shunting across the ductus arteriosus is impaired. - Heart failure can masquerade in children as pneumonia, asthma, or bronchiolitis because clinical signs also include wheezing, grunting, and difficulty breathing. In heart failure, increased left atrial pressure transmits upstream to the pulmonary circulation, and the resulting increased pulmonary capillary hydrostatic pressure in turn leads to pulmonary edema. Increased lung water and decreased pulmonary compliance can cause tachypnea and grunting, and compression of neighboring bronchioles can lead to so-called "cardiac wheezing." It is therefore imperative to recognize shock in this patient. This is especially true because treatment with bronchodilators can worsen heart failure by causing tachycardia, diastolic hypotension, lactic acidosis, and increased myocardial oxygen demand. In this patient, hypovolemic shock is less likely in light of the rales, hepatomegaly, and worsened condition after receiving fluids. - Patients with cardiogenic shock may also be dehydrated because of general illness and increased insensible losses from tachypnea. Therefore, very cautious use of fluid boluses can help such a preload-dependent patient experiencing heart failure. However, boluses should be limited to 5 mL/kg at a time, followed by careful reassessment. If preload is adequate and cardiac output is still impaired, a cardiology consultation should be sought, and inotropes should be started

A 17-year-old female soccer player is brought to the emergency department (ED) following a head injury that occurred while she was playing in a soccer game. Approximately 20 minutes ago, she collided head-to-head with another player while she was running and fell to the ground. Her mother, who accompanied her daughter to the ED, tells you that she had a very brief loss of consciousness (less than 15 seconds) immediately following the injury and that she has seemed "dazed" since it occurred. She vomited once about 10 minutes ago.A review of the patient's medical history indicates that she has no significant past medical or surgical history, takes no medications, has no allergies, and has had no prior head injuries. In the ED, the patient is sleepy, but answers all questions appropriately and follows instructions. When you ask her about her current symptoms, she states: "My head still hurts, but it is starting to feel a little better now." Her vital signs are within normal limits. On physical examination, her pupils are equal in size and reactivity. She has a 3 x 4 cm area of ecchymosis near the center of her forehead, but no hematomas or step-offs on palpation of her entire forehead and scalp. There are no focal deficits on a complete neurologic examination, although she tells you that she feels tired and wants to lie down again after you ask her to walk back and forth across the room. The remainder of the physical examination is unremarkable. Of the following, the MOST appropriate next step in the management of this patient is to

*continue to observe her in the emergency department* - While computed tomography (CT) of the brain is a rapid and accurate way to identify intracranial injuries in children after head trauma, use of this diagnostic modality has downsides, including exposure to ionizing radiation, identification of minor lesions or incidental findings with unclear clinical importance, the need for sedation for younger or uncooperative pediatric patients, and significant increases in healthcare costs - The goal of pediatric providers should be to identify children with clinically important intracranial injury after head trauma to prevent deterioration and secondary brain injury, while limiting unneeded radiographic imaging in children at low risk. - For patients falling into the "intermediate risk" category for intracranial injury, observing patients clinically before obtaining computed tomography of the brain allows providers to selectively image only those whose symptoms worsen or fail to improve.

A 2-week-old female neonate is brought to your office for an urgent visit because bright red blood was noted in her stool this morning. She was born at term by spontaneous vaginal delivery after an uncomplicated prenatal course. She was breastfed and began receiving standard infant formula supplementation because of poor weight gain in the first week after birth. Her parents report that she has had increasing fussiness over the past week. They deny fever or rash. Of the following, the MOST likely cause for this neonate's presenting symptoms is

*cow milk protein intolerance* - Cow milk protein intolerance is the most likely cause of blood in stools and fussiness in this 2-week-old neonate. Cow milk protein intolerance is a reaction to cow milk protein found in either breast milk or cow milk-based formulas. Five percent to 15 percent of infants are affected by an allergy or intolerance to cow milk. This may be immunoglobulin E (IgE)- or non-IgE-mediated. Symptoms commonly occur in the first 6 months after birth. - Gastrointestinal signs and symptoms (diarrhea, vomiting, and/or guaic-positive stools) are the most common presentation, ranging from a frequency of 50% to 80%. Cutaneous symptoms are seen in 20% to 40%, and respiratory symptoms in 4% to 25% of affected infants. While fussiness is common, weight loss and failure to thrive are uncommon. - Sixty percent of affected infants are exclusively breastfed. Most infants with cow milk protein intolerance experience resolution between 6 months (50%) and 2 years of age (80%-90%). It is rare for the blood loss to result in anemia. In breastfed infants, a maternal elimination diet is often effective. Formula-fed infants should make a transition to extensively hydrolyzed formula (eHF) or an amino-acid formula for those with severe or life-threatening symptoms, such as respiratory distress. Up to 50% of infants with cow milk intolerance will also have soy protein intolerance before age 6 months, and will require a hydrolyzed or amino acid formula. Therefore, a trial of soy formula should be delayed until after 6 months of age if there are financial limitations. - Evaluation should include a detailed history and physical examination and a stool occult blood test to confirm the presence of blood. A maternal elimination diet or food challenge with a non-cow milk diet should be attempted for 2 to 4 weeks. A complete blood cell count should be obtained if blood loss appears to be significant.

An 18-month-old previously healthy boy presents to the emergency department with 3 days of fever, poor feeding, vomiting, and foul-smelling diarrhea. He has been vomiting about 15 times per day and has had about 10 watery stools per day. There are several other children in his daycare that have similar symptoms. His mother says he has not been able to keep down sips of water or juice without vomiting. He has been increasingly lethargic and has developed difficulty breathing in the last 12 hours. Vital signs show a temperature of 38.2°C, heart rate of 160 beats/min, respiratory rate of 32 breaths/min, blood pressure of 80/60 mm Hg, and oxygen saturation of 100% on room air. On physical examination, the child is awake but minimally interactive. Mucous membranes are dry, eyes are sunken, and extremities are cool with capillary refill time of 4 seconds. He is breathing comfortably and his lungs are clear to auscultation and equal bilaterally. Heart is hyperdynamic, with no rubs, murmurs, or gallops. Of the following physiologic derangements, the MOST likely to be present in this child is

*decreased intravascular volume* The boy in this vignette has hypovolemic shock from gastroenteritis and dehydration. He is tachycardic and has a capillary refill time of 4 seconds. Of the choices listed, the most likely physiologic derangement is decreased intravascular volume. Shock is a life-threatening condition characterized by inadequate oxygen or substrate delivery to meet metabolic demands of end organs. The most common form of shock worldwide is hypovolemic shock because of the high incidence of life-threatening diarrheal illness. Other causes of hypovolemic shock include bleeding, burn injury, and excessive diuretic use. Oxygen delivery is equal to the product of cardiac output and oxygen content. Cardiac output is the product of stroke volume and heart rate. Stroke volume is dependent on preload, afterload, and contractility. Preload is the volume of blood filling the ventricles at the end of diastole. In hypovolemic shock, stroke volume is low because of inadequate preload from decreased intravascular volume. In compensated hypovolemic shock, elevated circulating catecholamines cause tachycardia, increased inotropy, and arteriolar vasoconstriction. Signs of hypovolemic shock include delayed capillary refill time, cool skin, dry mucous membranes, skin tenting, and diminished peripheral pulses. Capillary refill time is tested by compressing a capillary bed briefly so that the skin blanches and recording the time required for the skin to become pink. It is a simple, objective, noninvasive clinical indicator. Determinants of capillary refill time include stroke volume and arteriolar capacitance. Accordingly, cardiogenic shock with decreased stroke volume and other conditions associated with systemic vasoconstriction, such as some instances of sepsis, toxic ingestions, and cool ambient temperatures, can also cause delayed capillary refill time. The American Heart Association's Pediatric Advanced Life Support curriculum recommends using capillary refill time as an early marker of compensated shock and that interventions such as aggressive fluid resuscitation can restore perfusion and reverse shock. Decreased capillary refill time, or "flash" (rapid) capillary refill, is seen in conditions of vasodilation, such as "warm" sepsis and some toxic ingestions. A patient can have shock with either normal or decreased capillary refill time. Indeed, conditions causing vasodilation may be associated with pooling of blood in the arterioles and venules, causing low preload. Also, diastolic hypotension may occur in these conditions because blood continues to "run off" into dilated arteriolar beds during diastole. Since ambient temperatures may also affect capillary refill time, the entire clinical picture should be considered before making a judgment of intravascular volume.

An otherwise healthy 14-year-old boy complains of occasional discomfort and heaviness in the left side of his scrotum. He denies sexual activity and any history of trauma and has had no dysuria or discharge. He is afebrile. On physical examination, he is at sexual maturity rating 4. Upon inspection, there is no sign of inflammation and no discoloration. Of the following, the BEST next step in evaluation of this patient is to

*examine him in the upright position with and without valsalva maneuver* - While most are asymptomatic, 10% of boys with a varicocele will present with heaviness or discomfort in the scrotum as described by the boy in the vignette. - It can decompress when the patient is in the supine position, therefore it is important to examine the patient standing, especially noting whether the mass enlarges with the Valsalva maneuver. - A grade 1 varicocele is palpable only with Valsalva maneuver; a grade 2 is not visible but is palpable without Valsalva; and a grade 3 is found on visual inspection. - Typically, varicoceles occur on the left side, with bilateral involvement occurring in only 2% of cases and unilateral right side involvement even less frequent. - In advanced cases the involved testicle may be smaller than the contralateral side, although this is not usually apparent on physical examination. - The higher the varicocele grade, the greater the risk for poor outcome, notably decreased fertility. - The diagnosis of varicocele is made clinically, and additional studies are rarely needed for confirmation. However, testing may be indicated to evaluate complications or to rule out other diagnoses. Ultrasonography with Doppler flow is used most often to evaluate for testicular torsion. - The physical findings of that condition (acute pain, scrotal swelling, marked testicular tenderness, absent cremasteric reflex) readily differentiate it from a varicocele. - The treatment goal for varicoceles is to preserve fertility, although how best to do this is controversial. To date, it is unknown if aggressive surgical treatment during adolescence preserves fertility.

You have diagnosed pelvic inflammatory disease (PID) due to Neisseria gonorrhoeae in a 16-year-old girl. Which of the following is most likely to reduce the chance of reinfection after appropriate treatment?

*expedited partner therapy* - refers to the treatment of sexual partners of patients diagnosed with chlamydia or gonorrhea by providing the patient with medication for his or her partner(s) without requiring a visit to a health care provider. - to facilitate immediate partner treatment and to improve compliance. - The risk of reinfection increases significantly when sexual partners do not receive adequate treatment. - legal in the majority of states and should be offered to all patients (men and women) diagnosed with chlamydia and gonorrhea whenever possible.

The infant in this vignette has steatorrhea (suggested by frequent greasy, malodorous stools in the context of severe diaper dermatitis), malnutrition (failure to thrive, hypoalbuminemia with ascites), and hepatomegaly consistent with a diagnosis of

cystic fibrosis-associated liver disease - best test to establish the diagnosis of cystic fibrosis is a sweat chloride test - in neonates, a liver edge greater than 3.5 cm below the right costal margin is considered abnormal

A six-year-old boy is brought to the clinic by his mother due to abdominal pain. For the past month, he would complain of abdominal pain, bloating, and occasional diarrhea. He would describe his pain as cramping that is usually in the periumbilical area. He would occasionally have watery stools without blood. His diet includes meat, vegetables, and three glasses of milk per day. Physical examination is positive for borborygmi. Which of the following is the most likely diagnosis?

*lactose intolerance* - Physical examination may reveal borborygmi. - Patients with lactose intolerance have a stool osmotic gap greater than 125 mOsm/kg due to the presence of unabsorbed carbohydrates within the intestinal lumen. Also, patients have stool pH less than 6 due to bacterial fermentation of lactose in the colon. - A presumptive diagnosis of lactose intolerance can be made in patients with mild symptoms that occur with significant lactose ingestion (more than two servings of dairy/day or more than one serving in a single dose that is not associated with a meal) and resolve after five to seven days of avoidance of lactose-containing foods. The diagnosis of lactose intolerance can be established by the use of lactose hydrogen breath test.

A 6-year-old previously healthy, fully immunized boy is brought to your office by his mother. He has had a cough and runny nose for 1 week and developed fever yesterday. Today, the boy is complaining of neck pain, resists turning his head to the side, is refusing to eat, and will only take small sips of water. On physical examination, the boy is tired appearing with a temperature of 40.3°C, heart rate of 130 beats/min, respiratory rate of 45 breaths/min, and oxygen saturation of 98% by pulse oximetry. His lung fields are clear with good aeration. He has tender anterior cervical lymphadenopathy, torticollis, and his posterior oropharynx appears erythematous. The remainder of his physical examination is within normal limits. Of the following, the test MOST likely to confirm this boy's diagnosis is

*lateral neck radiograph* - The boy in the vignette has a retropharyngeal abscess that could be visualized on lateral neck radiography as abnormally thickened prevertebral soft tissue. - Contrast-enhanced computed tomography is sometimes necessary to differentiate between a retropharyngeal abscess and retropharyngeal cellulitis. - Retropharyngeal abscesses occur most commonly in younger children, typically through lymphatic spread. - Initial symptoms are nonspecific and insidious. Children may have a preceding upper respiratory infection, followed by fever, sore throat, and decreased oral intake. They may develop neck stiffness or pain, and as symptoms progress, tachypnea, drooling, or stridor. - Physical examination may reveal enlarged and/or tender cervical lymph nodes. Laboratory evaluation usually shows an increased white blood cell count and signs of inflammation, but blood cultures are unlikely to reveal a causative organism. - Medical management with empiric antibiotics is effective in up to 25% of patients; refractory cases require surgical management.

A 14-month-old girl is brought to the emergency department in February with trouble breathing. She was in good health until 3 days ago when she developed low-grade fever and malaise. Yesterday, she had high fever, runny nose, mild cough, and a significant decrease in activity. The fever has persisted, and today she appears to have difficulty breathing. She has a temperature of 38.6°C, heart rate of 171 beats/min, respiratory rate of 50 breaths/min, blood pressure of 119/68 mm Hg, and oxygen saturation of 87% on room air. She is distressed and has nasal flaring and retractions. Bilateral rhonchi are heard on lung examination. Results of a rapid influenza test and a rapid respiratory syncytial virus test are negative. A chest radiograph shows increased perihilar markings with no focal consolidation. Of the following, the BEST next step in management of this patient is to test for influenza with a

*molecular assay and start oseltamivir before result is available* The sensitivity of molecular assays is quite high, ranging from 86% to 100%. In comparison, the sensitivity of rapid diagnostic assays ranges from 10% to 70%. Thus, as suggested in this vignette, a negative rapid influenza test result does not exclude influenza infection. Repeating the rapid test would not be of value. Although influenza can be cultured, culture results are often not available for 3 to 10 days. However, results from a molecular assay can be available in hours. The child in this vignette warrants treatment because she is younger than 2 years, which puts her at risk for developing complications from influenza infection, and because of the severity of her illness. Treatment for influenza infection should not be deferred until confirmatory test results are available because antiviral therapies are most efficacious when given within 48 hours of symptom onset. However, in individuals with severe or progressive influenza disease, antiviral therapy is recommended even when the duration of illness has been longer than 48 hours.

A 4-year-old girl presents for evaluation of bumps on the arm for several weeks. There have been no associated symptoms and the girl is otherwise well. Her physical examination is remarkable only for several papules located in the left antecubital fossa. Of the following, the MOST likely diagnosis is

*molluscum contagiosum* - The girl described in the vignette has several "translucent" papules (ie, that mimic vesicles) located in the left antecubital fossa, some of which have a central umbilication (depression or dell) - Molluscum contagiosum (MC) is a common poxvirus infection that is spread by direct contact including sexual contact, autoinoculation, or fomites. It is especially prevalent in children who have atopic dermatitis, with a disrupted skin barrier and impaired cutaneous immunity. - Papules vary in number and range in size from 1 to 6 mm in diameter, and can affect most body surfaces, although involvement of the palms, soles, and mucous membranes is rare. - Early lesions often are small and lack umbilication. Dermatitis surrounding the lesions of MC is common. - Individual lesions of MC may resolve in 1 to 2 months, but often are replaced by new papules. As a result, the condition may persist for months to years (average 13 months). - Especially in young children who do not tolerate discomfort well, cantharidin (a blister beetle extract) may be applied in the office to individual lesions. - For children who can tolerate discomfort, cryotherapy and curettage are effective.

You are seeing a 12-year-old boy in your office with a complaint of right knee pain and swelling of 4 days' duration. He has pain with movement, making it difficult to walk. He has no fever, rash, conjunctivitis, or diarrhea. There is no history of travel, injury, or tick exposure. The boy was seen 2 weeks ago for fever and cough, at which time a throat culture was negative and he was treated for suspected community-acquired pneumonia. He was then well until the knee swelling occurred. On physical examination, you find an afebrile, well-appearing boy with swelling, erythema, and decreased range of motion of the right knee. He winces when you passively flex his knee. The remainder of the physical examination is unremarkable. The boy was seen in the emergency department 2 days earlier, where an evaluation for septic arthritis was negative. Laboratory results from the emergency department show: White blood cells, 11,300/µL (11.3 x 109/L) Red blood cells, 4.5 x 106/µL (4.5 x 1012/L) Hemoglobin, 11.2 g/dL (112 g/L) Hematocrit, 37.1% Platelet count, 300 x 103/µL (300 x 109/L) Erythrocyte sedimentation rate, 25 mm/h C-reactive protein, 2.0 mg/L Synovial fluid analysis: Yellow and opaque White blood cell count, 20,000 cells 50% polymorphonuclear leukocytes Low viscosity Gram-stain negative Culture no growth for 48 hours Of the following, the BEST next step in management of this boy's symptoms is

*naproxen* - The boy in the vignette is well appearing and afebrile, with normal blood counts. His inflammatory markers are only slightly elevated and the joint fluid does not appear to be infectious. The child most likely has reactive arthritis. Naproxen is a nonsteroidal anti-inflammatory drug that is recommended as the first line for treatment of reactive arthritis. The drug, with both pain relieving and mild anti-inflammatory properties, would be the best initial choice for this patient. With no history of sexual activity and a physical examination and laboratory studies not consistent with a pyogenic arthritis, no antibiotic therapy is needed. Prednisone is not recommended in this case as this treatment can mask the symptoms of chronic arthritis.

A four-year-old boy presents to the Emergency Department unresponsive following an unknown ingestion. His arterial blood gas results include: pH 7.20, CO2 20 mm Hg, and HCO3 12 mEq/L. Which of the following is likely the cause of his symptoms?

*oil of wintergreen* - highly potent and concentrated form of methyl salicylate (5 ml of oil contains 9 grams of aspirin) - deadly to toddler salicylate poisoning - oil of green contains methyl salicylates and acetylsalicylates --> rapidly converted to salicylate once ingested - causes mixed acid base disturbance via early respiratory alkalosis via stim of resp center in brain and elevated anion gap met acidosis - signs and symptoms of acute toxicity begin to develop above 30 mg/dl - urine alkalinization = main method to tx overdoses Na bicarb given with goal serum pH of 7.5

A 14-year-old boy is being evaluated for back pain. He describes bilateral low back pain that began 9 months ago. The pain is intermittent, and is worse after physical activity or prolonged sitting. He does not recall a preceding acute injury or incident. He denies radiation of pain, fever, rash, joint swelling, weakness, and bowel or bladder changes. The boy was evaluated for the pain at an urgent care facility 3 months ago. Radiographs obtained at that visit were reportedly normal. On physical examination, the boy has pain with lumbar flexion and bilateral rotation. He is tender to palpation along the lumbar paraspinal muscles and has tightness of his hamstring and calf muscles. Full neurologic examination findings are normal. Of the following, the MOST appropriate next step in this boy's evaluation and management is:

*physical therapy for core stabilization* - Back pain is common in children and adolescents. Mechanical (muscular) low back pain is the most likely diagnosis. - Children and adolescents with spondylolysis typically report back pain with extension.

A 4-year-old boy has behavioral problems. His foster mother states that when he is upset, he will have a tantrum, throw toys, and hit anyone near him. He was dismissed from preschool because of concerns for the safety of his classmates. The boy was recently placed in the care of his foster mother who does not know the circumstances leading to his removal from his biological family. She asks about possible contributors to his aggressive behaviors. Of the following, the MOST accurate response is that these behaviors have been associated with:

*prenatal exposure to cocaine* - Aggressive behaviors in children have been associated with prenatal exposure to substances including cocaine, alcohol, and tobacco. - Aggression is influenced by the interaction between the prefrontal cortex, which can inhibit actions, and the limbic system, where emotional arousal resides. - Children who have anxiety may demonstrate reactive aggression when their anxiety is triggered and the child is unable to cope.

You are asked to see a 3-week-old neonate who has just been hospitalized because of an apparent life-threatening event. He was delivered at term following an uncomplicated pregnancy. He weighed 2,800 g. He was started on a soy protein-based formula at birth because of a family history of allergy to cow-milk protein. At about 2 weeks of age, he developed postprandial emesis and was switched to a hydrolyzed protein-based formula. However, the vomiting episodes have persisted, and the baby spits up once or twice after each feeding. He currently consumes 6 oz of formula every 3 hours to 4 hours. Several hours ago, immediately after feeding, the baby appeared to be choking, stopped breathing, and developed perioral cyanosis. His mother slapped him on his back and formula was expelled from his nose and mouth. The parents called 911, and the infant was brought to the emergency department, where he appears active and alert. His weight is 3,700 g, temperature is 37.0°C, respiratory rate is 30 breaths/min, pulse rate is 130 beats/min, and oxygen saturation is 99% on room air. Physical examination findings are unremarkable. In the emergency department, blood, urine, and spinal fluid cultures are obtained, and he is started on intravenous antibiotics and admitted for observation and continued care. Of the following, the MOST appropriate next step is

*reduced feeding volumes* - 3-week-old newborn presents with an apparent life-threatening event. This is an observer-dependent phenomenon, characterized by a combination of apnea, abnormal muscle tone (limpness, rigidity), choking, and color change (pallor, cyanosis, plethora). Apparent life-threatening events typically are reported in infants at 1 to 2 months of age, and are rarely described after 8 months of age. Although specific etiologies are poorly understood, gastroesophageal reflux (GER) has been causally implicated. However, supportive data are conflicting, and the relationship between apnea and GER remains controversial. Accordingly, in this infant whose clinical history otherwise suggests a diagnosis of uncomplicated GER, which may be exacerbated by overfeeding, current best evidence indicates that the most appropriate approach to management would include a reduction in feeding volumes. - Gastroesophageal reflux is defined by the passage of gastric contents into the esophagus, with or without regurgitation or vomiting. It is a normal physiologic process occurring several times per day in healthy infants, children, and adults. Most episodes are transient, lasting less than 3 minutes, occurring in the initial 2 hours postprandially, and followed by rapid esophageal clearance of the refluxate. These brief, recurring GER events are generally unassociated with clinical signs or symptoms. During the first 3 months after birth, regurgitation (defined as effortless passage of gastric contents into the mouth) or vomiting events (which may be projectile) are noted daily in 50% of infants. These GER episodes arise from several potential anatomic and physiologic mechanisms, the most prominent being repeated, transient relaxation of the lower esophageal sphincter (LES). Available reports show that 60% to 70% of infants experience more than 1 episode of emesis per day by 3 to 4 months of age. Resolution of GER symptoms occurs in most infants by 1 year of age. The emesis prevalence rate decreases to 10% by 7 to 9 months of age and to 5% by 10 to 12 months of age. As in the vignette, a diagnosis of GER in infants is based solely on a history of spitting-up gastric contents. Formal intraesophageal pH monitoring is of no value in routine evaluation. This study should be reserved for assessing equivocal reflux cases, or in an attempt to correlate symptoms with reflux episodes. A barium esophagram cannot quantify the extent or severity of GER, and is of use solely to document anatomic integrity. - Early studies suggest that a potential mechanism for reflux-induced apnea involved acid stimulation of pharyngeal and esophageal chemoreceptors, leading to laryngospasm. However, more recently, large case series have failed to demonstrate a consistent GER-apnea link. Where respiratory status was monitored along with both esophageal pH and bioelectrical impedance in infants presenting with a history of apnea, only 15% of apneic episodes were correlated with GER. Furthermore, these episodes were as likely to occur with nonacid as with acid GER. Additional data have failed to demonstrate any clinical efficacy of acid reduction therapy in preventing or ameliorating apnea events. Thus, acid blockade with either an H2-receptor antagonist or a proton pump inhibitor is not indicated in this clinical setting.

For diagnosis of BPD, the patient has to have ...

*required at least 28 days of > 21% oxygen or has to have continued need for supplemental oxygen at or ≥ 36 wk postmenstrual age* Chest x-ray - initially shows diffuse haziness due to accumulation of exudative fluid; appearance then becomes multicystic or spongelike, with alternating areas of emphysema, pulmonary scarring, and atelectasis. - alveolar epithelium may slough, and macrophages, neutrophils, and inflammatory mediators may be found in the tracheal aspirate. https://www.merckmanuals.com/professional/pediatrics/respiratory-problems-in-neonates/bronchopulmonary-dysplasia-bpd

A 2-year-old boy is admitted to the pediatric floor for observation due to poor oral intake and mild dehydration. He is an otherwise healthy child but is unvaccinated due to parental preference. His father reports that he has had "a head cold" for a few days with subjective fever and malaise. Over the past 24 hours, he has been increasingly uncomfortable, and this morning his parents noticed swelling on the left side of his face. On examination, the child is nontoxic but tired appearing. He has swelling over his left cheek that obscures the angle of the mandible. His left earlobe is displaced outward due to the swelling. The affected area is nonerythematous and not warm to touch but is tender. Intraorally, there is edema and erythema at the opening of the Stensen duct on the left, but no discharge. The oropharynx is noninflamed and free of exudate. The boy's ear canals and tympanic membranes are normal, as is the remainder of the examination. Which of the following is the most appropriate management?

*supportive care and expectant management* - Mumps typically presents in association with a mild upper respiratory illness with unilateral parotid swelling that develops over 12 to 24 hours. In most cases, there will be subsequent progression to bilateral parotitis shortly thereafter. Parotid swelling localizes anteriorly and inferiorly to the ear and often displaces the earlobe. It may cause dysphagia, headache, or earache, and trismus may be seen. - Oral examination should reveal localized erythema and edema at the orifice of the affected Stensen duct on the buccal mucosa. - Systemic symptoms of fever, malaise, and myalgias are common. Diagnosis is clinical, although serologic testing for the virus may be performed for confirmation and epidemiologic use. - Supportive care and expectant management are the standard of care. In most children, systemic symptoms resolve within five days, and facial swelling resolves within one week.

Lymphadenopathy in what region region should always raise concern for malignancy.

*supraclavicular* - Transient recurring lymphadenopathy that is temporally related to intermittent viral illnesses is almost always benign in young children. - A thorough history and physical examination is the first step in determining the cause of lymphadenopathy. - Lymphadenopathy with rapid progression in size or duration of more than 4 weeks merits further investigation.

A 2-week-old female newborn is brought to your office for a health supervision visit. The mother is concerned that, for the past 2 days, the baby has not been breastfeeding well. The newborn tires after feeding for only 5 minutes and had only 2 wet diapers today. She was delivered at 39 weeks of gestation to a 23-year-old gravida 1 para 0 woman. Her Apgar scores were 8 and 9 at birth and 5 minutes, respectively, and she had a birthweight of 3,500 g. The pregnancy was uncomplicated and the mother was group B Streptococcus negative. On physical examination, the newborn appears sleepy. Her heart rate is 270 beats/min, respiratory rate is 60 breaths/min, blood pressure is 82/40 mm Hg, and oxygen saturation is 95% in room air. She is warm and well perfused. Breath sounds are clear. A 2/6 systolic murmur can be heard at the left upper sternal border, with no gallop or rub. Her liver is palpable 2 cm below the right costal margin. Capillary refill time is 2 seconds. You refer the patient to the emergency department where they perform electrocardiography. Of the following, the MOST likely finding on this test is

*supraventricular tachycardia* - The newborn in this vignette was previously well, with no history of a murmur at birth. Her oxygen saturation is minimally decreased, but is not suggestive of a mixing lesion or congenital heart disease with decreased pulmonary blood flow. The most common cause for a heart rate of 270 beats/min in this age group is an accessory pathway-mediated, reentrant supraventricular tachycardia (SVT). This may be associated with Wolff-Parkinson-White syndrome (WPW) or pre-excitation. Electrocardiography (ECG) in the usual form of reentrant SVT will be narrow complex and very regular. P waves will not be seen before the QRS complexes - The baby in the vignette has a heart rate that is too rapid for sinus tachycardia in a term newborn, and a history of fluid loss or fever would be expected. - The most common cause for SVT in infants is reentry due to an accessory pathway. The ECG will most commonly show a regular, narrow complex rhythm greater than 220 beats/min.

A 10-year-old boy is seen for evaluation of left heel pain. He was previously healthy until the onset of pain several months ago, shortly after the beginning of his soccer season. The pain initially occurred during and after running, but it is now present throughout the day and is associated with a limp. The boy and his mother report no traumatic injury, and there has been no associated swelling, bruising, or other musculoskeletal concerns. The pain improved significantly during a 1-week break from soccer, but it recurred within several days of return to practice. He has focal tenderness over the medial and lateral aspects of his left heel and prefers to walk on his toes. The remainder of his physical examination findings are unremarkable.Of the following, the BEST next step in his care is

*trial of heel cups or cushions* - Sever disease often results from participation in sports with cleats or poorly cushioned footwear. - Assuring adequate cushioning in shoes worn for sports and daily activity can markedly reduce the symptoms of Sever disease.

Decision regarding treatment of polycythemia in infants should be made on basis of...

*venous* Hct

Spirometry for Obstructive - FVC - FRC - TLC - FEV1 - FEV1/FVC - FEF25-75

- FVC normal or increased - FRC increased - TLC increased - FEV1 decreased - FEV1/FVC decreased - FEF25-75 decreased

How to use depo-provera (DMPA)

- IM or SQ (can self-inject) - every 3 mos (12 weeks) - efficacy to 15 weeks

Chloride in pyloric stenosis

- Low serum chloride levels and metabolic alkalosis

Moderate persistent asthma

- Symptoms daily - Nighttime awakenings more more than 1 night/week (but not every night) - Use SABA daily - Have an FEV1 60-80% predicted - Preferred treatment is low-dose inhaled corticosteroid with a long acting beta agonist or medium-dose inhaled corticosteroid

Fabry disease is an X-linked inborn error of glycosphingolipid metabolism characterized by ...

- angiokeratomas (telangiectatic skin lesions) - hypohidrosis - corneal and lenticular opacities - acroparesthesias - vascular disease of the kidney, heart, and/or brain

Bacterial sinusitis is present in 5% to 10% of cases of URI; it is distinguished from a simple viral URI by:

- at least 10 days of symptoms without improvement - new or worsening fever (> 39°C) - worsening cough after initial improvement

Treatment for Medium-chain acyl-CoA dehydrogenase deficiency (MCAD)

- avoid fasting - frequent feeding - glucose infusion - uncooked starch

Nebulizer - used for: - only medications available via nebulizer:

- babies and younger children unable to use mask - pulmicort (budesonide), atrovent (ipratropium), albuterol

Chronic prostatitis can also present with

- chronic pelvic pain syndrome - new onset premature ejaculation - hesitancy with frequency

Techniques to detect strabismus...

- cover-uncover - alternate cover - corneal light reflex

IV infusion for persistent hypoglycemia in newborns....

- give initial bolus of D10W IV at 2-4 cc/kg - continue IV infusion of D10W at 4-8 mg/kg/min - recheck blood glucose 20 min after starting infusion --> if glucose still low, consider increasing infusion by 2-4 mg/kg/min - if infusion rate reaches >140 cc/kg/24h, increase glucose concentration to D12.5W - central line placement required if D15W necessary to maintain normal blood glucose level

Goals for T1D pts - glucose before meals: - glucose at bedtime/overnight: - A1C

- glucose before meals = 90-130 - glucose at bedtime/overnight = 90-150 - A1C = <7.5%

The 3 key components of clinical brain death diagnosis are

- irreversible coma/unresponsiveness - absence of brainstem reflexes - apnea More recommendations - For children aged 7 days to 2 mo, 2 examinations separated by at least 48 hr are recommended. - For children aged 2 mo to 1 yr, 2 examinations separated by at least 24 hr are recommended. - For children older than 1 yr the recommendation is a 12-hr observation period between examinations. - According to the 1987 recommendations, confirmatory testing should be performed on all children <1 yr of age.

Length of appendix is highly variable between individuals and is never measured. The sonographic criteria for appendicitis include:

- non-compressible tubular structure in RLQ - attached to cecum - blind-ending - 6mm or greater in diameter - wall is hypoechoic - ill-defined May also be - appendicolith - air in appendix or in wall - fluid around tip of appendix

Characteristics of intermittent asthma include

- normal pulmonary function test results - daytime symptoms fewer than 2 days per week - nighttime symptoms fewer than 2 days per month

Visual screening for newborn to 6 mos:

- ocular history - external inspection of eyes/lids - red reflex testing - pupil exam

Visual screening for 1-3 yo

- ocular history - external inspection of eyes/lids - red reflex testing - pupil exam - ocular motility assessment - instrument-based screening when available - visual acuity: age-appropriate ototype assessment

Visual screening for >6 yo

- ocular history - external inspection of eyes/lids - red reflex testing - pupil exam - ocular motility assessment - visual acuity: age-appropriate ototype assessment

All children should undergo cholesterol screening once when?...

- once between ages 9-11 yo - once between 17-21 yo

Tx for ophthalmia neonatorum via chlamydia trachomatis (most common)

- oral erythromycin 12.5 mg/kg q6hrs x2weeks or - azithromycin suspension 20 mg/kg qdaily for 3 days

The AAP interim guidance on administration of palivizumab during this delayed season is the same as for a typical season. Consider administering the humanized monoclonal antibody in up to five monthly doses to prevent RSV in high-risk infants and children in areas experiencing high rates of the virus. Eligible children include

- premature infants, especially those born at less than 29 weeks' gestation - infants with chronic lung disease of prematurity - infants with certain types of hemodynamically significant congenital heart disease - infants and young children with certain immunodeficiency states - infants with pulmonary abnormalities or neurological and neuromuscular conditions that impair ability to clear secretions from the upper airway Clinicians should reassess the need for palivizumab at least monthly

Normal infant crying - starts at: - peaks at: - improves by:

- starts at 2 weeks - peaks at 6 weeks - improves by 3 months

You're called into an L&D room for a delivery! First check the warmer to make sure...

- warmer on - Oxygen flowing - PEEP to 5 - FiO2 to 21% - PIP to 20

Typical dexamethasone dosage for peds asthmatic

0.6mg/kg PO/IM Q24hr x2 doses PO preferred

A 16-year-old boy adolescent end-stage renal disease who is scheduled to undergo related-donor renal transplantation in 3 months is undergoing a review of his immunization status. He has received all age-appropriate immunizations, including 4 doses of 7-valent pneumococcal conjugate vaccine (PCV7). He has not received any doses of 13-valent pneumococcal conjugate vaccine (PCV13) or 23-valent pneumococcal polysaccharide vaccine (PPSV23). Of the following, the MOST appropriate next step for this patient is to administer

1 dose of PCV13 followed by 1 dose of PPSV23 at least 8 weeks later - The 16-year-old high-risk patient in the vignette who has end-stage renal disease and is scheduled to receive a renal transplant in 3 months has not received any dose of 13-valent pneumococcal conjugate vaccine (PCV13) or 23-valent pneumococcal polysaccharide vaccine (PPSV23). He should receive 1 dose of PCV13 followed by 1 dose of PPSV23 at least 8 weeks later. - In children aged 6 through 18 years at high risk of pneumococcal disease who have not received any doses of PCV13 or PPSV23, administration of 1 dose of PCV13 followed by 1 dose of PPSV23 at least 8 weeks later and a second dose of PPSV23 5 years after the first is recommended.

Large VSDs in infants usually cause symptoms at what age?

1 to 2 mos of age - With the onset of ventilation, pulmonary vascular resistance is markedly decreased as a consequence of both active (PO2-related) and passive (mechanical related) pulmonary vasodilation. - In a normal neonate, closure of the ductus arteriosus and the fall in pulmonary vascular resistance decreases pulmonary arterial and right ventricular pressures. - The largest decline in pulmonary resistance from the high fetal levels to the low "adult" levels in the human infant at sea level usually occurs within the 1st 2-3 days but may be prolonged for 7 days or more. - Over the next several weeks of life, pulmonary vascular resistance decreases even further, secondary to a remodeling of the pulmonary vasculature, including thinning of the vascular smooth muscle and recruitment of new vessels. This decrease in pulmonary vascular resistance significantly influences the timing of the clinical appearance of many congenital heart lesions that are dependent on the relative levels of systemic and pulmonary vascular resistances. The left-to-right shunt through a large ventricular septal defect may be minimal in the 1st wk after birth when pulmonary vascular resistance is still high. - As pulmonary resistance decreases in the next week or two, the volume of the left-to-right shunt through the ventricular septal defect increases and eventually leads to symptoms of heart failure within the 1st 2 months of life.

For children with asymptomatic stage 1 hypertension, repeat measurement is recommended in

1 to 2 weeks - A third measurement is done in 3 months and if BP is still high, a diagnostic evaluation is initiated - Children with asymptomatic stage 2 hypertension are recommended to have repeat measurement in 1 week. Once the repeat BP reading is noted to be high, diagnostic evaluation is initiated and a referral to a subspecialist should also be considered.

Instrument-based screening. Photoscreeners: detect amblyopia risk factors. No info on visual acuity. Start using this at...

1 year

Incidence of congenital heart disease

1% - 1st degree relative = 5%

In general, what is adequate for local analgesia?

1% lidocaine buffered with sodium bicarbonate

Adequate protein ingestion to repair and build muscle after working out. Young athletes are generally advised to ingest about

1.5 to 1.8 g of protein/kg body weight per day - A 150-lb (68-kg) athlete needs 105 to 110 g/day of protein. Ingesting more than this does not result in faster increases in muscle mass. - One meat/fish/poultry serving (3 oz) contains about 25 to 30 g of protein. Portion sizes in many restaurant and home-cooked meals are larger than 3 oz. - One dairy serving (8 oz milk or yogurt or 1 oz cheese) contains about 8 g of protein. Greek yogurt can have twice this amount. - One bean/legume serving (1 cup of pinto, black, or kidney beans) contains about 40 g of protein. Soybeans have almost 70 g of protein per cup.

At what age do maternal HIV antibodies typically wane in infants?

10 months

Baby Doe is 2 weeks old. He has a central line. He needs a GIR of 12. You can give him 130 ml/kg/day of dextrose containing fluid. What concentration dextrose would that be?

12 (goal GIR)/130 (available fluid)/0.69 (constant) = 0.1337 = 13.4% dextrose

For what age is the MMRV (proquad) approved?

12 months - 5 years BUT increased risk of febrile seizures --> less common 4 years and older - screen for fam hx of febrile seizures

The most recent international guidelines state that orchiopexy to treat cryptorchidism or undescended testicle should be completed by

12 months of age - During normal development, the testes form in the abdomen and then descend into the scrotum. - Having one or both testes undescended at birth is not uncommon (2%-9% of male neonates), but only 1% remain undescended at 3 months of age. - Those infants with testes that remain undescended require surgery to bring the testicle into the scrotum (orchiopexy) or remove the testicle (orchiectomy) because they will not spontaneously descend from the abdomen into the scrotum after this age.

An infant with laryngomalacia will likely resolve without intervention by age

12-18 mos - Evidence of good feeding and growth, normal oxygen saturation, and lack of respiratory compromise are encouraging findings for a benign course. - Reassurance is the only intervention indicated at this point. - Because the stridor is often dynamic, it may not be as obvious at an office visit as it may seem to the parents during the night at home. - Inviting parents to bring audio/video recordings of the breathing pattern to an office visit is a way to make sure they feel heard and understood.

Hepatitis A vaccine or immune globulin can be used for hepatitis A postexposure prophylaxis and should be administered within

14 days of exposure - Hepatitis A vaccine can be used in individuals who are vaccine eligible between the ages of 1 and 40 years, and is preferred over immune globulin because it provides active and more durable immunity. - Immune globulin should be used for hepatitis A postexposure prophylaxis in individuals younger than 12 months, older than 40 years, those who have vaccine contraindications, immunocompromised individuals, and individuals with chronic liver disease.

A child can say three words, not including mama and dada, and can follow directions without a specific gesture. Based on this description of the child's language development, how old is she?

15 months - At age 15 months, a child should be able to use at least three words, excluding names. - A lack of first words by 15 months is considered a red flag and needs urgent evaluation. If this developmental milestone is not met, further investigation should be pursued, including a hearing evaluation and referral to a speech and language specialist. - In addition to saying three words, a 15-month-old child should be able to point to and ask for an object (social development), drop and take an object out of a container (fine motor), and begin to run (gross motor).

Which types of human papillomavirus are collectively responsible for 70% of cervical cancers?

16 and 18.

Healthy women who are moderately active and not pregnant or lactating need how many calories per day?

1800-2000 calories/day - Pregnant women are advised to keep the calories the same in the first trimester (about 1800 calories/day), increase ~200-300 (about 2200 calories/day)in the second trimester, and then increase ~300-400 in third trimester (about 2400 calories/day). - When pregnant or breastfeeding women should eat a diet rich in protein, omega-3 polyunsaturated fats, calcium, iron and folic acid, along with all the necessary vitamins, minerals, and fiber.

In the human brain, most neurons develop prior to ....

18th week of gestation - early gestational growth retardation would be expected to affect fetus is symmetric manner --> permanent neurologic consequences for infant --> exs = genetic or chromosomal causes, early gestational intrauterine infections (TORCH), maternal alcohol use

Recommended schedule of hep A vaccine

2-dose series - starting at 12 mo - administered at least 6 mos apart and begun before 2nd birthday - series begun before 2nd bday should be completed even if child turns 2 before 2nd dose is adminsitered

Head circumference at birth ...

35 cm - head growth = 1 cm/month in first 6 mos; 0.5 cm/month 6-12 mos

Eruption of primary teeth usually occurs around

4 - 15 months - lower teeth usually erupt before top ones - delayed eruption = after 18 months (think of hypopit, hypothyroid, down syndrome)

AAP recommends exclusive breastfeeding until

4 - 6 months of life

Most children won't be able to read the eye chart until they are how old?

4 or 5 yo

In many populations, physiologic knee valgus peaks at

4 years of age - then decreases until final alignment is reached at about 7 years of age - there may be some racial and ethnic variation in this timing, with recent studies showing that peak valgus occurs at 5 to 6 years of age in Indian children and age 6 to 7 years in Turkish children - most older children and adults have mild valgus knee alignment - valgus onset before age 2 years, or which worsens after expected peak age, warrants additional evaluation

The most important complication of DDH is avascular necrosis of the femoral epiphysis. Reduction of the femoral head under pressure or in extreme abduction can result in occlusion of the epiphyseal vessels and produce either partial or total infarction of the epiphysis. Revascularization soon follows, but if the physis is severely damaged, abnormal growth and development can occur. The hip is most vulnerable to this complication before

4-6 mo - when the ossific nucleus appears

Extrusion reflex usually gone by

4-6 mos - allows for swallowing of non-liquids - infant has doubled birth wt by this time - ability to digest / absorb proteins, fats & carbohydrates is usually sufficiently mature by 4-6 months

Juice intake should be limited to what for ages 1-6 yr?

4-6 oz/day

At which ages are the two peaks of presentation for type 1 diabetes mellitus?

4-6 years and 10-14 years.

Normal glucose utilization rate in a newborn is

4-8 mg/kg/min - Initial glucose administration rate should be in that range.

For the premature infant with respiratory distress syndrome, warm humidified oxygen should be provided at a concentration initially sufficient to keep arterial oxygen pressure between ...

40 and 70 mm Hg (85-95% saturation) - in order to maintain normal tissue oxygenation while minimizing the risk of oxygen toxicity - If oxygen saturation cannot be kept > 85% at inspired oxygen concentrations of 40-70% or greater, applying CPAP at a pressure of 5-10 cm H2O via nasal prongs is indicated and usually produces a sharp improvement in oxygenation

A 9 yr old girl presented with persistent fatigue and hepatomegaly and was found to have an ALT of 275 IU/L; total bilirubin, 1.2 mg/dL; alkaline phosphatase, 265 IU/L: and antinuclear antibody titer, 1:80. Liver biopsy revealed a lymphoplasmacytic infiltrate of the portal tracts. The most appropriate initial therapy would be:

40 mg of prednisone daily - Prednisone, with or without azathioprine or 6-mercaptopurine, improves the clinical, biochemical, and histologic features in most patients with autoimmune hepatitis and prolongs survival in most patients with severe disease. - The goal of therapy is to suppress or eliminate hepatic inflammation with minimal side effects. - Prednisone at an initial dose of 1-2 mg/ kg/24 hr is continued until aminotransferase values return to less than twice the upper limit of normal. - The dose should then be lowered in 5-mg decrements over 2-4 mo until a maintenance dose of 0.1-0.3 mg/kg/24 hr is achieved. - In patients who respond poorly, who experience severe side effects, or who cannot be maintained on low-dose steroids, azathioprine (1.5-2.0 mg/ kg/24 hr, up to 100 mg/24 hr) can be added, with frequent monitoring for bone marrow suppression. - The initial response to therapy in autoimmune hepatitis is generally prompt, with a > 75% rate of remission. - Transaminases and bilirubin fall to near-normal levels, often in the 1st 1-3 mo. When present, abnormalities in serum albumin and prothrombin time respond over a longer period (3-9 mo).

In the NICU, for oxygen & air sources and blender, for most cases, set blender to deliver

40% O2

Basal/background insulin dose: Basal/background Insulin Dose =

40-50% of Total Daily Insulin Dose

The AAP recommends all infants and children, including adolescents, have minimum daily intake of who much vitamin D?

400 iu/day - any breastfeeding infant, regardless of whether he or she is being supplemented with formula, should be supplemented with 400 iu/day

How much vitamin D should BF'ing infants be taking?

400 units vitamin D

You are assisting in the elective intubation of an average-sized 4-year-old child with respiratory failure. A colleague is retrieving the color-coded length-based tape from the resuscitation cart. Which of the following is likely to be the estimated size of the uncuffed ET tube for this child?

5-mm tube

Which of the following estimates the percentage of children who have received long-term parenteral nutrition that will go on to develop intestinal failure-associated liver disease?

50% - Intestinal failure-associated liver disease typically develops after medical and surgical treatment for intestinal failure. - Diagnosis can be made in children who have a history of intestinal failure and cholestasis from use of long-term parenteral nutrition, in whom other causes of liver injury are ruled out. A - Associated laboratory findings often include elevated conjugated bilirubin, with mild to moderate increases in aspartate aminotransferase (AST), alanine transaminase (ALT), and gamma-glutamyl transpeptidase (GGTP). - Continued use of parenteral nutrition usually leads to progression, end-stage liver disease, and eventually to the need for liver transplantation. - It has been suggested the soybean oils used in intravenous lipid emulsions of parenteral nutrition play a role in development of intestinal failure-associated liver disease.

A 2-month-old male infant is seen for his health supervision visit. He is tracking, smiling socially, and holding his head up without support for a few seconds. He has a 4-year-old sister who is developmentally normal. His mother's brother died at 20 years of age with complications related to a muscle disease. He had calf hypertrophy and used his hands to crawl from a lying to a standing position. He required the use of a wheelchair by 10 years of age. The mother reports that she has no muscle weakness or cramps. The mother underwent echocardiography 5 years ago for chest pain that showed mild dilated cardiomyopathy felt to be related to her brother's disorder. The parents are concerned about their son's risk of experiencing the same condition. Of the following, the MOST accurate percentage chance that this infant has the same condition as his uncle is

50% Duchenne muscular dystrophy - inherited in an X-linked recessive manner ( - delayed achievement of motor milestones, calf pseudohypertrophy in early stages, and proximal muscle weakness that leads to wheelchair dependence by the age of 10 to 12 years - Female carriers are reported to have mild muscle weakness and dilated cardiomyopathy; hence, cardiac surveillance also should be offered to female carriers in the family - Patient's uncle had DMD, the mother is symptomatic with dilated cardiomyopathy, and the maternal grandmother is an obligate carrier because two of her children are affected - The risk of the infant's having DMD is 50%, because the mother could pass on either the mutated or the normal DMD allele to her son

Children older than 5 should be placed in a booster seat until regular seat belts fit correctly, typically at the height of

57 inches (145 cm)

In Erb-Duchenne paralysis, the brachial plexus injury is limited to the ...

5th and 6th cervical nerves - The infant loses the power to abduct the arm from the shoulder, rotate the arm externally, and supinate the forearm - Immobilization should be intermittent throughout the day while the infant is asleep and between feedings - Gentle massage and range-of- motion exercises may be started by 7-10 days of age. Infants should be closely monitored with active and passive corrective exercises - If the paralysis persists without improvement for 3 months, neuroplasty, neurolysis, end-to-end anastomosis, and nerve grafting offer hope for partial recovery

A 12 yr old girl with moderate to severe asthma is sensitive to cat dander. Her family elects to remove the pet cat from the house but to retain the present carpeting and upholstered furniture. What is the length of time required before the levels of cat allergen drop to levels found in homes without a cat?

6 mo - Removing the pet from the home is obviously the most effective means of reducing exposure to animal allergens, although it has been demonstrated that without other interventions, such as removing carpeting and upholstered furniture and wiping down walls, it takes 6 months or more for the levels of cat allergen to drop to a level found in houses without a cat. - As a result, cat owners who remove their pets from their homes should be informed not to expect immediate results.

An infant presents for a routine well-child visit with her mother. The mother reports no concerns other than the baby has started to cry when he sees someone unfamiliar. What age does stranger anxiety usually develop?

6 months

Frist pair of deciduous teeth at what age?

6 mos - all baby teeth by 2 yo

In those with congenital growth hormone deficiency, linear growth becomes abnormal at what age?

6 to 12 months - Patients with acquired growth hormone deficiency should undergo investigation for a brain neoplasm and other pituitary hormone deficiencies. - The weight-for-length or body mass index curve is important to consider in narrowing the differential diagnosis of abnormal growth.

At what age should children make eye contact and smile?

6 weeks

Crying peaks at what age in infants?

6 weeks - then gradually decreases

At a boy's health supervision visit, his father wishes to know how to manage the child's recent behaviors. Last week, after a trip to the store, he found a new toy in his son's jacket pocket. When asked, the boy stated that he did not know how the toy came to be there. He later admitted to taking the toy because he did not think that his parents would buy it for him. The boy's father has also noted that when playing board games, his son will change the rules to his advantage. The child has been in good health and his development and behaviors have been age appropriate up to this point. Of the following, this child's behavior is MOST like that of a

6 years - The behavior of the child in the vignette is most like that of a typical 6 year old who may understand that there are rules but may have a hard time controlling his impulses.

In newborn, saturation goal at 1 minute...

60-65%

A 5-year-old is intubated with a 5.0 cuffed ET tube. To suction his ET tube, which one of the following suction catheter size is most appropriate?

7 F - ET tube size refers to the internal diameter of the tube in mm, so a 5.0 tube's inner diameter is 5 mm. - Suction catheter come in French sizes (F). French size refers to the circumference of the tube (circumference = diameter × 3.14). - To convert ET tube size to French size, multiply it by 3, (5 × 3 = 15), then divide this number by 2, in order to use the half or less the ET tube diameter (7.5).

The ratio of upper body segment to lower body segment changes from 1.7 at birth to 1.0 by what age?

7 years - The head and trunk are relatively large at birth, with progressive lengthening of the limbs throughout development, particularly during puberty. - The lower body segment is defined as the length from the symphysis pubis to the floor, and the upper body segment is the height minus the lower body segment. - The ratio of upper body segment divided by lower body segment (U/L ratio) equals approximately 1.7 at birth, 1.3 at 3 yr of age, and 1.0 after 7 yr of age. - Higher U/L ratios are characteristic of short-limb dwarfism or bone disorders, such as rickets.

Myelinization is complete by what age?

7 yo

Incubation period of pertussis lasts how long?

7-10 days

Frontal sinuses in children begin at what age?

7-8 years

A 5 yr old girl presents with cola-colored urine, oliguria, and body edema 2 wk after being treated for group A β-hemolytic streptococcal pharyngitis. Her complement C3 is noted to be very low at 15 mg/dL. When should this patient's complement C3 level be repeated in order to confirm your suspected diagnosis?

8 weeks Poststreptococcal glomerulonephritis (GN) - likely in a child presenting with acute nephritic syndrome, evidence of recent streptococcal infection, and a low C3 level - renal biopsy should be considered only in the presence of acute renal failure, nephrotic syndrome, absence of evidence of streptococcal infection, or normal complement levels - renal biopsy is considered when hematuria and proteinuria, diminished renal function, and/or a low C3 level persist more than 2 mo after onset - persistent hypocomplementemia can indicate a chronic form of postinfectious GN or another disease such as membranoproliferative GN

A girl comes to the clinic with her mother for a health supervision visit. She sits quietly reading a book, which she can do with just a little help from her mother. The girl's participation in age-appropriate activities and her development are reviewed. She has joined a soccer team this year and really enjoys playing competitive sports. Math is her favorite class; they are learning fractions. She names several friends and says she enjoys playing with both sexes but does not have one single best friend. Of the following, the girl's age is MOST likely

8 years - children's social and cognitive skills grow substantially from 6 to 12 years of age - 8-year-old is developing relationships with her peers, often of both sexes, but may not have a single best friend yet - at this age, it is appropriate to participate in competitive sports, as well as other group activities such as scouting - efficiency and confidence in reading and school subjects, as well as independence, are growing

Juice intake should be limited to what for ages 7-18 yr?

8-12 oz per day

In newborn, saturation goal at 5 minutes...

80-85%

In newborn, saturation goal at 10 minutes...

85-95%

Baby Smith does not have a central line. He can only have D12.5. He needs to get to a GIR of 9. How much fluid will you need to give him?

9 (goal GIR) /0.69 (constant) /0.125 (dextrose available) = 104.3 = 104 ml/kg/day

CPM is more common in patients who are treated for chronic hyponatremia than in those treated for acute hyponatremia. Presumably, this difference is based on the adaptation of brain cells to the hyponatremia. The reduced intracellular osmolality that is an adaptive mechanism for chronic hyponatremia makes brain cells susceptible to dehydration during rapid correction of the hyponatremia, and this may be the mechanism of CPM. Even though CPM is rare in pediatric patients, it is advisable to avoid correcting the serum sodium concentration by ...

>12 mEq/L/24 hr or >18 mEq/L/48 hr - Desmopressin is a potential option if the serum sodium level is increasing too rapidly.

What describes the recommended use of HRIG for postexposure rabies prophylaxis in the United States?

Administer as much of the HRIG dose as possible at the exposure site and the remainder at another site intramuscularly; always give rabies vaccine as well - Rabies PEP is most effective when applied expeditiously. - The series should be begun in the asymptomatic person as soon as possible, regardless of the length of time since the bite. - The 1st step in rabies PEP is to cleanse the wound thoroughly. - The 2nd component of rabies PEP consists of passive immunization with RIG. - Most failures of PEP are attributed to not using RIG. - Human RIG, the formulation used in industrialized countries, is administered at a dose of 20 IU/kg. - As much of the dose is infused around the wound as possible, and the remainder is injected intramuscularly in a limb distant from the one injected with the killed vaccine. - Like other immune globulin preparations, RIG interferes with the take of live viral vaccines for at least 4 mo after administration of the RIG dose. - The 3rd component of rabies PEP is immunization with inactivated vaccine.

A 2100-g infant of a diabetic mother experiences seizures on the first day of life. Pregnancy was complicated by severe diabetic vasculopathy and placental insufficiency, with the development of late decelerations and the need for emergency cesarean section. The Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. During the first 6 hr of life, the child was well and tolerated formula feedings well. Jitteriness developed at 10 hr of age and progressed to tonic-clonic seizures at 18 hr of age. Laboratory studies revealed blood glucose of 80 mg/dL and calcium of 6.0 mg/dL. Thereafter, calcium gluconate (10%), 2 mL/kg, was given repeatedly without affecting the frequency of seizures. The most appropriate step to manage this infant's condition is to:

Administer magnesium sulfate - Rarely, hypomagnesemia of unknown cause may occur in newborn infants, usually in association with hypocalcemia. It also may be associated with insufficient stores of skeletal magnesium secondary to deficient placental transfer, decreased intestinal absorption, neonatal hypoparathyroidism, hyperphosphatemia, renal loss, a defect in magnesium and calcium homeostasis, or iatrogenic deficiency. - Infants of diabetic mothers may have lower than normal serum magnesium levels. - The clinical manifestations of hypomagnesemia are indistinguishable from those of hypocalcemia and tetany and may, in fact, contribute to the accompanying hypocalcemia. - Because the hypocalcemia accompanying hypomagnesemia is inadequately corrected by administration of calcium alone, hypomagnesemia also should be suspected in any patient with tetany not responding to calcium therapy.

A 2 yr old toddler is brought to the emergency department in the morning because he is lethargic. His parents report that he did not eat much dinner the night before. This morning they checked on him at 9 AM, 2 hours after he normally wakes, and they were unable to fully arouse him. In the emergency department, blood glucose level is 29 mg/dL and plasma insulin is low. A urine sample is positive for ketones. IV D10W bolus is given with appropriate rise in glucose. In addition to observing the patient take carbohydrates by mouth and documenting normoglycemia, the most appropriate management is:

Advise parents to limit overnight fasting to 10 hours; provide urine dipsticks to check for ketones when the patient is ill Idiopathic ketotic hypoglycemia - most common form of childhood hypoglycemia - usually presents between the ages of 18 mo and 5 yr and commonly remits spontaneously by the age of 8-9 yr - hypoglycemic episodes typically occur during periods of intercurrent illness when food intake is limited - classic history is of a child who eats poorly or completely avoids the evening meal, is difficult to arouse from sleep the following morning and hence eats poorly again, and may have a seizure or be comatose by mid morning - another common presentation occurs when parents sleep late and the affected child is unable to eat breakfast, thus prolonging the overnight fast - at the time of documented hypoglycemia, there is associated ketonuria and ketonemia; plasma insulin concentrations are appropriately low, ≤5U/mL, thus excluding hyperinsulinemia - In anticipation of spontaneous resolution of this syndrome, treatment of ketotic hypoglycemia consists of frequent feedings of a high-protein, high-carbohydrate diet - During intercurrent illnesses, parents should test the child's urine for the presence of ketones, the appearance of which precedes hypoglycemia by several hours - In the presence of ketonuria, liquids of high carbohydrate content should be offered to the child - If these cannot be tolerated, the child should be treated with IV glucose administration in a hospital.

Neonate with microcephaly, intracranial calcifications, hepatosplenomegaly, petechial rash, thrombocytopenia, and hepatitis has congenital cytomegalovirus (CMV) infection. What is the recommended treatment for term neonates with symptomatic congenital CMV infection with and without central nervous system disease?

Antiviral treatment with oral valganciclovir for 6 months - Therapy must be started by 4 weeks of age - Cytomegalovirus is the most common congenital viral infection in the United States, occurring in 0.5% to 1% of all live births. Mother-to-infant transmission of CMV can occur during pregnancy through transplacental spread of maternal virus, during delivery via exposure to infected maternal cervical secretions, or postnatally through breast milk or by receiving blood products from CMV-positive donors. The rate of intrauterine transmission is 30% to 40% in women with primary CMV infection (ie, mothers with no preexisting immunity) but is only 1% in women with nonprimary CMV infection (ie, CMV reactivation or reinfection with a different viral strain in mothers with preexisting immunity). - Real-time PCR is the preferred diagnostic test for detecting CMV because of its superior sensitivity and specificity, quick turnaround time, and lower cost compared with viral culture and other methods. - Ophthalmologic evaluation may detect chorioretinitis; computed tomography imaging of the brain may detect periventricular calcifications suggestive of congenital CMV infection. - Infants and children with congenital CMV infection must have close follow-up with audiology, ophthalmology, and neurodevelopment services. - Adverse effects of oral valganciclovir therapy include significant neutropenia (in 20% of infants) and hepatotoxicity. - Consultation with a pediatric infectious disease physician is recommended when considering antiviral treatment of symptomatic congenital CMV infection.

The treatment of choice for pediculosis capitis is:

Application of 0.5% malathion - Because of resistance of head lice to pyrethroids, malathion 0.5% in isopropanol is the treatment of choice for head lice and should be applied to dry hair until hair and scalp are wet and left on for 12 hours. A 2nd application 7-9 days after initial treatment may be necessary. - This product is flammable, so care should be taken to avoid open flames. - Malathion, like lindane shampoo, is not indicated for use in neonates and infants. - Additional approved therapies include spinosad (if > 4 yr old), benzyl alcohol lotion (if > 6 mo), and ivermectin for difficult-to-treat head lice. - All household members should be treated at the same time. - Nits can be removed with a fine-toothed comb after application of a damp towel to the scalp for 30 min. - Clothing and bed linens should be laundered in very hot water or dry-cleaned; brushes and combs should be discarded or coated with a pediculicide for 15 min and then thoroughly cleaned in boiling water. - Children may return to school after the initial treatment.

A 1-month-old former full-term Caucasian male presents with poor feeding and abdominal distension. He has had no fever, vomiting, or respiratory symptoms. His mother says his belly seems "full" all the time. He has had somewhat less oral intake over the past week. On examination, he seems moderately uncomfortable. Heart and lung examinations are normal. His abdomen is moderately protuberant, especially on the right side. There is perhaps mild tenderness in the area. Complete blood count is normal. Electrolytes are normal except for a calcium of 11.8 mg/dL. An ultrasound shows a solid mass around the right kidney. Which of the following is the most likely diagnosis?

Congenital mesoblastic nephroma - very young infant with a renal tumor and hypercalcemia. - although the most common type of renal tumor in children is Wilms tumor, patients with Wilms tumor are almost always older than this patient, typically young school age, when they present. - three pathologic subtypes of congenital mesoblastic nephroma: classic, cellular, and mixed types. - standard of care for patients with congenital mesoblastic nephroma is unilateral nephrectomy. - Prognosis for these patients appears to be quite favorable.

A 6-year-old girl is brought to her pediatrician's office with the recent onset of a red-purple rash on her arms and legs. Her physical exam is notable for short stature, petechiae, and café au lait macules. A complete blood count reveals anemia, leukopenia with neutropenia, thrombocytopenia, and the absence of blasts on the differential. The reticulocyte count is low. Which of the following is the mechanism for the pancytopenia seen in this child?

Congenital progressive bone marrow failure Fanconi anemia. - pancytopenia associated with this inherited disease is caused by congenital progressive bone marrow failure. - inability to undergo chromosome breakage repair leading to abnormal development of of multiple organ systems. - absent or hypoplastic thumbs, café au lait macules, short stature, and VACTERL association (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities). - Progressive bone marrow failure is one of the hallmarks of this disease, though time of onset may be variable. P - chromosome breakage test followed by gene sequencing confirms the diagnosis. Due to the inability to repair deoxyribonucleic acid, affected patients are at higher risk for malignancies, most commonly acute myeloid leukemia, followed by solid tumors and squamous cell carcinoma.

Which genetic test should be performed in patients with suspected Beckwith-Wiedemann syndrome?

DNA methylation test (otherwise known as methylation-sensitive multiplex ligation probe analysis)

A 30 wk old average-for-gestational-age male infant is born after 24 hr of ruptured membranes and another 10 hr of labor. After birth, he develops grunting, flaring, and restrictions, as well as cyanosis. He requires intubation, ventilation, and surfactant therapy. Despite these measures he is still hypoxic and hypotensive. You should now:

Do a blood culture and begin intravenous antibiotics - premature infant born after prolonged rupture of membranes is at high risk of infection - respiratory distress may be a manifestation of sepsis and/or pneumonia - important to begin IV antibiotics rapidly after obtaining a blood culture - although a lumbar puncture is eventually indicated, this neonate is too ill to undergo the procedure currently

What is typically seen on biopsy of a molluscum contagiosum lesion?

Eosinophilic cytoplasmic inclusion bodies within keratinocytes.

A 3-year-old girl presents to your office for a follow-up visit after being seen yesterday in the emergency room for a simple febrile seizure and fever due to acute otitis media. This was her first febrile seizure, she recovered completely without any further seizures, and she is being treated with antibiotics for acute otitis media. Which of the following characteristics places her at higher risk for seizure recurrence?

First degree relative with a history of febrile seizures Febrile seizures - most commonly in children ages 6 months through 5 years with a prevalence of approximately 2 to 4% of children in the United States. - Approximately one-third of children who have a febrile seizure will have another one, with the risk of recurrence being highest in the year following the first febrile seizure. - One of the risk factors associated with recurrence of febrile seizures is a family history of febrile seizures in a first degree relative.

Thyroid hormone replacement dosing for hypothyroidism in neonate

First get repeat labs: FT4, TSH Start with 12-17 mcg/kg/d (27.5 - 50 mcg/d) - Then 10-15 mcg/kg/d - Typical (term infant) = 37.5 mcg/day - Crush tablet and mix with water and give via spoon or syringe - Do not give at same time as multivitamin, simethicone, or iron

A 16-year-old boy presents to clinic for a well-child visit. He has no concerns today. His temperature is 36.8°C, heart rate is 82 beats per minute, respiratory rate is 16 breaths per min, and blood pressure is 114/68 mm Hg. Findings on his physical examination are unremarkable. His urinalysis shows a urine specific gravity of 1.020, pH of 6.5, 3+ protein, and no blood. Microscopy findings are negative. What is the next best step in management?

First morning void urine specimen to measure protein and creatinine concentrations asymptomatic isolated proteinuria without any signs or symptoms - Since this patient has a normal blood pressure and does not have any associated hematuria, he is unlikely to have renal disease. - The most likely diagnosis for this adolescent is orthostatic proteinuria, as it is the most common cause of asymptomatic persistent proteinuria in children. - Patients with orthostatic proteinuria are asymptomatic and are diagnosed incidentally when a urinalysis is done as part of routine care. - In orthostatic proteinuria, the amount of urinary protein excreted in the supine position is normal, but that excreted in the upright position may be as much as tenfold higher than normal. - Orthostatic proteinuria is best diagnosed with a first morning void urine specimen to measure protein and creatinine concentrations. If the first morning void does not show any protein, orthostatic proteinuria is confirmed. - Annual urinalysis is recommended for patients with orthostatic proteinuria to monitor for progression.

A 26 mo old child with sickle cell disease appears in your office for the 1st time. He has a history of a prior hospitalization for pneumococcal bacteremia. Currently the child is on penicillin prophylaxis, but he has not received either the conjugate or polysaccharide pneumococcal vaccine. Which of the following is the optimal means for protecting this child from developing another episode of invasive pneumococcal disease?

Give 1 dose of the pneumococcal conjugate vaccine followed 1 month later by one dose of the pneumococcal polysaccharide vaccine and continue penicillin prophylaxis - Immunization with PCV13 is recommended for all infants on a schedule for primary immunization, in previously unvaccinated infants, and for transition for those partially vaccinated with PCV7. - High-risk children ≥ 2 yr of age, such as those with asplenia, sickle cell disease, some types of immune deficiency (e.g., antibody deficiencies), HIV infection, cochlear implant, CSF leak, diabetes mellitus, and chronic lung, heart, or kidney disease (including nephrotic syndrome), may benefit also from PPSV23 administered after 2 yr of age following priming with the scheduled doses of PCV13. - Thus, it is recommended that children ≥ 2 yr of age with these underlying conditions receive supplemental vaccination with PPSV23. - A 2nd dose of PPSV23 is recommended 5 yr after the 1st dose of PPSV23 for persons aged ≥ 2 yr who are immunocompromised, have sickle cell disease, or functional or anatomic asplenia.

Severe, isolated anemia is caused either by inadequate red blood cell production, which would manifest with a low reticulocyte count, or by increased destruction, which would manifest with an increased reticulocyte count. The definitive therapy for autoimmune hemolytic anemia is immune suppression. What are the first-line treatment of choice?

Glucocorticoids - In the setting of autoimmune hemolytic anemia, symptoms suggestive of inadequate oxygen delivery to a critical organ (eg, brain [confusion]) or heart [chest pain]) would make transfusion of a least incompatible unit of packed red blood cells an appropriate first step in treatment.

A 7-month-old boy presents to the ED with recurrent seizures precipitated by hypoglycemia. He is stabilized, and a metabolic workup commences to find the etiology of the hypoglycemia. It is noted that he has ketosis. Serum uric acid, lipids, and lactic acid are all elevated. What is the most likely diagnosis?

Glucose-6-phosphatase deficiency - glycogen storage disease in which glucose-6-phosphatase is not converted to glucose in the liver. - Symptoms of recurrent hypoglycemia occur after 3 months of age, and signs include cherubic facies, hepatomegaly, renomegaly, and distended abdomen. - This patient has hypoglycemia with ketosis along with hyperuricemia, hyperlipidemia, and lactic acidosis, which are consistent with glucose-6-phosphatase deficiency. - DNA testing is done to confirm glucose-6-phosphatase deficiency.

What is harlequin ichthyosis?

Harlequin ichthyosis is a severe congenital disorder of keratinization that causes thick, fissured, armor-plate-like skin, eclabium (lip eversion), and severe ectropion (eyelid eversion). It is distinct from and not to be confused with harlequin color change.

A history of recurrent painless gross hematuria, proteinuria, hypertension, and azotemia favor a diagnosis of acute glomerulonephritis (AGN). A normal serum complement level in this child further supports a diagnosis of

IgA nephritis - Acute glomerulonephritis or nephritic syndrome is characterized by sudden-onset hematuria (macroscopic or microscopic), proteinuria, hypertension, edema, and acute kidney injury. - The presence of cola-colored or tea-colored urine without clots and red blood cell casts on urinalysis suggest hematuria of a glomerular origin. - Laboratory evaluation in a child presenting with AGN includes urinalysis with microscopy, serum electrolytes, blood urea nitrogen, serum creatinine, serum albumin, and a complete blood count. - The serum complement levels (C3 and C4) differentiate causes of hypocomplementemic AGN from those with normal complement levels - Immunoglobulin A nephritis characteristically presents with recurrent episodes of gross hematuria triggered by an upper respiratory tract or gastrointestinal illness. - The synpharyngitic (concomitant with an upper respiratory illness) presentation of IgA nephritis differentiates it from acute poststreptococcal glomerulonephritis, in which a prior history of sore throat is present. - The gross hematuria usually resolves within a few days to a week. - The microscopic hematuria may persist or completely clear in between episodes of recurrent gross hematuria. - Occasionally, children with IgA nephritis may present only with persistent microscopic hematuria and worsening proteinuria.

A 12 yr old boy sustains a nail puncture of the right foot through an old sneaker. Two days later, he limps and complains of pain and swelling in that area. The most appropriate 1st step in management of the boy is:

Incision, drainage, debridement - Most puncture wound injuries to the foot may be adequately managed in the emergency department. Treatment involves a thorough irrigation and a tetanus booster, if appropriate; many clinicians recommend antibiotics. - A subset of patients develop cellulitis, most often due to Staphylococcus aureus, and require intravenous antibiotics with or without surgical drainage. - Deep infection is uncommon and may be associated with septic arthritis, osteochondritis, or osteomyelitis. The most common organisms are S. aureus and Pseudomonas; the treatment involves a thorough surgical debridement followed by a short (10-14 days) course of systemic antibiotics. - Plain radiographs demonstrate any metallic fragments, but ultrasonography may be necessary to identify glass or wooden objects. R - Pain and/or gait disturbance is more likely with superficial objects under the plantar surface of the foot. - One special situation occurs when a puncture wound from a nail comes through an old sneaker. - There is a high risk of a pseudomonal infection, and thorough irrigation and debridement under general anesthesia followed by systemic antibiotics for 10-14 days should be considered.

A 6 mo old infant has a 6-wk history of intermittent vomiting and poor weight gain. The prenatal history is unremarkable. Meconium was passed on the 2nd day of life. Which of the following findings are diagnostic studies most likely to show?

Inversion of the superior mesenteric artery and vein noted on abdominal ultrasound examination - The majority of patients present in the 1st yr of life, and > 50% present within the 1st month of life, with symptoms of acute or chronic obstruction. Vomiting is the most common symptom in this age group. - Infants often present in the 1st week of life with bilious emesis and acute bowel obstruction. - Older infants present with episodes of recurrent abdominal pain that can mimic colic and suggest intermittent volvulus. - Malrotation in older children can manifest with recurrent episodes of vomiting and/or abdominal pain. - Patients occasionally present with malabsorption or protein-losing enteropathy associated with bacterial overgrowth. - Symptoms are caused by intermittent volvulus or duodenal compression by Ladd bands or other adhesive bands affecting the small and large bowel. - Volvulus occurs when the small bowel twists around the superior mesenteric artery leading to vascular compromise of the bowel. - Upper gastrointestinal series is the imaging test of choice and the gold standard in the evaluation and diagnosis of malrotation and volvulus. It is the best exam to visualize the malposition of the ligament of Treitz and can also reveal a corkscrew appearance of the small bowel or a duodenal obstruction with a "bird's beak" appearance of the duodenum. - A superior mesenteric vein located to the left of the superior mesenteric artery suggests malrotation. - Malrotation with volvulus is suggested by duodenal obstruction, thickened bowel loops to the right of the spine, and free peritoneal fluid.

The classic presentation is of an older male adolescent or young adult with small testes, gynecomastia (38%), tall stature, long arms and legs, and possibly learning or behavioral difficulties

Klinefelter syndrome or 47,XXY syndrome - Classic laboratory abnormalities include a significantly elevated follicle-stimulating hormone concentration, increased luteinizing hormone concentration, low testosterone levels, and a 47,XXY karyotype - Testosterone replacement therapy, as well as identification and management of the comorbid disorders, remain important outcome determinants for children with Klinefelter syndrome

Neonates born to mothers with this infection are often delivered prematurely with brown- or green-stained amniotic fluid. This early-onset neonatal infection is uncommon, with a high rate of mortality. These neonates may present with respiratory distress and an erythematous papular rash.

Listeria - an uncommon cause of neonatal infection, with mortality ranging from 14% to 56% - immunity against Listeria is dependent on T-cell-mediated immunity and granuloma formation --> pregnant women and other patients with immunosuppression are at increased risk of infection. - pregnant women acquire Listeria via contaminated food such as ready-to-eat deli meat, unpasteurized milk, soft cheese, ice cream, or frozen fruits and vegetables - affected pregnant women may report a flu-like illness with fever before delivery - up to 22% of affected pregnancies will result in a stillbirth - more than 70% of women with Listeria deliver before 35 weeks' gestation - green or brown-stained amniotic fluid is often noted with Listeria infection - affected neonates may present with respiratory distress because of pneumonia; chest radiographic findings show bilateral streaky infiltrates or a miliary pattern

A 12 yr old boy complains of pain and swelling in his right upper arm with onset 2 wk previously. He has also had night pain. He has had fever with temperatures to 101°C for the past 2 days.Radiography of the humerus shows periosteal elevation along the shaft. The most appropriate next step is:

MRI of humerus - diagnosis of Ewing sarcoma should be suspected in a patient who presents with pain and swelling, with or without systemic symptoms, and with a radiographic appearance of a primarily lytic bone lesion with periosteal reaction, the characteristic onion-skinning - large associated soft tissue mass often is visualized on MRI or CT - MRI of the tumor and the entire length of involved bone should be performed to determine the exact extension of the soft tissue and bony mass and the proximity of tumor to neurovascular structures - thorough evaluation for metastatic disease includes CT of the chest, radionuclide bone scan, and bone marrow aspiration and biopsy specimens from at least two sites

A 7 yr old child with a 3-yr history of cough, intermittent wheezing, and poor growth has 2 sweat chloride values of 36 and 41 mEq/L. Additional diagnostic testing to rule out cystic fibrosis should include:

Nasal potential difference measurement Diagnosis of CF has been based on a positive quantitative sweat test (Cl− ≥ 60 mEq/L) in conjunction with 1 or more of the following features: - typical chronic obstructive pulmonary disease - documented exocrine pancreatic insufficiency - positive family history. - More than 60 mEq/L of chloride in sweat is diagnostic of CF when 1 or more other criteria are present. Threshold levels of 30-40 mEq/L for infants have been suggested. Borderline (or intermediate) values of 40 to 60 mEq/L have been reported in patients of all ages who have CF with atypical involvement and require further testing. - The finding of increased potential differences across nasal epithelium, the loss of this difference with topical amiloride application, and the absence of a voltage response to a β-adrenergic agonist have been used to confirm the diagnosis of CF in patients with equivocal or frankly normal sweat chloride values. - An alternative test is DNA testing; the patient must have two mutations identified (over 1500 mutations are associated with CF, one of which is ΔF508 mutation).

A 2 yr old boy is seen for his routine check-up, 4 weeks after an episode of bilateral acute otitis media that resolved uneventfully with antibiotic treatment. He seems generally well, but his mother reports that he is not hearing as well as usual. On pneumatic otoscopy, both his eardrums appear amber, opaque, and retracted, and both are immobile. Otherwise his ENT examination is unremarkable. Which of the following treatment regimens is the most appropriate to institute at this time?

Observation without treatment for at least 2 more mo - In OME, bulging of the tympanic membrane is absent or slight or the membrane may be retracted; erythema also is absent or slight but may increase with crying or with superficial trauma to the external auditory canal incurred in clearing the canal of cerumen. - In children with MEE but without tympanic membrane fullness or bulging, the presence of unequivocal ear pain is usually indicative of AOM. - Commonly, both before and after episodes of OM and also in the absence of otitis media, the tympanic membrane may be retracted as a consequence of negative middle ear air pressure. - To distinguish between persistence and recurrence, examination should be conducted monthly until resolution; hearing should be assessed if effusion has been present for > 3 mo. - Most cases of OME resolve without treatment within 3 mo. - When MEE persists longer than 3 mo, consideration of surgical management with tympanostomy tubes is appropriate. - Antimicrobials have some efficacy in resolving OME, presumably because they help eradicate nasopharyngeal infection or unapparent middle ear infection or both. However, mainly because of the short-term nature of their benefit and because of the contribution of antimicrobial usage to the development of bacterial resistance, routine antimicrobial treatment of OME is generally not recommended.

A 17-year-old African-American girl with past medical history of chronic kidney disease due to focal segmental glomerulosclerosis presents to the emergency department with fatigue, blurry vision, polyuria, and weight loss for the past two weeks. She denies any upper respiratory symptoms or fevers. She has received a kidney transplant six months ago and her chronic medication regimen includes oral tacrolimus. Based on this history, what is your next step in management?

Obtain fingerstick glucose - The girl in this vignette is likely experiencing glucose intolerance secondary to tacrolimus toxicity. Therefore, it is important to obtain a fingerstick glucose to rule out hyperglycemia. - Up to one-third of nondiabetic kidney transplant recipients develop impaired glucose tolerance by six months post-transplantation. Moreover, the risk of diabetes after transplant is increased in patients who are obese, African-American, Hispanic, or have a family history of diabetes mellitus. That risk is further increased with glucocorticoid and calcineurin inhibitor use for immunosuppression. There is significantly higher incidence of diabetes mellitus in patients receiving tacrolimus compared with cyclosporine due to the more toxic effect of tacrolimus on pancreatic islet cells. It is vital to diagnose and control diabetes after transplantation as it can negatively impact allograft survival. - All transplant recipients should have a fasting blood glucose measured weekly during the first four weeks, at three and six months, and then yearly. Other adverse effects of tacrolimus are nephrotoxicity, bone marrow suppression, opportunistic infections, hepatotoxicity, and neurotoxicity.

A 4-year-old boy presents to the clinic to establish care. His mother is concerned that he has hearing difficulty because he doesn't turn readily when spoken to and has difficulty following directions in preschool. She does not have any prior records with her and can't recall the results of his newborn hearing screen. Physical exam is unremarkable for otitis media but his tympanic membranes appear retracted and scarred. He is referred to audiology who finds that he has a type B, flat tympanogram. What is the next best step in management?

Referral for tympanostomy tubes type B, flat tympanogram = highest probability of the presence of middle-ear effusion or tympanic membrane perforation as compared to type A and type C tympanograms. - type A tympanogram signifies normal mobility of the eardrum and conduction bones while a type C tympanogram may suggest conductive hearing loss but is not as precise as a type B tympanogram.

A 4 yr old child with a history of poor sucking and swallowing as an infant recently experienced excessive sweating and blotchy erythema, especially when excited. Walking is clumsy. There has been new onset of episodes of cyclic vomiting lasting 24-27 hr, with retching and vomiting every 15-20 min as well as profuse sweating, blotchy erythema, and evidence of apprehension and irritability. The most likely diagnosis is:

Riley-Day Syndrome / Familial dysautonomia - expressed in infancy by poor sucking and swallowing - aspiration pneumonia can occur - after 3 yr of age, autonomic crises begin, usually with attacks of cyclic vomiting lasting 24-72 hr or even several days - retching and vomiting occur every 15-20 min and are associated with hypertension, profuse sweating, blotching of the skin, apprehension, and irritability - prominent gastric distention can occur, causing abdominal pain and even respiratory distress - hematemesis can complicate pernicious vomiting

Over a 6-hr period, 4 children who attend the same elementary school present to the emergency department with vomiting, double vision, slurred speech, fatigued appearance, and weakness of the neck and arms, but no fever. Laboratory results include normal results on complete blood cell count and spinal fluid analysis. Their parents report that other classmates are having similar symptoms, as are a parent and a teacher who chaperoned the class on its field trip yesterday to the state capitol building. You suspect that these illnesses represent an outbreak from either natural occurrence or bioterrorism. Which of the following steps should be taken immediately?

Telephone the local or state health department to report a possible outbreak of botulism - In older children with food-borne or wound botulism, the onset of neurologic symptoms follows a characteristic pattern of diplopia, blurred vision, ptosis, dry mouth, dysphagia, dysphonia, and dysarthria, with decreased gag and corneal reflexes. - Importantly, because the toxin acts only on motor nerves, paresthesias are not seen in botulism, except when a patient hyperventilates from anxiety. - The classic triad of botulism is the acute onset of a symmetric flaccid descending paralysis with clear sensorium, no fever, and no paresthesias. - Suspected botulism represents a medical and public health emergency that is immediately reportable by telephone in most U.S. health jurisdictions. - State health departments (1st call) and the U.S. Centers for Disease Control and Prevention (CDC; telephone 770-488-7100 at any time) can arrange for diagnostic testing, epidemiologic investigation, and provision of equine antitoxin.

The clinical presentation and cerebrospinal fluid (CSF) findings of the adolescent described in the vignette are highly suggestive of aseptic meningitis or meningoencephalitis. Given his recent mosquito bites while hiking in New York, the most likely etiology of his illness is West Nile virus (WNV). The diagnosis is generally made by

detection of WNV IgM antibody in the CSF or blood - The incubation period for WNV illness is 2 to 6 days. - Most individuals with WNV infection are asymptomatic. Symptomatic illness is characterized by fever, headache, eye pain, gastrointestinal symptoms, and fatigue. -The duration of acute illness is 3 to 5 days. - During convalescence, individuals may develop a rash, which starts on the trunk and may extend to the extremities and face. - Neuroinvasive disease occurs in less than 1% of infected individuals. West Nile virus can cause a wide spectrum of neuroinvasive clinical syndromes including aseptic meningitis, encephalitis, acute flaccid myelitis (causing a polio-like illness) and rarely, Guillain-Barré syndrome or movement disorder. Other unusual manifestations of WNV disease include hepatitis, cardiac dysrhythmias, myocarditis, rhabdomyolysis, and optic neuritis.

An 11-year-old boy of Japanese ancestry presents to the office for a hospital follow-up after recovering from bacterial meningitis. Neisseria meningitidis grew in the culture obtained from his cerebrospinal fluid studies. He has had two previous episodes of Neisseria meningitis but has otherwise been healthy. He is up to date on his vaccinations. You suspect he has a terminal complement deficiency. What is the most appropriate initial test to order in the workup of this disorder?

Total hemolytic complement assay - multiple infections with Neisseria meningitidis is suspicious for a deficiency in the membrane attack complex. - most appropriate initial test to order for the workup of a deficiency in the classical pathway is a total hemolytic complement (THC or CH50) assay. - this test uses sheep erythrocytes coated with rabbit antibodies that bind to antigens on the erythrocyte membrane. The sheep erythrocytes are then mixed with the patient's serum, and the results indicate how many of the sheep erythrocytes are lysed. A result of 150 to 250 units/mL is normal, meaning approximately 50% of the sheep erythrocytes were lysed in the patient's serum. - A low or undetectable total hemolytic complement assay result should be repeated, and if the result is low again, measurement of specific complement proteins should be performed, as this is indicative of a complement deficiency. - Individuals with a heterozygous complement deficiency are usually healthy and will usually have a normal total hemolytic complement assay, while a homozygous deficiency will have an undetectable level. - The exception to this is a C9 deficiency, which will usually have very low levels of total hemolytic complement. The membrane attack complex includes C5, C6, C7, C8, and C9 proteins. A deficiency in the membrane attack complex increases risk of recurrent Neisseria meningitidis infections, as well as infections by other Neisseria species.

A 6-year-old girl presents to the pediatrician's office one week after completing a course of oral amoxicillin for streptococcal pharyngitis. She has previously been treated with amoxicillin for an otitis media and tolerated it well. Her mother brought her in today because she has developed a rash over her trunk, arms, and legs. She complains that the rash is itchy but otherwise she is asymptomatic and well-appearing. Physical exam reveals a morbilliform maculopapular blanching rash across her trunk and both arms. There is no blistering. There is no involvement of mucus membranes. Which of the following is the most appropriate next step in management?

Treat with topical steroids and oral antihistamines until the rash resolves - maculopapular rash that is due to a delayed type IV hypersensitivity reaction to the amoxicillin. - Type IV reactions such as the rash experienced by the child in this scenario are T cell mediated. - The child in this scenario has a mild form of type IV hypersensitivity reaction characterized by morbilliform skin eruption. These reactions can typically be treated symptomatically with topical steroids and oral antihistamines until the rash resolves.

A 2 yr old child who has completed 8 days of a 10-day course of cefaclor presents with low-grade fever, malaise, irritability, lymphadenopathy, and a generalized erythematous rash that is mildly pruritic. The most likely diagnosis is:

Type III hypersensitivity reaction Serum sickness - classic example of a type III hypersensitivity reaction caused by antigen-antibody complexes - attributed to drug allergy, triggered in particular by antibiotics (e.g., cefaclor) - serum sickness-like reactions from drugs are characterized by fever, pruritus, urticaria, and arthralgias that usually begin 1-3 wk after drug exposure - urticarial skin eruption becomes increasingly erythematous as the reaction progresses and can evolve into dusky centers with round plaques

Currently the best charts available for children < 2 years of age, international populations that do not have more specific data for their geographical area, or potentially for immigrant children as these are international growth charts.

WHO standards - The CDC recommends using the WHO charts for children 0-2 years of age, and then switching to the CDC charts for children in the United States after age 2.

A 7 yr old boy, previously an "A" student, demonstrates progressive hyperactivity and is diagnosed with attention-deficit/hyperactivity disorder. Despite therapy, his school performance deteriorates. He has difficulty understanding people when spoken to on the telephone. Seizures develop 6 mo later. Physical examination reveals slight diffuse hyperpigmentation, spasticity, and reduced deep tendon reflexes. The most likely diagnosis is:

X-linked adrenoleukodystrophy - symptoms are first noted most commonly between the ages of 4 and 8 yr - most common initial manifestations are hyperactivity, which often is mistaken for an attention-deficit disorder, and worsening school performance in a child who had previously been a good student - auditory discrimination is often impaired, although tone perception is preserved - may be evidenced by difficulty in using the telephone and greatly impaired performance on intelligence tests in items that are presented verbally - spatial orientation is often impaired - other initial symptoms are disturbances of vision, ataxia, poor handwriting, seizures, and strabismus - visual disturbances are often due to involvement of the cerebral cortex, which leads to variable and seemingly inconsistent visual capacity - seizures occur in nearly all patients and may represent the 1st manifestation of the disease - impaired cortisol response to ACTH stimulation is present in 85% of patients, and mild hyperpigmentation is noted - in most patients with this phenotype, adrenal dysfunction is recognized only after the condition is diagnosed because of the cerebral symptoms

A 1 yr old child with recurrent sinusitis is found to have a normal WBC count but no circulating B cells, small tonsils, and no palpable lymph nodes. Serum concentrations of IgG, IgA, IgM, and IgE are below the 95% lower limits for age. The most likely diagnosis is:

X-linked agammaglobulinemia (XLA) - Most boys afflicted with XLA remain well during the 1st 69 mo of life by virtue of maternally transmitted IgG antibodies. Thereafter, they acquire infections with extracellular pyogenic organisms, such as Streptococcus pneumoniae and Haemophilus influenzae, unless they are given prophylactic antibiotics or immunoglobulin therapy. - Infections include sinusitis, otitis media, pneumonia, or, less often, sepsis or meningitis. Infections with Mycoplasma also are particularly problematic. - The diagnosis of XLA should be suspected if lymphoid hypoplasia is found on physical examination (minimal or no tonsillar tissue and no palpable lymph nodes), and serum concentrations of IgG, IgA, IgM, and IgE are far below the 95% confidence limits for appropriate age and race matched controls, usually with total immunoglobulins < 100 mg/dL.

A 15 yr old boy with a 12-yr history of microscopic hematuria is noted to have bilateral high-frequency sensorineural hearing loss, blood pressure of 140/90 mm Hg, serum creatinine of 1.5 mg/dL, and urinary protein of 2,000 mg/24 hr. This patient's mother also has microscopic hematuria. The most likely mode of inheritance for this child's glomerular disease is:

X-linked dominant Alport syndrome (AS), hereditary nephritis - genetically heterogeneous disease caused by mutations in the genes coding for type IV collagen, a major component of basement membranes - 85% of patients have X-linked disease caused by a mutation in the COL4A5 gene encoding the α5 chain of type IV collagen - bilateral sensorineural hearing loss, which is never congenital, occurs in 90% of hemizygous males with X-linked AS, 10% of heterozygous females with X-linked AS, and 67% of patients with autosomal recessive AS. This deficit begins in the high-frequency range but progresses to involve conversational speech, prompting the need for hearing aids.

The parents of a 26 month old child who had been following Dr. Sears's Alternative Immunization Schedule brings their child in a panic because of a measles outbreak in their community, though no known cases in the child's daycare or history of direct exposure to a case yet. The child received mumps at age 12 months, and rubella at age 24 months. There is no TB risk in the family. There is no more supply of monovalent measles vaccine available. The parents ask whether you can give their child any measles vaccine. You respond:

Yes, MMR can be given - MMR can be given in an outbreak setting to children as young as 6 months of age. Even had MMR first dose had been given at age 12 months, a second dose could have been given as early as 28 days later. Immune globulin can be given to prevent measles in a susceptible person within 6 days of exposure, though in this case, there was no history of close contact or direct exposure.

Three months following allograft renal transplantation, a 4-year-old boy develops fever and right flank tenderness. Laboratory findings show a sharp elevation from his baseline creatinine. A renal biopsy is performed that reveals significant interstitial inflammation and tubulitis. Which of the following is the most likely cause of his findings?

acute graft rejection - significant deterioration in allograft function along with certain pathologic changes on renal biopsy. - Pediatricians should consider graft rejection in the differential diagnoses in a patient with fever and a recent renal transplant. - Symptoms of rejection include fever, malaise, oliguria, hypertension, and graft-site tenderness; however, the patient can be asymptomatic. - Laboratory findings may demonstrate an acute rise of the patient's creatinine from baseline, pyuria, or proteinuria. - Since many patients are asymptomatic, sometimes the only finding is a rise in the serum creatinine. - Definitive diagnosis for renal allograft rejection is made by renal biopsy, which can distinguish between T-cell mediated rejection and antibody-mediated rejection. - occurs when T-cells attack donor histocompatibility antigens on renal graft tubules, interstitium, vessels, and glomeruli.

A 3 yr old boy presents to your office with sudden onset of cola-colored urine, progressive facial swelling over the past 3 days, and decreased urine volume over the past day. His examination is notable for blood pressure 130/80 mm Hg, periorbital edema, bibasilar rales, and ankle swelling. His urinalysis is remarkable for 3+ hematuria, 1+ proteinuria, 100 red blood cells per high-power field, and red blood cell casts. His serum electrolytes are normal and the serum albumin is 3.2 g/liter. This clinical presentation is most consistent with:

acute nephritic syndrome - Tea- or cola-colored urine, facial or body edema, hypertension, and oliguria are classic symptoms of acute nephritic syndrome. - Diseases commonly manifesting as acute nephritic syndrome include postinfectious glomerulonephritis, immunoglobulin A (IgA) nephropathy, membranoproliferative glomerulonephritis, Henoch-Schönlein purpura (HSP) nephritis, systemic lupus erythematosus (SLE) nephritis, Wegener granulomatosis, microscopic polyarteritis nodosa, Goodpasture syndrome, and hemolytic-uremic syndrome. - A history of recent upper respiratory, skin, or gastrointestinal infection suggests postinfectious glomerulonephritis, hemolytic-uremic syndrome, or HSP nephritis. - Rash and joint complaints suggest HSP nephritis or SLE nephritis.

A patient comes in with an acute asthma exacerbation and is tachypneic and wheezing. Their VBG results are: pH 7.45 PCO2 33 HCO3 22. How would you classify their acid base status?

acute respiratory alkalosis - Your patient is in respiratory distress and is tachypneic. They have increased their alveolar minute ventilation and are in an acute respiratory alkalosis, as the pH is high and the PCO2 is low with a bicarbonate level that is essentially normal.

A 17-year-old adolescent girl is seen for a health supervision visit. Her medical history is significant for a diagnosis of acute T-lymphoblastic leukemia at 12 years of age. She was treated with chemotherapy and 1,800-cGy cranial irradiation. She completed all treatment 2 years ago and remains in complete remission. The importance of healthy eating, particularly given the treatment she received, is discussed. Of the following, the BEST description of how she compares with her peers is that she has

an increased risk of experiencing metabolic syndrome - Survivors of childhood cancer, especially those who received cranial radiation therapy, are at increased risk of developing metabolic syndrome. - A significant majority of survivors of childhood cancer have a chronic health condition as a consequence of the disease or therapy they received. - Metabolic syndrome is a disease state characterized by overweight, hypertension, hyperlipidemia, and hyperglycemia.

A previously healthy 7 yr old girl living in rural South Carolina presents with a 1-wk history of fever, cough, and shortness of breath. Her temperature is 37.5°C, pulse 80/min, respiratory rate 30 breaths/min, and blood pressure 95/60 mm Hg. Chest film reveals bilateral pulmonary infiltrates. The complete blood cell count is normal except for eosinophilia. Which of the following organisms is the most likely cause of the patient's illness?

ascaris lumbricoides - Approximately 1 billion persons are estimated to be infected, with 4 million cases in the USA. - Key factors linked with a higher prevalence of infection include poor socioeconomic conditions, use of human feces as fertilizer, and geophagia. - Even though infection can occur at any age, the highest rate is in preschool or early school-aged children. - The most common clinical problems are due to pulmonary disease and obstruction of the intestinal or biliary tract. - Larvae migrating through these tissues may cause allergic symptoms, fever, urticaria, and granulomatous disease. - The pulmonary manifestations resemble Loeffler syndrome and include transient respiratory symptoms such as cough and dyspnea, pulmonary infiltrates, and blood eosinophilia. - Larvae may be observed in the sputum. - Vague abdominal complaints have been attributed to the presence of adult worms in the small intestine, although the precise contribution of the parasite to these symptoms is difficult to ascertain. - A more serious complication occurs when a large mass of worms leads to acute bowel obstruction.

A 2 yr old girl presents with high, remittent fever of 9 days' duration associated with marked irritability, nonpurulent conjunctival injection, red cracked lips, swollen and erythematous hands and feet, and a maculopapular erythematous rash on the trunk and extremities. Laboratory testing at presentation would most likely show evidence of:

aseptic meningitis - Associated symptoms other than the clinical criteria are common in the 10 days prior to diagnosis of KD. - Gastrointestinal symptoms (vomiting, diarrhea, or abdominal pain) occur in almost 65% of patients, and respiratory symptoms (interstitial infiltrates, effusions) occur in 30%. - Other clinical findings include significant irritability that is especially prominent in infants and likely due to aseptic meningitis, mild hepatitis, hydrops of the gallbladder, urethritis and meatitis with sterile pyuria, and arthritis.

A 17 yr old boy with cystic fibrosis develops recurrent bronchospasm and transient pulmonary infiltrates. Laboratory testing reveals mycelia in the sputum and a peripheral eosinophilia. The most likely causative organism is:

aspergillus Allergic bronchopulmonary aspergillosis (ABPA) - hypersensitivity disease resulting from immunologic sensitization to Aspergillus antigens - primarily seen in patients with asthma or cystic fibrosis 7 primary diagnostic criteria for ABPA: - episodic bronchial obstruction - peripheral eosinophilia - immediate cutaneous reactivity to Aspergillus antigens - precipitating antibodies to Aspergillus antigen - elevated IgE, pulmonary infiltrates - central bronchietasis Secondary diagnostic criteria include - repeated detection of Aspergillus from sputum by identification of morphologically consistent fungal elements or direct culture - coughing up brown plugs or specks - elevated Aspergillus antigen-specific immunoglobulin E (IgE) antibodies - late skin reaction to Aspergillus antigen Radiologically, bronchial wall thickening, pulmonary infiltrates, and central bronchiectasis can be seen

A 16-year-old adolescent girl is having a health supervision evaluation. She has bipolar disorder, and at the time of her last visit, was being treated with aripiprazole by her psychiatrist. She discontinued this medication 6 months ago, because she did not like the adverse effects (insomnia and weight gain). Instead, she has been smoking marijuana 3 times per day to manage her symptoms. Her mother is aware of her marijuana use and is supportive of her daughter using a "natural" plant-based drug to control her mood, as opposed to a psychiatric medication. The patient was recently caught with marijuana at school and expelled. She is currently enrolled in an alternative school, but has not been attending regularly because of her marijuana smoking schedule. She plans to start homeschooling and online courses. The counselor at her former school filed a report with child protective services, and the patient and her mother have been interviewed by a caseworker. The case is currently open, but the family expects it will soon be closed. The mother agrees that her daughter's marijuana use is having a negative impact on her education, but is not sure how to help her. This patient's practice of self medication with marijuana is a concern for the primary care physician. Of the following, the BEST next step in this adolescent's management is to

assess the mother's readiness for change, including the possibility of signing her daughter into a drug rehabilitation program without her assent - The adolescent girl in the vignette is self-treating her bipolar disorder and has signs of cannabis use disorder based on the frequency of daily use, failure to attend school, and continued use despite the negative effects it has had on her life. She would benefit from a drug rehabilitation program to address her substance use, but may need parental intervention if she is not willing to go on her own and believes she does not have a problem.

A 15 yo M is admitted to the PICU with a diagnosis of meningococcemia after presenting with fever, altered mental status, hypotension, and purpura. He is started on ceftriaxone and vancomycin, receives 60 cc/kg of NS as boluses, and a R IJ line is placed. The nurse calls you and states that he is oozing from his IV sites and the R IJ line. You suspect that he has DIC and want to send labs to evaluate. Which of the following labs is the most sensitive for DIC?

decreased platelet count - thrombocytopenia is the most sensitive indicator for DIC. - Thrombocytopenia was followed by increased fibrin degradation products, prolonged PT, prolonged aPTT, and low fibrinogen in terms of sensitivity for DIC.

A 2-week old neonate is having a health supervision evaluation. He was born at term to first-time parents. Prenatal ultrasonography revealed shortened femur length and macrocephaly; postnatal evaluation confirmed the diagnosis of achondroplasia. The boy's parents wish to know what the typical pattern of development is in children with this diagnosis. Of the following, the MOST accurate statement is that children with this diagnosis typically have

delayed gross motor dev't - Egross motor delays typically exist because of atypical physical proportions; delays resolve by age 2 to 3 years. - Children with achondroplasia are at increased risk of developing otitis media, obstructive sleep apnea, obesity, chronic joint pain, and cervical medullary compression.

Which of the following is the inheritance pattern of the most common forms of spinal muscular atrophy?

autosomal recessive Spinal muscular atrophy is - diffuse, symmetric muscle weakness and atrophy that is more pronounced in the lower limbs. - Deep tendon reflexes are significantly decreased or absent. - degeneration of the spinal cord's anterior horn cells and the motor nuclei in the lower brainstem. - The most common types of spinal muscular atrophy result from mutations on chromosome 5q. - These 5q-related spinal muscular atrophies are inherited in an autosomal recessive pattern. - There are five phenotypes of spinal muscular atrophy, with types 0 and 1 being the most severe. Type 0 presents at birth with respiratory failure by age 6 months. - Type 1 presents before age 6 months with respiratory failure by age 2 years. - Severity decreases and age of onset increases with each subsequent type, with type 4 being the mildest form and having adult onset. - Treatment is mostly directed toward supportive care, with nutrition and respiratory assistance. - Nusinersen, a recently-studied oligonucleotide that has been shown to improve motor milestone achievement in a percentage of affected patients, may also be considered for treatment.

Child has sustained a subluxation of the radial head, colloquially known as a "nursemaid's elbow." The typical mechanism for a radial head subluxation is

axial traction on an extended elbow while the forearm is pronated - Reduction of radial head subluxation can be achieved via either hyperpronation of the forearm or supination of the forearm and flexion of the elbow

What is the recommended antibiotic for treatment and prophylaxis of Bordetella pertussis in a 2 wk old infant?

azithromycin - Azithromycin is the preferred agent for most patients and particularly in neonates, although cases of IHPS have followed its use. - All infants < 1 mo of age treated with any macrolide should be monitored for symptoms of pyloric stenosis.

A 17-year-old girl presents to clinic with two weeks of a persistent, nonproductive cough. She otherwise feels well. Physical exam reveals an afebrile, well-appearing girl with scattered crackles and wheezes on lung auscultation. She has no prior history of asthma or wheezing. Initiation of which of the following medications is the best choice for treatment?

azithromycin Mycoplasma pneumoniae infection. - symptoms typically begin with headache, malaise, and low-grade fever - more than 75% develop a persistent, nonproductive cough. - Lung exam may reveal scattered crackles or wheezing, but many patients have normal findings. - Recommended treatment for atypical pneumonia is a macrolide, such as azithromycin. However, if resistance is suspected, doxycycline or a fluoroquinolone should be considered.

Patients with latex allergy may also experience food-allergic reactions from homologous proteins in foods such as ...

bananas, kiwi, avocado, chestnut, and passion fruit

scaphoid abdomen and respiratory distress should alert one to the possibility of...

diaphragmatic hernia

A 10-month-old male infant is seen for follow-up. He was diagnosed with bilateral acute otitis media 3 days ago after developing fever and otalgia with abnormal tympanic membrane findings. He was prescribed a 10-day course of high-dose amoxicillin and ibuprofen as needed. He has received 6 doses of amoxicillin, but the fever persists despite administration of ibuprofen every 6 hours. He has had a poor appetite and a few episodes of nonbloody, nonbilious emesis. His parents have also noted that his eyes seem swollen and he is urinating less frequently. His history includes a previous history of otitis media treated successfully with amoxicillin at 4 months of age. He appears to be nontoxic and well hydrated. His weight and length are in the 75th percentile, and his blood pressure is in the 87th percentile. There is mild periorbital edema without conjunctival or scleral injection and no discharge. Other significant findings include erythematous, opaque, and bulging tympanic membranes bilaterally. The remainder of the physical examination findings are within normal limits. Laboratory findings reveal sterile pyuria, proteinuria, hematuria, and elevated levels of blood urea nitrogen and creatinine. Findings of a urine culture are negative. Of the following, the BEST initial step in management is to

discontinue ibuprofen Acute interstitial nephritis (AIN) caused by the nonsteroidal anti-inflammatory drug (NSAID) ibuprofen, which can result in acute kidney dysfunction - Acute kidney injury induced by NSAIDs may be hemodynamically mediated or secondary to AIN - Nonsteroidal anti-inflammatory drugs lead to a decrease in glomerular filtration rate by causing reversible renal ischemia, even more so in a setting of decreased intravascular volume (dehydration, vomiting, and diarrhea) - The child in the vignette has decreased appetite and vomiting, and is at risk of experiencing acute kidney injury secondary to NSAID-induced renal vasoconstriction. However, the presence of hematuria, proteinuria, and sterile pyuria favors a diagnosis of AIN. - Acute interstitial nephritis should be included in the differential for sterile pyuria. - The mainstay of treatment for drug-induced AIN is discontinuation of the offending agent, which in this case is ibuprofen. The majority of patients improve after the offending medication is withdrawn. - Nonsteroidal anti-inflammatory drug-induced AIN does not typically respond to glucocorticoid therapy.

A 16-year-old boy who has recently undergone chemotherapy induction therapy for Burkitt lymphoma presents to the emergency department due to new-onset fever that began this morning. Vital signs reveal a temperature of 38.4°C and a normal blood pressure. Capillary refill time is three seconds. Aside from some nasal congestion and fatigue, he has a negative review of systems. He has no altered mental status, headache, photophobia, or nuchal rigidity on exam, and a neurological exam is normal. His respiratory exam is normal. His chemotherapy port has no surrounding erythema or drainage. A complete blood count with differential reveals an absolute neutrophil count of 100 cells/microL. Which of the following is indicated in this patient's management?

blood culture - febrile patients with chemotherapy-induced neutropenia, such as this patient, it is important to obtain blood cultures as soon as possible - this patient has an indwelling central line, a blood culture should be drawn from each lumen of the port, if possible. Peripheral blood cultures may be drawn as well. - Blood cultures should be obtained daily for two days after the fever remains in the setting of antibiotic therapy, and repeated if the patient's clinical status changes or the fever recurs after an afebrile period - minimum workup for a patient such as this should also include a complete blood count with differential, electrolytes, creatinine, blood urea nitrogen, liver transaminases, and total bilirubin. - Additional workup may be clinically indicated, depending on the individual case. For example, a urine culture may be indicated in female patients or patients who cannot reliably relay signs or symptoms of a urinary tract infection.

A 5-year-old boy presents to clinic for evaluation of fever for six days. He recently returned from a family trip to India to visit relatives and did not receive pretravel vaccinations but is otherwise up to date on childhood vaccinations. Vital signs show a temperature of 38.5°C, respiratory rate of 32 breaths/min, heart rate of 120 beats/min, and blood pressure of 108/72 mm Hg. On physical examination, he has abdominal tenderness and hepatosplenomegaly, and no jaundice is appreciated. Laboratory data are shown: White blood cell count: 18 000/microL Hemoglobin: 9.4 g/dL Segmented neutrophils: 80% Lymphocytes: 20% Aspartate aminotransferase: 220 U/L Alanine aminotransferase: 270 U/L What is the best test to make the diagnosis?

blood culture Typhoid fever - caused by the gram-negative bacterium Salmonella enterica serotype Typhi (as well as the Paratyphi serotypes). - should be considered when there is a history of travel to a country where typhoid is endemic. - fever and chills followed by abdominal pain. - Hepatosplenomegaly and a salmon-colored macular rash ("rose spots") may be appreciated - anemia, leukopenia or leukocytosis, and elevated liver transaminases. - Blood culture is the best test to make the diagnosis of typhoid fever, however, antibiotics should be started based on clinical presentation, as blood culture has low yield and should not be relied on. - may lead to intestinal perforation and septic shock if left untreated, though more commonly it gradually self-resolves over weeks to months.

A 4 wk old infant has the history of gagging and gasping episodes of acute onset, with mild cough and rhinorrhea. The infant is afebrile and appears well, and findings on lung auscultation are normal. An episode of apnea is witnessed. Complete blood cell count reveals hemoglobin 12.0 g/dL, WBC count 42,000/mm3 (10% neutrophils, 3% monocytes, 87% lymphocytes), and platelet count 650,000/mm3. The most likely etiologic agent of this illness is:

bordetella pertussis - Infants < 3 mo of age do not display the classic stages. - The catarrhal phase lasts only a few days or is unnoticed, and then, after the most insignificant startle from a draft, light, sound, sucking, or stretching, a well-appearing young infant begins to choke, gasp, gag, and flail the extremities, with face reddened. - Cough may not be prominent, especially in the early phase. - Whoop infrequently occurs in infants < 3 mo of age who at the end of a paroxysm lack stature or muscular strength to create sudden negative intrathoracic pressure. - Apnea and cyanosis can follow a coughing paroxysm, or apnea can occur without a cough. Apnea may be the only symptom. - Leukocytosis (15,000-100,000 cells/mm3) due to absolute lymphocytosis is characteristic in the catarrhal stage.

A 3 yr old girl has a 2-wk history of fever associated with bifrontal headache, lethargy, and vomiting. She has a history of perioral cyanosis and dyspnea with exertion beginning in infancy. She suddenly has a 10-min focal tonic-clonic seizure. The child is obtunded and has a temperature of 100.8°F (38.2°C), pulse of 118 beats/min, and blood pressure of 96/70 mm Hg in her right arm, measured while she is supine. Perioral cyanosis is noted at rest. A harsh pansystolic murmur is heard best along the left sternal border. Examination of her eye grounds reveals bilateral papilledema. She has right-sided weakness associated with hyperreflexia and an extensor plantar reflex. The most likely cause of the hemiparesis is:

brain abscess - can occur in children of any age but are most common in children between 4 and 8 yr and neonates - early stages of cerebritis and abscess formation are associated with nonspecific symptoms, including low-grade fever, headache, and lethargy - as the inflammatory process proceeds, vomiting, severe headache, seizures, papilledema, focal neurologic signs (hemiparesis), and coma may develop

A 2-year-old girl undergoing chemotherapy for acute lymphocytic leukemia presents to the emergency department with her parents who express concerns that she had a fever at home. The patient has a temperature of 38.4°C and is mildly tachycardic but is otherwise stable with normal blood pressure and peripheral perfusion, and the rest of her exam is normal. A complete blood count with differential reveals an absolute neutrophil count of 380 cells/microL. Of the following, which is the most appropriate empiric therapy?

cefepime - In a febrile pediatric cancer patient, neutropenia is defined as an absolute neutrophil count of fewer than 500 cells/microL. - Fever in a neutropenic patient is defined as a single temperature of 38.3°C or higher, a temperature of 38°C or higher for more than one hour, or two measurements of temperature greater than 38°C in a 12-hour period. - Patients with febrile neutropenia are categorized as either high risk or low risk for severe infection. - Patients considered to be at high risk include those with neutropenia expected to last more than seven days, medical comorbidities, and hepatic or renal insufficiency. - The most common infections identified in children with chemotherapy-induced neutropenia and fever are usually bloodstream infections, the majority of which are caused by gram-positive organisms, so it is important to cover these patients with antibiotic therapy appropriately. - Piperacillin-tazobactam, meropenem, or cefepime are options for empiric antibiotic monotherapy for these high-risk patients.

A 10-year-old girl with type 1 diabetes is seen for decreased appetite and fatigue over the past month. She eats three meals per day, but she consumes small portions because of nausea. She has not had abdominal pain, vomiting, diarrhea, constipation, or recent illness. Her diabetes is treated with rapid-acting insulin via an insulin pump. She has had a 2.3-kg weight loss since her last health supervision visit 4 months ago. Her vital signs and the remainder of her physical examination findings are normal. Her most recent hemoglobin A1c level was 7.9% 2 months ago. Today, her hemoglobin level is 10 g/dL (100 g/L) with a mean corpuscular volume of 72 fL. Of the following, the MOST likely underlying diagnosis causing this girl's symptoms is

celiac disease - Autoimmune thyroid disease, usually hypothyroidism, is the autoimmune condition most commonly associated with type 1 diabetes. - Celiac disease is the autoimmune condition second most commonly associated with type 1 diabetes. - Guidelines recommend routine screening for thyroid disease and celiac disease in patients with type 1 diabetes at the time of diagnosis of diabetes and periodically thereafter

An 18 mo old white boy manifests failure to thrive, poor appetite, abdominal distention, diarrhea, and irritability. He had been well until 9 mo of age. Thereafter, he was weaned from breast milk to regular foods. His growth curve is flattening. The most likely diagnosis is:

celiac disease - prolonged breast-feeding has been associated with a reduced incidence of symptomatic disease - intestinal symptoms are common in children whose disease is diagnosed within the 1st 2 years of life - failure to thrive, chronic diarrhea, vomiting, abdominal distention, muscle wasting, anorexia, and irritability are present in most cases.

Boy is experiencing polydipsia and polyuria immediately after undergoing cranial surgery. His laboratory evaluation shows dilute urine and hypernatremia, favoring a diagnosis of

central diabetes insipidus - results from inadequate secretion of ADH from the posterior lobe of the pituitary gland - commonly a result of a primary brain tumor or cranial surgery or injury - in infants and children, other causes of CDI include familial history, Langerhans cell histiocytosis, head trauma, associated brain malformations, and other central nervous system injuries (infectious, vascular, and granulomatous) - In CDI, lack of ADH causes large amounts of free water to be lost from the collecting duct of the kidney, resulting in polyuria. New-onset nocturia can sometimes be the presenting symptom of CDI. - In children with CDI secondary to head trauma or cranial surgery, a triple-phase response is seen: initial intense transient polyuria lasting hours to several days, followed by an antidiuretic period and then by persistent polyuria. - Children old enough to ask for water will try to compensate for the urinary losses with increased thirst and intake of water and typically will have a craving for ice-cold water. - Infants and young children too young to ask for or get free water on their own will develop hypernatremic dehydration. - Untreated CDI can also lead to growth failure in children. - A 24-hour urine collection and intake recording is done to confirm polyuria and polydipsia. Laboratory evaluation in CDI shows low urine specific gravity and low urine osmolality on a first-voided morning specimen. Normal or high serum sodium (hypernatremia) is seen, depending on the child's free water intake. - When the diagnosis is unclear, a water deprivation test is performed to differentiate CDI from nephrogenic diabetes insipidus. Nephrogenic diabetes insipidus results from an inability of the renal tubules to respond to ADH and concentrate the urine despite normal ADH secretion (ADH insensitivity).

The most important extramedullary site of relapse in childhood ALL is:

central nervous system - The 2nd phase of treatment focuses on CNS therapy in an effort to prevent later CNS relapses. - Intrathecal chemotherapy is given repeatedly by lumbar puncture in conjunction with intensive systemic chemotherapy. The likelihood of later CNS relapse is thereby reduced to < 5%. - A small percentage of patients with features that predict a high risk of CNS relapse may receive irradiation to the brain. This includes patients who, at the time of diagnosis, have lymphoblasts in the CSF and either an elevated CSF leukocyte count or physical signs of CNS leukemia, such as cranial nerve palsy.

A 5 yr old boy presents with progressive headache that is associated with nausea and vomiting. Physical examination reveals bilateral papilledema. The most likely diagnosis is:

central nervous system tumor - Signs and symptoms are related to obstruction of cerebrospinal fluid (CSF) drainage paths by the tumor, leading to increased intracranial pressure (ICP) or causing focal brain dysfunction. - Subtle changes in personality, mentation, and speech may precede these classic signs and symptoms; such changes often occur with supratentorial (cortical) lesions. - Infants with open cranial sutures may present with signs of increased ICP, such as vomiting, lethargy, and irritability, as well as the later finding of macrocephaly. - The classic triad headache, nausea, and vomiting as well as papilledema are associated with midline or infratentorial tumors. - Disorders of equilibrium, gait, and coordination occur with infratentorial tumors.

A 14-year-old adolescent boy is brought to the emergency department with a chief concern of fatigue. He states that at the end of the school day he is unable to keep his eyes open. His friends have noted that his eyelids will droop during this time. When he gets home from school, he will close his eyes and rest for about 30 minutes with improvement in his symptoms. On occasion, he has had associated double vision. He has not noted any additional focal neurological deficits. His general physical examination findings are normal. His neurological examination is notable for bilateral ptosis provoked by sustained upward gaze with an otherwise normal cranial nerve examination. His strength is full in all 4 extremities with normal deep tendon reflexes at the biceps, triceps, patella, and ankle bilaterally. Sensation is intact to light touch and pin prick. His coordination and gait are normal. Electromyography with nerve conduction studies demonstrates an electro-decremental response on repetitive nerve stimulation consistent with a neuromuscular junction disorder. Anti-acetylcholine receptor antibodies are positive on serum testing, confirming the diagnosis of myasthenia gravis. Treatment is initiated. Of the following, the MOST appropriate additional screening test is

chest CT - The patient in this vignette with autoimmune myasthenia gravis should be screened with chest computed tomography for thymoma and thymic hyperplasia. Approximately 10% to 30% of adults with myasthenia gravis have thymoma or thymic hyperplasia with increased incidence over the age of 40 years. In the pediatric population, co-occurrence of thymoma is less common; however, thymic hyperplasia is commonly seen on pathologic specimens.

A 15 yr old white girl reports that she has had a fever, weight loss, and night sweats for 3 mo. On physical examination, she is found to have painless swelling of the left cervical and supraclavicular lymph nodes. Her liver and spleen are not enlarged. The nearest pediatric oncology center requires a 4-hr drive. Before transfer, the initial evaluation of the patient should include:

chest radiography - Patients commonly present with painless, nontender, firm, rubbery, cervical or supraclavicular lymphadenopathy and usually some degree of mediastinal involvement. - Clinically detectable hepatosplenomegaly is rarely encountered. - Depending on the extent and location of nodal and extranodal disease, patients may present with symptoms and signs of airway obstruction (dyspnea, hypoxia, cough), pleural or pericardial effusion, hepatocellular dysfunction, or bone marrow infiltration (anemia, neutropenia, or thrombocytopenia). - Disease manifesting below the diaphragm is rare and occurs in approximately 3% of all cases. - Systemic symptoms, classified as B symptoms that are considered important in staging, are unexplained fever > 39°C, weight loss > 10% total body weight over 3 mo, and drenching night sweats. B - efore transporting the patient over a long distance, chest radiography will identify the size of a likely mediastinal mass and possible risk of breathing difficulty.

A 2.9-kg girl is born at term after having carried a prenatal diagnosis of left congenital diaphragmatic hernia since 19 wk of gestation. Delivery and the early postnatal period are uneventful, with mild tachypnea and retractions developing at 24 hr of age. Chest radiograph reveals a normal abdominal gas pattern and multiple lucent areas in the left lower thorax. The most likely diagnosis is:

congenital cystic adenomatoid malformation - Prenatal ultrasonagraphy may suggest congenital diaphragmatic hernia. - After delivery, a chest radiograph is needed to confirm the diagnosis. - In these infants, chest radiograph should show a stomach, nasogastric tube, and small bowel contents in the thoracic cavity. - This infant has a normal abdominal gas pattern, with an abnormal lesion in the left chest. - A CT scan would be required to make the diagnosis.

Cardioversion for infants in ICN....

setting = 0.5 - 2 Joules/kg Mode - synchronous for narrow QRS - asynchronous for v fib

The American Academy of Pediatrics guidelines for the management of otitis media list what abx as acceptable alternatives in the setting of penicillin allergy?

several cephalosporins, including cefdinir - Only 2% of individuals with a penicillin allergy are expected to react to cephalosporins, and less than 1% will react to carbapenems.

How does milrinone affect the end diastolic pressure volume (EDPV) curve?

shifts it to the right Milrinone - PDE3 inhibitor and has the unique property of increasing lusitropy, or diastolic function. - increases the compliance of the LV and thus would shift the EDPV curve to the right- for any given LV volume, there is less LV pressure given the increase in lusitropy and hence, ventricular compliance.

Constitutional delay of puberty is the most common cause of delayed puberty, especially in boys. Constitutional delay of puberty in boys with associated psychosocial distress is an indication for

short course of testosterone - to accelerate pubertal development and promote earlier initiation of the pubertal growth spurt - puberty is considered delayed in boys when there is a lack of testicular growth = 4 mL in volume (or 2.5 cm in length) by age 14 years. - in girls, puberty is delayed when there is a lack of breast development by age 13 years.

A 4-year-old child can gallop and hop on one foot, copy a square, wash her face and hands, draw a four- to six-part person, has 100% intelligible speech, and parallel plays with other children. Based on the description of her skills, which domain of this child's development is delayed?

social and emotional - At age 48 months, a child's social and emotional development allows them to participate in group play and have a preferred friend. - Parallel play begins as early as 24 months, however, by 48 months, the child should have developed interactive play with others. If a child ignores other children, this is considered a red flag at this age, and further investigation should be pursued. - Other red flags at this age include unclear speech or the inability to answer simple questions (language development) and the inability to jump in place (gross motor development). - Normally developing 4-year-olds are also able to brush their teeth, dress themselves, draw recognizable pictures, grasp a pencil with thumb and fingers, and climb stairs by alternating feet.

A diet low in which of the following is recommended as initial treatment for children with nephrogenic diabetes insipidus?

sodium Nephrogenic diabetes insipidus - caused by kidney resistance to antidiuretic hormone. - can be caused by lithium use, hypercalcemia, or other conditions affecting the kidneys. - First-line therapy includes a low-sodium diet and "double voiding" to prevent bladder distension and dysfunction. - Additionally, a thiazide diuretic can significantly improve polyuria in patients who are also on a low-sodium diet, and its use is recommended in children. - Addition of amiloride should also be considered in patients who are still having significant polyuria despite first-line therapy. - Infants and young children should be offered water every two hours, day and night. - Additionally, intravenous hydration is often necessary in children who have extrarenal fluid losses, as in the setting of fever, vomiting, or diarrhea.

Osmolality may be calculated using the serum concentrations of ...

sodium, glucose, and urea - Osmolality = 2 × [Na] + [glucose] ÷ 18 + [BUN] ÷ 2.8. - Glucose and blood urea nitrogen (BUN) are measured in mg/dL. Division of these values by 18 and 2.8, respectively, as shown, converts the units into mmol/L. - Multiplication of the sodium value by 2 accounts for its accompanying anions, principally chloride and bicarbonate. - The calculated osmolality is usually slightly lower than the measured osmolality.

A 1 wk old infant is diagnosed with achondroplasia on the basis of a combination of clinical and x-ray findings. The parents are being counseled on potential serious complications to be anticipated during infancy and childhood. Which of the following is the most likely potential complication?

spinal cord compression - The spinal canal is stenotic, and spinal cord compression can occur at the foramen magnum and in the lumbar spine. The former usually occurs in infants and small children; it may be associated with hypotonia, failure to thrive, quadriparesis, central and obstructive apnea, and sudden death. - Surgical correction may be required for severe stenosis. - Symptoms include paresthesias, numbness, and claudication in the legs. - Bowing of the legs is common and might need to be corrected surgically. - Other common problems include dental crowding, articulation difficulties, obesity, and frequent episodes of otitis media, which can contribute to hearing loss.

A 5 yr old child has a 6-mo history of increasing difficulty in walking associated with urinary incontinence. Physical examination shows an alert child with increased deep tendon reflexes, clonus and bilateral Babinski reflexes in the lower extremities, and absent deep tendon reflexes in the upper extremities associated with grade 3/5 weakness symmetrically in all extremities. The most likely diagnosis is:

spinal cord tumor - intramedullary spinal cord tumors present in a very insidious manner - back pain related to the level of the tumor is a common presenting complaint - it is likely that this pain will awaken the child from sleep and improve as the day progresses - before the use of MRI became routine, the time from the 1st onset of symptoms to diagnosis of the tumor could be as long as 9 yr - weakness, gait disturbance, and sensory deficits are usually minor and are often found when formal neurologic examinations are performed - scoliosis, urinary urgency, and incontinence may be the presenting complaints associated with intramedullary spinal cord tumors

A 4-year-old boy with symptoms of a viral respiratory infection for 3 days is being evaluated for sudden onset of fever to 39oC, respiratory distress with barking cough, and inspiratory/expiratory monophasic wheezing. His medical history is unremarkable. On physical examination, he appears ill and uncomfortable, with intercostal and suprasternal retractions. Of the following, the pathogen MOST likely associated with this boy's condition is

staphylococcus aureus - The boy in the vignette has had a viral respiratory illness, with a sudden worsening of symptoms, including respiratory distress and fever, indicative of a bacterial tracheitis as a superinfection. - Staphylococcus aureus is the most common organism implicated in bacterial tracheitis. - Adenovirus or respiratory syncytial virus might be the underlying cause of his initial respiratory symptoms, though parainfluenza and influenza viruses are also common precursor infections. - Bacterial tracheitis can be rapidly progressing and life threatening. - Up to 80% of patients with bacterial tracheitis require intubation, thus the first priority in patient management is careful evaluation and stabilization of the airway. - Once the airway is stable, airway radiography and direct visualization of the upper airway in a controlled setting will help to confirm the diagnosis. - Dense purulent secretions and tracheal pseudomembranes may be present and cultures of the secretions should be performed.

An 8 yr old boy from Tennessee received a liver transplant 6 mo ago and is on corticosteroids and azathioprine. He presents to the emergency department with a 2-day history of cough and wheezing. Hemoptysis was observed within the previous 12 hr. A stool specimen is most likely to reveal which of the following microorganisms?

strongyloides stercoralis - The highest prevalence of Strongyloides infection in the USA (4% of the general population) is in impoverished rural areas of Kentucky and Tennessee. - Strongyloidiasis is potentially lethal because of the ability of the parasite to cause overwhelming hyperinfection in immunocompromised persons. - The hyperinfection syndrome is characterized by an exaggeration of the clinical features that develop in symptomatic immunocompetent individuals. - The onset is usually sudden, with generalized abdominal pain, distention, and fever. - Multiple organs can be affected as massive numbers of larvae disseminate throughout the body and introduce bowel flora. The latter may result in bacteremia and septicemia. - Cutaneous manifestations may include petechiae and purpura. - Cough, wheezing, and hemoptysis are indicative of pulmonary involvement. Whereas eosinophilia is a prominent feature of strongyloidiasis in immunocompetent persons, this sign may be absent in immunocompromised persons.

A neonate in the well baby nursery is being prepared for circumcision. Physical examination reveals no abnormalities. Of the following, the MOST effective method of analgesia in this patient is

subcutaneous ring block - Contraindications to circumcision include anatomical concerns, such as hypospadias, epispadias, penile torsion, buried penis, webbed penis, and chordee, and medical contraindications, such as connective tissue disorders.

Active linear LS is typically treated with

systemic corticosteroids and/or methotrexate - to reduce the chances of permanent disfigurement, though sometimes small lesions that do not involve joints or the face can be treated with localized phototherapy, topical corticosteroids, or topical immunomodulators

The time of presentation and intervention for Tetralogy of Fallot is determined by ....

the degree of RV outflow obstruction and limitation of pulmonary blood flow - clinical indicators = oxygen sat levels & dev't of hypercyanotic spells - children with sats less than 80% or those having hypercyanotic spells are scheduled for surgery

Most common complication of an UAC

thrombosis - may cause damping of arterial tracing, NEC, renal insufficiency, HT (renovascular or secondary to aortic obstruction) or decreased blood flow to legs - remove if there are signs of thrombosis

A 5-day-old female neonate is brought to the office in response to a call from the state laboratory reporting a thyroid-stimulating hormone (TSH) level higher than 200 mIU/L (normal <30 mIU/L) on her newborn screen. The neonate was born via uncomplicated spontaneous vaginal delivery at 40 weeks' gestation with a birthweight of 3.5 kg. Her mother took levothyroxine for Hashimoto thyroiditis and prenatal vitamins during pregnancy. The neonate has been breastfeeding well on demand. Her vital signs are normal. She has mild jaundice and a small, reducible umbilical hernia. Her thyroid does not appear enlarged. The physical examination findings are otherwise normal. Confirmatory thyroid function tests show a TSH of 520 mIU/L (normal 0.6-5.6 mIU/L) and a free thyroxine of 0.5 ng/dL (6.4 pmol/L; normal 1.2-3.4 ng/dL [15.4-43.7 pmol/L]). Of the following, the MOST likely cause of this neonate's laboratory findings is

thyroid dysgenesis - Levothyroxine 10 to 15 μg/kg per day is the treatment for congenital hypothyroidism and should be initiated by 2 weeks of age. - Thyroid dysgenesis is the most common cause of congenital hypothyroidism in the United States and most often occurs sporadically.

A 5-day-old female neonate is brought to the office in response to a call from the state laboratory reporting a thyroid-stimulating hormone (TSH) level higher than 200 mIU/L (normal <30 mIU/L) on her newborn screen. The neonate was born via uncomplicated spontaneous vaginal delivery at 40 weeks' gestation with a birthweight of 3.5 kg. Her mother took levothyroxine for Hashimoto thyroiditis and prenatal vitamins during pregnancy. The neonate has been breastfeeding well on demand. Her vital signs are normal. She has mild jaundice and a small, reducible umbilical hernia. Her thyroid does not appear enlarged. The physical examination findings are otherwise normal. Confirmatory thyroid function tests show a TSH of 520 mIU/L (normal 0.6-5.6 mIU/L) and a free thyroxine of 0.5 ng/dL (6.4 pmol/L; normal 1.2-3.4 ng/dL [15.4-43.7 pmol/L]). Of the following, the MOST likely cause of this neonate's laboratory findings is

thyroid dysgenesis - most common cause of congenital hypothyroidism in the United States - includes the lack of or incomplete development of the thyroid gland as well as ectopic thyroid tissue - ectopic thyroid tissue, which does not function normally, may be found anywhere along the path of embryologic migration from the base of the tongue (lingual thyroid) to its normal position in the neck - thyroid dysgenesis most often occurs sporadically

A 14-year-old girl is being evaluated for fatigue, a 7-lb weight loss, decreased exercise tolerance, and difficulty concentrating at school for the past few weeks. Her medical history is not significant and she takes no medication. On physical examination, her temperature is 37°C, blood pressure is 130/68 mm Hg, heart rate is 110 beats/min, respiratory rate is 16 breaths/min, and oxygen saturation is 99% in room air. Her body mass index is at the 10th percentile. She has mild proptosis. Her thyroid is symmetrically enlarged. She has a hyperdynamic precordium, mild tremor, and prominent deep tendon reflexes. The remainder of her examination findings are unremarkable. Thyroid function tests reveal an undetectable thyroid-stimulating hormone level and a free thyroxine level of 4.5 ng/dL (58 pmol/L; reference range, 0.9-1.8 ng/dL [12-23 pmol/L]). Of the following, the test MOST likely to reveal this girl's diagnosis is

thyrotropin receptor antibodies - The girl in the vignette has hyperthyroidism caused by Graves disease. The test most likely to reveal this diagnosis is measurement of thyrotropin receptor antibodies. - Graves disease is an autoimmune disorder caused by functional antibodies that stimulate the thyrotropin (TSH) receptor. Thyroid hormone production by the thyroid gland is increased and the gland enlarges in size. Graves eye disease, manifested as proptosis for the girl in the vignette, can help distinguish Graves disease clinically from other causes of hyperthyroidism. Graves disease is the most common cause of hyperthyroidism in adolescents.

A 2 yr old boy refuses to hold his mother's hand when walking across the parking lot of a shopping mall. He attempts to run away from her, but she quickly grabs his hand before he darts out in front of a car. She is exasperated and scared by his actions. Of the following, which technique is most effective in addressing the behavior?

time-out - discipline should be immediate, specific to behavior, and time-limited - for appx 1 min/year od age = effective

A 9-month-old infant is seen for a health supervision visit. Her initial visit was 5 weeks ago when her new adoptive parents brought her to the clinic, and she received her initial set of vaccinations including DTaP. At that time, she was well nourished and developing well. The infant's mother has heard a news story about pertussis and inquires about when her daughter can have her second dose of DTaP. Of the following, the BEST response to the mother is

today - The Centers for Disease Control and Prevention catch-up vaccination schedule recommends 4 weeks between the first and second dose of DTaP. For the infant in this vignette, the first dose was given 5 weeks ago, making today the preferred response for the timing of the next dose. DTaP contains several diphtheria and tetanus toxoids as well as acellular pertussis. Children should receive 5 doses of DTaP prior to the age of 7 years. Typically the first dose is given at 2 months but can be given as early as 6 weeks. The following doses are typically given at 4 months, 6 months, 15 months, 18 months, and between 4 and 6 years of age. If a child does not receive vaccines on a typical schedule, the minimum intervals between doses are as follows: - Between first and second doses: 4 weeks - Between second and third doses: 4 weeks - Between third and fourth doses: 6 months - Between fourth and fifth doses: 6 months

Refers to inflammation of a part of the large intestine known as the cecum. It's a severe condition that usually affects people with a weakened immune system.

typhlitis

Refers to inflammation of a part of the large intestine known as the cecum. It's a severe condition that usually affects people with a weakened immune system. They can't fight off infections like people with healthy immune systems

typhlitis

2 day old ex FT infant, repeat C-sxn, poor feeding, looks more tired to mom. Labs: Electrolytes normal, BG 77, BUN <3, Cr 0.2, pH 7.5, PCO2 29. Most likely diagnosis:?

urea cycle defect - The patient has a respiratory alkalosis in addition to a normal blood glucose and very low BUN, making an urea cycle defect the most likely IEM present.

A 17-year-old boy with a solitary kidney experiences pain with urination for 5 days, and an intermittent purulent penile discharge. He denies abdominal pain, nausea, vomiting, or fever, and has not noticed any blood in his urine. He has not experienced similar symptoms in the past. In private, he discloses that he has been sexually active since age 16 years, and has had 4 female sexual partners. He recently had unprotected sex with an 18-year-old girl whom he has been seeing for 2 weeks. Of the following, the boy's MOST likely diagnosis is

urethritis - Purulent urethral discharge and dysuria are the most common findings associated with gonococcal urethritis in men. - Screening of asymptomatic heterosexual men should be based on the disease prevalence and individual risk; individuals treated for gonorrhea and/or chlamydia should be rescreened after 3 months.

Magnetic resonance imaging of a newborn shows a right-sided supra-renal lesion. Given its location, this lesion is most likely an adrenal mass. The most common diagnoses for a prenatally identified adrenal mass include adrenal hemorrhage and perinatal neuroblastoma. What is a test most likely to confirm the diagnosis for this neonate?

urine vanillylmandelic acid (VMA) - In a case of suspected neuroblastoma, spot urine testing for VMA and HVA should be performed, and elevated levels can confirm the diagnosis. - However, consumption of vanilla can artificially elevate the levels of VMA and HVA. Neuroblastoma - most common extracranial solid tumor in children Factors used to risk stratify neuroblastoma include: - Age (< 18 months is favorable) - Stage (higher stage is less favorable) - MYCN amplification (amplification is unfavorable) - DNA index (diploid is unfavorable) - Loss of heterozygosity at 1p and 11q (loss is unfavorable)

A 4 yr old boy attends the clinic with a minor upper respiratory tract infection. During the interview the mother tells you that the family shortly will leave the United States for several years' residence in northern Thailand. His vaccinations are up to date for his age. What is your recommendation with regard to travel immunization?

vaccine against japanese encephalitis - mosquito-borne viral disease of humans as well as horses, swine, and other domestic animals that causes human infections and acute disease in a vast area of Asia, northern Japan, Korea, China, Taiwan, Philippines, and the Indonesian archipelago and from Indochina through the Indian subcontinent - travelers to endemic countries who plan to be in rural areas of the endemic region during the expected period of seasonal transmission and travelers in rural areas experiencing endemic transmission should receive JE vaccine

A 1-year-old boy with a seizure disorder is maintained on phenobarbital. He has been having breakthrough seizures and will be starting an additional anticonvulsant. Addition of which of the following medications may lead to phenobarbital toxicity?

valproate Phenobarbital - some data that supports its use for insomnia and hyperbilirubinemia. - acts by enhancing the effect of gamma-aminobutyric acid by facilitating a longer duration of open chloride channels in the neuronal membrane. This increased flux of chloride inhibits membrane depolarization. - metabolized mainly by the liver, with about one-quarter eliminated renally. - Maintenance dosing of phenobarbital typically begins about 12 hours after the loading dose, and pediatric dosing is 3-6 mg/kg/day. - Therapeutic levels are 10 to 40 mcg/mL. - Side effects include, but are not limited to, sedation and respiratory depression. - Phenobarbital interacts with several other anticonvulsants, including valproate. Coadministration may result in decreased metabolism and phenobarbital toxicity, and it may also lead to decreased efficacy of valproate.

Name that disorder: Neonate has a subtype that manifests with severe early-onset cardiac failure that can progress to multiorgan failure. Cardiac findings include hypertrophic or dilated cardiomyopathy, arrhythmias, and pericardial effusion. Affected neonates and infants will have hypotonia and hepatomegaly. Laboratory findings include hypoketotic hypoglycemia, hepatic dysfunction, elevated creatine kinase, and increased dicarboxylic acids on urine organic acid analysis. Acylcarnitine analysis, an appropriate second-tier test, would reveal elevated C14:1, C14:2, C14, and C12:1 metabolites. This disorder is typically detected via newborn screening with elevated C14:1 of more than 1 mmol/L; screening is performed because early intervention and treatment will improve morbidity and mortality in affected children.

very-long-chain acyl-coenzyme A dehydrogenase deficiency - autosomal recessive inborn error of metabolism, can manifest in the neonatal period or infancy with severe early-onset cardiac failure that can progress to multiorgan failure if not treated promptly - clinical findings include hypertrophic or dilated cardiomyopathy, arrhythmias, pericardial effusions, hypotonia, and hepatomegaly. - laboratory findings in very long-chain acyl-coenzyme A dehydrogenase include hypoketotic hypoglycemia, hepatic dysfunction, elevated creatine kinase, abnormal acylcarnitine analysis, and increased dicarboxylic acids on urine organic acid analysis. - prevention of metabolic decompensation with very long-chain acyl-coenzyme A dehydrogenase involves avoidance of triggers such as fasting, dehydration, and long-chain fats; affected children are maintained on a low-fat diet with supplementation of medium-chain triglyceride oil and frequent regular feeding.

A 4 yr old boy who recently moved from Puerto Rico to a northern city in the continental United States presents with a 1-wk history of fever and malaise. Physical examination is notable for temperature 38.5°C, pulse 130 beats/min, respiratory rate 22 breaths/min, blood pressure 100/60 mm Hg, and hepatomegaly. Laboratory findings include a white blood cell count of 60,000/mm3 with 66% eosinophils. His past history includes extensive use of community playground facilities. The most likely diagnosis is:

visceral larvae migrans - Human T. canis infections have been reported in nearly all parts of the world, primarily in temperate and tropical areas where dogs are popular household pets. - Young children are at highest risk because of their unsanitary play habits and tendency to place fingers in the mouth. - In North America, the highest prevalences of infection are in the southeastern USA and Puerto Rico, particularly among socially disadvantaged African-American and Hispanic children. - The classic presentation of VLM includes eosinophilia, fever, and hepatomegaly and occurs most commonly in toddlers with a history of pica and exposure to puppies. - A presumptive diagnosis can be established in a young child with eosinophilia (>20%), leukocytosis, hepatomegaly, fevers, wheezing, and a history of geophagia and exposure to puppies or unrestrained dogs.

Workup for alopecia

vitamin D zinc iron studies hair pull test thyroid studies

Well-trained athletes often develop physiologic cardiac changes known as the athletic heart syndrome, which can mimic pathologic changes on ECG. These changes include

voltage criteria for left ventricular hypertrophy early repolarization right bundle branch block sinus bradycardia first-degree AV block

Goal of "full feedings" for infant....

volume = 150-160 cc/kg/d calories = 110-120 kcal/kg/d

In hypoglycemia in diabetics, usually give fast-acting sugar, wait for sugar to take effect, re-check in 15 minutes, then repeat until BG is stable (over 100 mg/dl). Generally, what is the dose of fast-acting sugar based on age?

~15g in teens 10g in children 5g in small children For reference, 15g of rapid acting glucose = - 4oz fruit juice - 3-4 glucose tablets - 1 tube of glucose gel - 6-8 oz sports drink - 1 tablespoon honey - 6oz regular (not diet) soda (about half a can) - 3 teaspoons table sugar (3 packets) - one-half tube of cake gel

A mother brings her 5-year-old son to the clinic for evaluation of a harsh cough. The child has a 2-day history of clear rhinorrhea and cough, without fever or respiratory distress. The mother is demanding a prescription for an albuterol inhaler because she is concerned her child's condition will worsen over the next few days. She also requests a doctor's note to excuse the child from school for the entire upcoming week. The resident seeing the patient believes that the boy has a mild viral upper respiratory illness and he does not feel that the mother's requests are medically indicated. The boy's vital signs are normal. His physical examination findings are unremarkable other than mild clear rhinorrhea. In particular, the lung examination finds no wheezing or focal findings. The child's medical history helps with understanding the mother's concerns. The boy was born late preterm and stayed in the hospital for 2 weeks for observation and phototherapy. At 11 months of age, he required hospitalization for 3 days for bronchiolitis. He has been seen often for mild viral illnesses, but has no documented history of wheezing. However, the mother admits that she sometimes gives the patient his brother's albuterol when he is coughing a lot. The boy has been diagnosed and treated for otitis media twice, but has no history of pneumonia. He has received all his routine health maintenance care, including immunizations. He has normal growth and development, but has some behavioral problems at school. Of the following, the MOST appropriate next step in management is to

discuss with the mother her parental perception of vulnerability to illness Vulnerable child syndrome - parental over concern for minor health issues and excessive use of health services - Secondary behavioral problems in the child and issues with parent-child interactions are common in vulnerable child syndrome. - Families at greatest risk for vulnerable child syndrome have predisposing factors that may include environmental conditions, parental psychological conditions, complicated birth history, or a significant medical or family history. - Clear and direct communication with parents that past experiences with illness can affect their perception of their child's health is key to preventing vulnerable child syndrome.

A 3-month-old boy has had excessive tearing of the left eye since shortly after birth. His parents report that the discharge is typically clear but is sometimes more thick and opaque. They have not noticed redness or swelling of the eye or any injection of his conjunctiva. The discharge does not seem bothersome to the baby, but they are concerned that it has not decreased over time. Which of the following is the most likely site of the underlying obstruction?

distal nasolacrimal valve Obstruction of the nasolacrimal duct - symptoms often develop three to five weeks after birth and are usually due to a membranous blockage at the distal valve of the nasolacrimal duct (the valve of Hasner). - as a result of the obstruction, the contents of the nasolacrimal duct drain predominantly through the canaliculi into the puncta of the eye, either as tears or mucopurulent discharge. - Most cases spontaneously resolve before 6 months of age. If symptoms persist beyond 8 months of age, they are unlikely to self-resolve, and it is prudent to refer the patient to pediatric ophthalmology for procedural management, typically probing, to relieve the obstruction.

Infant has failure to thrive and normal anion gap metabolic acidosis. The higher-than-expected urinary pH and positive urine anion gap favors a diagnosis of

distal renal tubular acidosis - Diarrhea and renal tubular acidosis are the most common causes of normal anion gap acidosis in children. - The urine anion gap helps differentiate diarrhea from renal tubular acidosis. - In distal renal tubular acidosis, the urine anion gap is positive and ammonium (NH4+) excretion is low.

An 8-month-old boy presents to the emergency department for lethargy and poor feeding for two days. Mother denies any fever. His temperature is 100.4°F (37.0°C), heart rate is 160 bpm, respiratory rate is 45 bpm, and blood pressure is 65/40 mm Hg. On examination his capillary refill time is 5 seconds, and peripheral pulses are difficult to palpate. You obtain laboratory studies, order blood cultures, and administer a total of 80 mL/kg of normal saline fluid and initiate antibiotics. The infant now has a heart rate of 180 bpm, respiratory rate of 60 bpm, blood pressure of 50/28 mm Hg, and capillary refill time of greater than 6 seconds. A chest X-ray is obtained which reveals pulmonary edema and and increased cardiac silhouette. What is the most appropriate next step in management?

dobutamine infusion tachycardia, low blood pressures, and poor perfusion suggestive of shock. - though this patient receives aggressive resuscitation, he deteriorates clinically with chest X-ray significant for cardiomegaly and pulmonary edema. - chest X-ray findings suggest that the cause of this boy's shock is cardiogenic. - appropriate treatment of cardiogenic shock is respiratory stabilization, judicious use of fluids, and initiation of inotropic medications to improve cardiac function. - dobutamine is the inotrope of choice in cardiogenic shock as it works to improve cardiac contractility and systemic vascular resistance.

Pathophysiology of malrotation and volvulus...

doesn't rotate ccw around SMA

More appropriate for treating hypotension in an asphyxiated infant....

dopamine - starting dose: 5 mcg/kg/min

The initial management of opioid intoxication in both children and adults should focus on stabilization of the airway, support of breathing, and hemodynamic support. In cases of known or suspected opioid overdose, naloxone should be administered promptly. Naloxone, a pure opioid antagonist, can be given intravenously, subcutaneously, intramuscularly, via endotracheal tube, or by nebulizer. The onset of action of naloxone is rapid, usually within 1 minute of administration. In the setting of acute opioid overdose, for children up to 5 years of age and those weighing less than 20 kg, what dose should be administered?

dose of 0.1 mg/kg of naloxone - in older children, a rapid dose of 2 mg should be given - repeat doses may be given every 2 to 3 minutes, up to a max dose of 10 mg for children of all ages

A 2 yr old boy is presented to the emergency department at 3 AM with a chief complaint of fever and cough. His respiratory rate is 36 breaths/min, his temperature is 39°C, and his pulse oximetry reading is 96%. On physical examination he has a barky cough and stridor only with crying. He is well hydrated, able to drink, and consolable. What is the appropriate next step in patient management?

dose of dexamethasone - Effectiveness of oral corticosteroids in viral croup is well established. - Corticosteroids decrease the edema in the laryngeal mucosa through their anti-inflammatory action. - Most studies that demonstrated the efficacy of oral dexamethasone used a single dose of 0.6 mg/kg; a dose as low as 0.15 mg/ kg may be just as effective. - Intramuscular dexamethasone and nebulized budesonide have an equivalent clinical effect; oral dosing of dexamethasone is as effective as intramuscular administration.

The recommended agent for treatment of Rocky Mountain spotted fever (RMSF) in a child younger than 8 yr is:

doxycycline - The time-proven effective therapies for RMSF are tetracyclines and chloramphenicol. - The treatment of choice for suspected RMSF in patients of all ages, including for young children, is doxycycline (4 mg/kg/day divided every 12 hr PO or IV, maximum 200 mg/day). - Tetracycline (25-50 mg/kg/day divided every 6 hr PO, maximum 2 g/day) is an alternative. - Chloramphenicol (50- 100 mg/kg/day divided every 6 hr IV, maximum 4 g/day) should be reserved for patients with doxycycline allergy and for pregnant women, because chloramphenicol has been shown to be an independent risk factor for increased mortality vs. tetracycline. - Although tetracycline and doxycycline may be associated with tooth discoloration in children < 8 yr of age, RMSF is a life-threatening illness for which prompt therapy is imperative.

The most frequent toxic neuropathy in children in the United States currently is due to:

drugs used in chemotherapy 0 Antimetabolic and immunosuppressive drugs, such as vincristine, cisplatin, and paclitaxel, produce polyneuropathies as complications of chemotherapy for neoplasms. This iatrogenic cause is the most common etiology of toxic neuropathies in children. - It is usually an axonal degeneration rather than primary demyelination, unlike autoimmune neuropathies.

A male neonate has respiratory distress, dysphagia, and generalized muscular hypotonia and weakness. Pregnancy was uncomplicated, but the mother reports weak fetal movements in the last trimester. Delivery at term was uneventful, and Apgar scores at 1 and 5 min were 7 and 8. On examination, he has a dolichocephalic head, high-arched palate, thin muscle mass in trunk and extremities, and undescended testes. This clinical picture is suggestive of:

duchenne muscular dystrophy - most common hereditary neuromuscular disease affecting all races and ethnic groups - characteristic clinical features are progressive weakness, intellectual impairment, hypertrophy of the calves, and proliferation of connective tissue in muscle - infant boys are only rarely symptomatic at birth or in early infancy, although some are mildly hypotonic - early gross motor skills, such as rolling over, sitting, and standing, are usually achieved at the appropriate ages or may be mildly delayed - poor head control in infancy may be the 1st sign of weakness

A 3-year-old previously healthy girl is seen for evaluation of nasal drainage that started 2 weeks ago. Initially, it was clear and associated with low-grade fever and mild cough. Four to 5 days into the illness, the drainage became thick and yellow. Although the fever has resolved, she continues to have cough. She has a temperature of 37°C, blood pressure of 90/55 mm Hg, heart rate of 100 beats/min, and respiratory rate of 24 breaths/min. She has swelling and erythema of the right nasal turbinate. There is purulent drainage from the left nare and tenderness over the maxillary sinuses. Of the following, the clinical feature that BEST supports the suspected diagnosis is

duration of symptoms For the girl in this vignette, the clinical feature that best supports the diagnosis of acute bacterial sinusitis is duration of symptoms. There are 3 clinical criteria that help discern acute bacterial sinusitis from its more common counterpart viral upper respiratory tract infection (URI): - Persistence of symptoms beyond 10 days without improvement - Worsening of symptoms or new onset of symptoms after initial improvement - Severe symptoms at onset lasting for 3 consecutive days - Because the girl's symptoms have lasted for 2 weeks without improvement, she meets criteria for acute bacterial sinusitis on the basis of persistence of symptoms. Persistence of symptoms is the most common presentation of acute bacterial sinusitis in children. In contrast, viral URIs typically have a duration of 5 to 7 days.

A 2-year-old boy presents to the office with progressive muscle weakness. He started walking at 1 year of age, but his parents note that in the last six months, he seems to be more clumsy, falling more often and having difficulty pushing himself up from the floor to a standing position. He has to use his hands to accomplish this. On exam, his calves appear hypertrophic. His creatine kinase levels are grossly elevated. Molecular genetic testing is done to confirm the diagnosis. What other test should be ordered on this patient at the time of diagnosis?

echocardiogram Duchenne muscular dystrophy - X-linked progressive muscular dystrophy caused by a mutation of the dystrophin gene. - Pseudohypertrophy of the calves is common. - Diagnosis is confirmed by elevated creatine kinase levels and molecular genetic testing. - The mainstay of treatment is glucocorticoid administration. - It is important to obtain an echocardiogram and ECG at the time of diagnosis and annually, as dilated cardiomyopathy is common. - Additionally, conduction defects and dysrhythmias are seen. - Fibrosis of the ventricular wall can also occur. - Cardiomyopathy is one cause of mortality in this disease. - Angiotensin-converting enzyme inhibitor or angiotensin receptor blocker medications are used in these patients.

A 1 mo old female infant is presented with a chief complaint of poor feeding and lethargy. Her parents report that the child was well until 3 days earlier, when poor feeding began. Pulse rate is 280 beats/min, respiratory rate is 50 breaths/min, and blood pressure is 80/50 mm Hg. Physical examination shows a gallop rhythm and an enlarged liver palpable 2-3 cm below the right costal margin. The most appropriate diagnostic test for this patient would be:

electrocardiogram - Persistent tachycardia (>200 beats/min in neonates, 150 beats/min in infants, or 120 beats/min in older children), bradycardia, or an irregular heartbeat other than sinus arrhythmia requires investigation to exclude pathologic arrhythmias. - This infant has symptoms of heart failure, likely caused by an underlying tachyarrhythmia.

A 14-year-old adolescent girl is brought to the emergency department with a 1-day history of fever and left eye swelling, with an inability to open the eye. Two weeks ago, she had a mild upper respiratory tract infection. On physical examination, she is alert and interactive with a temperature of 39.2°C. Examination of the left eye reveals periorbital redness and swelling, proptosis, conjunctival edema, and limited extraocular movements. The remainder of the physical examination findings are normal. Laboratory findings are notable for a white blood cell count of 18,000/μL (18 × 109/L) and elevated C-reactive protein level. Of the following, the MOST likely source of this patient's infection is the

ethmoid sinus - Fever, unilateral periorbital swelling, and redness, with proptosis, restricted extraocular movements, and conjunctival chemosis after an upper respiratory tract infection. These physical examination findings and patient history are consistent with a diagnosis of orbital (postseptal) cellulitis. - Orbital cellulitis is a serious infection of the orbital tissue posterior to the orbital septum, usually complicating sinusitis (especially the ethmoid sinus). - The orbital septum is a protective layer of periosteal fibrous connective tissue extending to the upper and lower eyelids. - The ethmoid sinus is separated from the orbit by a thin medial orbital wall (lamina papyracea). - The microbiology of orbital cellulitis includes organisms associated with acute or chronic sinusitis including Streptococcus pneumoniae, Staphylococcus aureus, Streptococcus anginosus, Moraxella catarrhalis, nontypeable Haemophilus influenzae, and upper respiratory tract anaerobes. - Management of orbital cellulitis warrants an urgent and multidisciplinary approach, including specialist consultations from pediatric infectious disease, otolaryngology, and ophthalmology. - Empiric antimicrobial therapy of orbital cellulitis should be rapidly initiated; intravenous ampicillin-sulbactam and vancomycin are recommended to treat methicillin-resistant S aureus and other microorganisms associated with sinusitis. - A 5- to 7-day course of parenteral antibiotic therapy (until the eye examination results are greatly improved), followed by 2 weeks of oral antibiotic therapy, is recommended. - Surgical drainage of the affected sinus and abscess is indicated in patients with large, well-defined subperiosteal and/or orbital abscess, visual impairment, or complete ophthalmoplegia. - Medical management alone is often adequate in patients with orbital cellulitis associated with phlegmon or small subperiosteal abscess, if the patient clinically improves within 48 to 72 hours.

An otherwise healthy, asymptomatic 16-yr-old African-American girl is found to have hypertension and 3+ proteinuria by dipstick testing on mid-day and 1st morning voided urine samples. The microscopic analysis shows 0-2 red blood cells per high-power field. The most likely diagnosis is:

focal segmental glomerulosclerosis Idiopathic nephrotic syndrome - associated with primary glomerular disease without evidence of a specific systemic cause Idiopathic nephrotic syndrome - multiple histologic types: minimal change disease, mesangial proliferation, focal segmental glomerulosclerosis (FSGS), membranous nephropathy, and membranoproliferative glomerulonephritis. - more common in boys than in girls (2:1) - most commonly appears between the ages of 2 and 6 yr - has been reported as early as 6 mo of age and throughout adulthood. - more common cause of idiopathic nephrotic syndrome in this older age group is FSGS - may be more common in African-American, Hispanic, and Asian patients

A 2-year-old girl, not yet toilet trained, is being evaluated for diarrhea. She began having loose stools 4 months ago, and now has 4 to 6 nonbloody stools daily. Her first-morning stool tends to be somewhat formed. As the day progresses, her stools become looser, with mucus and whole pieces of food present. She has frequent diaper rashes that resolve after a single application of over-the-counter diaper cream. The girl is gaining weight well and meeting her developmental milestones. Her mother is lactose-intolerant and has been keeping lactose out of the girl's diet; the girl has no other diet restrictions. She drinks 24 oz of juice and 20 oz of lactose-free whole milk daily. Her favorite foods are pancakes and syrup, peanut butter and jelly sandwiches, and spaghetti and meatballs. Her medical and surgical history are unremarkable. Her weight is 12.6 kg (50th percentile), length is 87 cm (40th percentile), and body mass index is 17 kg/m2 (67th percentile). She is well nourished and interactive. Her abdomen is soft and nontender, with mild distention. She has no diaper rash. The remainder of the physical examination findings are within normal limits. Of the following, the dietary intervention MOST likely to improve the girl's symptoms is reduced consumption of

fructose - Functional diarrhea is a benign and self-limiting process in young children. - Functional diarrhea occurs in the absence of evidence of a malabsorptive syndrome, failure to thrive, hematochezia, and severe resistant diaper rash. - Helpful dietary interventions for functional diarrhea include limiting fructose and increasing fiber and fat intake.

A 3-year-old boy is brought to the emergency department after sustaining a scald burn to the majority of his torso. His family states that he reached up to the stove and pulled on the handle of a pot of boiling water that spilled on the anterior surface of his chest. The total body surface area burned is 40%. After the patient has undergone initial resuscitation and fluid management, the burn service asks about the plan for nutritional support. Of the following, the MOST appropriate nutritional regimen for this patient is administration of

full enteral feedings with high-protein formula supplemented with vitamins C and A and zinc sulfate to provide 30% above resting energy expenditure - Unlike other traumatic injuries, burns result in an increase in resting energy expenditure (REE) and hypermetabolic state. Thus, the prescription of nutrition must address the increase in caloric requirements. - Best nutritional plan to provide appropriate calories and nutrition for promotion of optimal wound healing. - Adequate nutritional support is required for optimal wound healing in pediatric patients with burns, and the enteral route is preferred, unless otherwise contraindicated, to ensure positive nitrogen balance and reduce gastrointestinal bacterial translocation. - For children with large total body surface area burns, caloric needs will be higher than usual resting energy expenditure. - Vitamins C and A and zinc sulfate should be prescribed in burn management to ensure adequate wound healing.

A 10-year-old boy presents to the ED with gross hematuria. He was treated for streptococcal pharyngitis two weeks ago. His blood pressure is elevated, but his physical exam is normal. Laboratory testing reveals low C3 and CH50 and elevated creatinine. Administration of which of the following is most appropriate?

furosemide poststreptococcal glomerulonephritis - typically presents one to three weeks after group A streptococcal pharyngitis. - often have asymptomatic microscopic hematuria but may present with increased creatinine, proteinuria, edema, or elevated blood pressure in addition to hematuria. - Supportive care is the cornerstone of treatment with management of symptoms, which typically result from fluid overload. - Hypertension and pulmonary edema can be treated with fluid restriction, salt restriction, and a loop diuretic such as furosemide. - In case of encephalopathy from hypertension, administration of oral nifedipine or intravenous nicardipine is indicated to rapidly decrease blood pressure. - Dialysis is a consideration for patients who have refractory fluid overload that may be life-threatening, hyperkalemia that is not responding to therapy, or uremia. - The prognosis for children with poststreptococcal glomerulonephritis is good, with > 90% having normal or near-normal renal function 5 to 18 years after presentation.

A 9 day old full-term infant is admitted to the hospital with lethargy, fever, and increasing jaundice. Physical examination also reveals hepatomegaly. Laboratory results reveal a blood glucose value of 10 mg/dL, total and direct bilirubin values of 15 and 7 mg/dL, respectively, and liver enzymes AST, 700 units/L, and ALT, 650 units/L. The next day the blood culture is positive for a gram-negative rod. The most likely diagnosis is:

galactossemia - uridyl transferase deficiency (galactosemia) - jaundice, hepatomegaly, vomiting, hypoglycemia, seizures, lethargy, irritability, feeding difficulties, poor weight gain or failure to regain birth weight, aminoaciduria, nuclear cataracts, vitreous hemorrhage, hepatic failure, liver cirrhosis, ascites, splenomegaly, or mental retardation - increased risk for Escherichia coli neonatal sepsis - onset of sepsis often precedes the diagnosis of galactosemia

A 15 yr old presents with chronic fatigue and severe bone pain of 1 year's duration. He has hepatosplenomegaly and a normal retinal examination. Laboratory studies reveal normocytic anemia and thrombocytopenia. Radiographs of the distal femur reveal Erlenmeyer flask deformities. The most likely diagnosis is:

gaucher disease - multisystemic lipidosis characterized by hematologic abnormalities, organomegaly, and skeletal involvement, the latter usually manifesting as bone pain and pathologic fractures - one of the most common lysosomal storage diseases and the most prevalent genetic defect among Ashkenazi Jews 3 clinical subtypes, delineated by the absence or presence and progression of neurologic manifestations: - type 1, or the adult, nonneuronopathic form; - type 2, the infantile or acute neuronopathic form; - type 3, the juvenile or subacute neuronopathic form - All are autosomal recessive traits - Type 1 accounts for 99% of cases - At presentation, patients may have bruising from thrombocytopenia, chronic fatigue secondary to anemia, hepatomegaly with or without elevated liver function test results, splenomegaly, and bone pain - Most patients develop radiologic evidence of skeletal involvement, including an Erlenmeyer flask deformity of the distal femur

A 9-year-old girl is brought to the office by her mother because she thinks her daughter may have a urinary tract infection. For the past week, the girl has been urinating frequently. Her mother reports that she has been waking up at least twice at night to use the bathroom. Her teacher is concerned because she leaves class to use the bathroom almost every hour. She has had no dysuria, fever, nausea, vomiting, or back pain. Her medical history is not significant, and she has had no previous urinary tract infection. Her temperature is 37°C, blood pressure is 98/60 mm Hg, heart rate is 110 beats/min, respiratory rate is 18 breaths/min, and room air oxygen saturation is 99%. She has had a 2.3-kg weight loss since her last health supervision visit 3 months ago. Her body mass index is at the 10th percentile. She appears tired. There is no suprapubic or costovertebral angle tenderness. The remainder of her examination findings are unremarkable. Of the following, the BEST test to inform management for this girl is a

glucose and electrolyte panel - The girl in the vignette has type 1 diabetes mellitus (DM). Her polyuria, weight loss, tachycardia, and lack of other symptoms of a urinary tract infection are suggestive of this diagnosis. - The best test to inform management for this girl is a glucose and electrolyte panel. A random plasma glucose level greater than or equal to 200 mg/dL (11.1 mmol/L) with symptoms of hyperglycemia is diagnostic of DM. The bicarbonate level will help determine the child's degree of acidosis and whether diabetic ketoacidosis (DKA) is likely. - Diagnostic criteria for DKA include plasma glucose greater than 200 mg/dL (11 mmol/L), venous pH less than 7.3 or bicarbonate less than 15 mEq/L (15 mmol/L), and ketonemia plus ketonuria. - Recommended tests at the time of diagnosis of type 1 diabetes mellitus include a glucose and electrolyte panel, venous pH, urinalysis for determination of ketone level, serum ketone level, and a baseline hemoglobin A1c.

A 6-month-old male infant who is limp and exhibiting obtundation in the morning is brought to the emergency department. Bedside blood analysis reveals a significant metabolic acidosis, including an elevated lactate level and a capillary blood glucose level of 35 mg/dL (1.9 mmol/L) and 3+ ketones in the urine. His newborn screening results were normal. His parents report that he only recently has been able to sleep through the night without waking for a feeding and that he has had a few episodes of morning lethargy that improved after his first morning feeding. The infant appears small and nearly comatose. He has chubby cheeks, a short nose, and a relatively small chin. He has a temperature of 37.8°C, a heart rate of 155 beats/min, a respiratory rate of 15 breaths/min, and a blood pressure of 75/45 mm Hg. Cardiac examination reveals no murmur and a normal S1 and S2. The liver edge is palpable 3 cm below the right costal margin. The remainder of the physical examination findings are unremarkable. Of the following, the MOST likely diagnosis is

glycogen storage disorder - Glycogen storage disease often presents in infancy and results in hepatomegaly, growth failure, and recurrent episodes of hypoglycemia with ketosis.

Adolescent's symptoms are consistent with acute intoxication which causes euphoria, tachycardia, hypertension, nausea, dizziness, weakness, vomiting, and diarrhea. Synesthesia, a state of altered perception where one sense is perceived as another (eg, hearing colors), is the most distinctive symptom indicating

hallucinogen use - act at serotonin receptors and there is a risk for serotonin toxicity with their use - they include lysergic acid diethylamide (LSD), mescaline, psilocybin, N,N-dimethyltryptamine (DMT), lysergic acid amide (LSA), Salvia divinorum, and recreational nutmeg. - Benzodiazepines may be used for significant agitation and dysphoria.

An 8-year-old boy with trisomy 21 is seen for a health supervision visit. His mother has no concerns. He has a history of an atrioventricular septal defect that was repaired in infancy. His only medication is a daily multivitamin. He is doing well in the second grade with an Individualized Education Program. A review of systems is unremarkable. He has a temperature of 37°C, heart rate of 62 beats/min, and blood pressure of 102/64 mm Hg. His weight and height are at the 50th percentile on syndrome-specific growth curves. His thyroid appears enlarged. There is no cervical lymphadenopathy. The remainder of his physical examination findings are unremarkable. Of the following, the MOST likely cause of his examination findings is

hashimoto thyroiditis - autoimmune thyroid disease - most common cause of thyroid enlargement in children - children with trisomy 21 are at increased risk for congenital hypothyroidism and acquired autoimmune thyroid disease - thyroid screening tests are recommended at birth, 6 months, 12 months, and annually thereafter

Decisions regarding whether to perform bone densitometry in children with fractures are more nuanced. In otherwise healthy children with fractures, the AAP currently recommends bone densitometry for children who

have sustained clinically significant fractures (≥2 long bone fractures before age 10 years, ≥3 long bone fractures before age 19 years, or a vertebral fracture occurring without significant trauma or local disease)

A 13-year-old girl is brought to the emergency department (ED) with vaginal discharge of 2 weeks' duration by her mother. Her mother is concerned about unusual behavior in her daughter over the past 6 months. She reports 3 to 4 male sexual partners and rare use of barrier protection. Her behavior in the ED is engaging and interactive. She does not know the day of the week and cannot recall her address. Despite the hot July weather, she is dressed in a heavy sweatshirt and jeans. A physical examination reveals cervical motion tenderness. The remainder of her physical examination findings are normal. Of the following, this adolescent's presentation is MOST concerning for

human trafficking - The adolescent in the vignette has vaginal discharge, cervical motion tenderness, and multiple sexual partners, all concerning for a sexually transmitted illness. Her inability to recall her address or the date, in combination with her inappropriate clothing for the weather, suggest that she may be a victim of human trafficking (HT). - While the girl in the vignette likely has a sexually transmitted disease which needs to be treated, the cervical motion tenderness suggests pelvic inflammatory disease and not chemical vaginitis. Although she does have unusual behavior, in not knowing the day of the week and ability to recall her address, the girls inappropriate clothes for the weather, multiple sexual partners and clinical presentation is more likely due to the trauma of human trafficking than acute psychosis or major depressive disorder.

A 4 yr old boy in good health presents with a 3-day history of a tender mass, 2 cm in diameter, midway between the umbilicus and the xiphoid process. He is afebrile, is eating normally, and has an otherwise normal physical examination. The most likely diagnosis is:

incarcerated epigastric hernia - ventral hernias in the midline of the abdominal wall between the xiphoid and the umbilicus - result from defects in the decussating fibers of the linea alba and are more likely congenital than acquired - most epigastric hernias are small and asymptomatic - reported incidence in childhood varies from < 1% to as high as 5% - may be single or multiple and are 2-3 times more common in males than females - defect typically contains only preperitoneal fat without a peritoneal sac or abdominal viscera - mass may be tender to examination, but strangulation of the hernia contents is uncommon - physical examination is almost always diagnostic and imaging studies are unnecessary - do not resolve spontaneously, and therefore operative repair is the recommended treatment

Hepatitis B surface antigen is present in individuals with acute or chronic infection and vaccinated individuals for 3 weeks after receiving the hepatitis B vaccination. Hepatitis B core antibodies develop only in the presence of

infection - core antigen is not contained in the vaccine

A multi-institutional study examines the impact of a web-based intervention for weight loss in adolescents who are obese. The study requires participation in an interactive internet-based intervention; measurement of height, weight, body mass index, and abdominal girth every month; and blood tests every 6 months. A 17-year-old adolescent boy seen in the local investigator's practice meets all eligibility criteria, and he is approached for participation in the study. Of the following, study enrollment requires

informed consent from the parents and assent from the adolescent - Involvement of minors (people younger than 18 years) in research requires informed consent from their adult proxies (parents or legal guardians), as well as assent from the minors themselves if they are old enough to understand their participation. - While the minimum age of assent varies, children as young as 7 years have developed limited logical thought processes and have the capacity for reasoned decision-making.

The most common cause of DKA in individuals whose diabetes is managed with an insulin pump is

interruption of insulin delivery, usually caused by a compromised infusion site - Insulin pumps deliver only rapid-acting insulin, so when interrupted, DKA occurs more quickly than in individuals receiving a long-acting basal insulin injection. - Education is critical for the prevention of DKA in individuals on an insulin pump. - Ketones should be monitored at home during times of significant hyperglycemia and on sick days. Additional insulin and oral fluid are needed if ketones are present, and the additional insulin should be administered by injection. - Families should also have 24-hour access to medical advice by telephone.

A 6-month-old infant is brought to the office for a health supervision visit. He was delivered at 28 weeks of gestation because of worsening maternal preeclampsia He remained in the neonatal intensive care unit for 3 months. His hospital course included continuous positive airway pressure for 1 month, treatment with ibuprofen for a patent ductus arteriosus, and a blood transfusion. At age 1 month, head ultrasonography demonstrated a left grade 2 intraventricular hemorrhage He was discharged from the hospital in room air. He receives physical therapy weekly for increased lower extremity tone. His mother is worried about his long-term development. Of the following, this infant's MOST important prognostic risk factor for neurodevelopmental outcomes is

intraventricular hemorrhage - For the premature infant, after gestational age, intraventricular hemorrhage (IVH) of any grade is the risk factor most closely associated with neurodevelopmental impairment. - Premature infants with grade 1 and 2 intraventricular hemorrhage (IVH) have worse neurodevelopmental outcomes than infants without IVH; grades 3 and 4 IVH have a clear association with neurodevelopmental impairment. - In preterm infants, bacteremia and necrotizing enterocolitis are associated with neurodevelopmental impairment. - Premature infants with and without intraventricular hemorrhage are at increased risk for cerebral palsy and periventricular leukomalacia.

Adolescent has acute myelogenous leukemia, persistent neutropenia, and fever while receiving broad-spectrum antibiotics; negative blood culture results; and a finding of bilateral pulmonary nodules—the greatest concern regards

invasive mold infection, especially Aspergillus species - Aspergillus is a ubiquitous mold that grows in soil. - In humans, Aspergillus colonizes the respiratory tract. - Acquisition of infection occurs via inhalation of airborne conidia (spores) from various environmental sources and soil. - The most common cause of invasive aspergillosis is Aspergillus fumigatus, followed by Aspergillus flavus. - Invasive aspergillosis may complicate the clinical course of immunocompromised patients (eg, those with acute myeloid leukemia, with aplastic anemia, undergoing intensive chemotherapy) with prolonged neutropenia; stem-cell transplant recipients during periods of neutropenia and graft-versus-host disease; patients with phagocyte dysfunction (eg, chronic granulomatous disease); or those receiving T-lymphocyte immunosuppressive therapy (eg, steroids, calcineurin inhibitors, tumor necrosis factor-ɑ inhibitors). - Pulmonary infection is the most common manifestation of invasive aspergillosis, but other sites, including the sinus, skin, and brain, may be involved. Invasive pulmonary aspergillosis often presents with unremitting fever in high-risk patients who are immunocompromised; other early symptoms may include dry cough or chest pain. Dyspnea is noted in individuals with diffuse pulmonary disease. Hemoptysis can occur and may be fatal, given the angioinvasive nature of Aspergillus spp. - The diagnosis of invasive pulmonary aspergillosis is suggested by positive galactomannan assay finding in the serum or bronchoalveolar lavage fluid (BAL) and evidence of dichotomously branched and septate hyphae on fungal smears of tissue or BAL specimens. Negative fungal culture results on a BAL specimen does not exclude disease. Definitive diagnosis of invasive aspergillosis is made via isolation of the mold in tissue biopsy specimens (eg, skin, lung, sinus). Chest radiography may show multiple, ill-defined, peripheral nodules that may coalesce to larger masses or lobar or diffuse consolidative lesions. High-resolution computed tomographic scan is the imaging modality of choice for evaluation of invasive pulmonary aspergillosis and may demonstrate multiple pulmonary nodules, early halo sign (ie, rim of ground-glass opacity surrounding the nodule seen in neutropenic hosts reflecting hemorrhagic nodule from angioinvasion), followed later by cavitation or the "air crescent" sign after resolution of neutropenia. These radiographic findings are nonspecific and may be seen in other invasive mold infections such as mucormycosis. Magnetic resonance imaging is the recommended imaging standard for central nervous system disease.

Puberty is considered delayed in boys when there is

lack of testicular growth ≥ 4 mL in volume (or 2.5 cm in length) by age 14 years. - Constitutional delay of puberty is the most common cause of delayed puberty, especially in boys. - Bone age is delayed and during adolescence more closely matches the pubertal stage than chronological age. - Best next step is referral for consideration of testosterone therapy. A short course of a testosterone ester intramuscularly once per month for 3 to 4 months can accelerate pubertal development and promote earlier initiation of the pubertal growth spurt. It can have a positive effect on the psychosocial distress that can accompany constitutional delay. There is no significant effect on final adult height. - Reassurance with observation would also be an acceptable management option.

Imaging for kid with.... suspected fluid in pleural space

lateral decubitus and radiograph

One must determine if ET tube is within trachea or within esophagus. This may be difficult to assess on AP view by more easily seen on ...

lateral view

An 8 yr old girl treated from infancy for adrenal insufficiency is noted to have inguinal gonads. Karyotype is 46,XY. The most likely diagnosis is:

lipoid adrenal hyperplasia - usually unable to synthesize any adrenal steroids - salt-losing manifestations are usual, and many infants die in early infancy - genetic males are unable to synthesize androgens and thus are phenotypically female but with gonads - genetic females appear normal at birth and may undergo feminization at puberty with menstrual bleeding. they, too, progress to hypergonadotropic hypogonadism when accumulated cholesterol kills granulosa (i.e., steroid synthesizing) cells in the ovary

For mania in classically defined bipolar I disorder, medication is the primary treatment; medications used with adults may be less effective with youths (<50% response rate). Standard pharmacotherapy includes ...

lithium valproate atypical antipsychotics (aripiprazole, olanzapine, risperidone, quetiapine, ziprasidone)

A 2-day-old neonate is evaluated in the nursery. She was born to a 20-year-old primigravida mother at 38 weeks of gestation by vaginal delivery. Prenatal ultrasonography was significant for hydronephrosis. She is breastfeeding well and has good urine output. Her weight is 3.1 kg, and her length is 48 cm. She is vigorous and alert, with normal physical findings. Laboratory testing indicates that her serum creatinine level is 0.7 mg/dL (62 µmol/L). Ultrasonography confirms a mild hydronephrosis on the right side and a normal left kidney. A medical student asks about this neonate's serum creatinine level and its relation to glomerular filtration rate. Of the following, the MOST accurate response is that the

maternal creatinine is responsible for the current serum creatinine value - The term newborn in this vignette has mild unilateral hydronephrosis on postnatal ultrasonography. A serum creatinine value of 0.7 mg/dL (62 µmol/L) for a 2-day-old neonate is reflective of the circulating maternal creatinine. Since the newborn has 1 normal kidney on ultrasonography, the elevation in serum creatinine cannot be explained on the basis of unilateral hydronephrosis. - In term neonates, the serum creatinine value at birth is reflective of circulating maternal creatinine (0.8-1 mg/dL [71-88 µmol/L]) and gradually declines to neonatal values (0.2-0.4 mg/dL [18-35 µmol/L]) in the first 1 to 2 weeks after birth. In preterm infants, the decline in serum creatinine is slower and can take 1 to 2 months to reach the normal range. The serum creatinine level again starts to rise in older children due to increased muscle mass.

During an evaluation of a 12-month-old girl with bronchiolitis, there is concern for possible language delay. The parents report she does not have any words and does not say "mama" or "dada." Her past medical history is only positive for being born large for gestational age. Her parents report they speak Spanish with each other and English with her 11-year-old sibling. The only significant family medical history is depression in the mother. Of the following, which is a risk factor for this girl's expressive speech delay?

maternal depression expressive speech delay - defined as not meeting a milestone that 90% of typically developing children have met for that specific age. - At 12 months of age, 90% of children can say "dada" or "mama," therefore, there is concern for expressive speech delay. - Risk factors associated with expressive speech delay are male sex, poverty, lack of parental education, low birth weight, prematurity, family history of language delays, and maternal depression. - Maternal depression is a significant risk factor for expressive language delay, as there is low quantity and quality of exposure to maternal speech, which creates a poor linguistic environment for the child. Evaluation for language delay should include a complete blood count to rule out iron-deficiency anemia, venous lead level, and a hearing test. Any child diagnosed with expressive speech delay should be referred to speech therapy.

Vaccine that can temporarily suppress reaction to tuberculin used in TST/PPD

measles - no evidence that other vaccine subtypes impact test results - place TST or draw blood for IGRA at same clinic visit as live vaccine or wait 4-6 weeks before testing

An 18-month-old boy is hospitalized for dehydration caused by acute gastroenteritis. He was previously healthy and is fully vaccinated, including an MMR vaccination at 15 months of age. He has a temperature of 37.5°C, blood pressure of 89/48 mm Hg, heart rate of 125 beats/min, respiratory rate of 28 breaths/min, and oxygen saturation of 100% on room air. He appears tired and has tacky mucous membranes. Earlier that morning while in the emergency department, he had a prolonged exposure to another child who has been confirmed to have measles. Of the following, the MOST appropriate management of this boy's exposure is to give

measles vaccine - Vaccination is the intervention of choice for vaccine-eligible individuals exposed to measles who have not been vaccinated or have only received 1 dose of a measles-containing vaccine. - The most appropriate management for the 18-month-old boy in the vignette with a significant exposure to measles, who has received 1 dose of measles vaccine, is administration of a second dose of measles vaccine. - Ninety percent of susceptible individuals exposed to measles develop disease. Vaccination is the intervention of choice for vaccine-eligible individuals exposed to measles that have not been vaccinated or have only received one dose of a measles-containing vaccine. The vaccine should be administered within 72 hours of the exposure. - Two doses of MMR are needed to have presumed immunity to measles. Approximately 96% of individuals develop measles antibodies after 1 dose of vaccine. A second dose of vaccine is recommended to capture the small percentage of people who do not respond to the first dose. - The first dose of MMR is recommended at 12 to 15 months of age, and the second dose is recommended between 4 to 6 years of age. - Although the MMR vaccine is typically administered starting at 12 months of age in the United States, it has been used in infants as young as 6 months of age if international travel is anticipated or during epidemics. However, 6-month-old infants may experience delays in humoral immune responses to MMR vaccine compared with older infants. Therefore, an MMR vaccine administered prior to 12 months of age does not count towards the 2 recommended doses. - For catch-up immunizations or in the case of a significant exposure, a second dose of vaccine can be administered 28 days after the previous dose.

Live-attenuated vaccines routinely recommended for children and adolescents include

measles, mumps, and rubella (MMR) rotavirus varicella

The girl in the vignette has recurrent sinopulmonary infections and a history of unexplained neonatal respiratory distress, together a hallmark of primary ciliary dyskinesia (PCD). Based on the 2018 Clinical Practice Guideline for diagnosis of PCD , the preferred test in a patient five years of age or older with at least two of the four key clinical features and for whom cystic fibrosis (CF) has been ruled out, is

measurement of exhaled nasal nitric oxide The four key clinical features of PCD are 1) year round, daily, productive cough 2) year round, daily non-seasonal rhinitis 3) neonatal respiratory distress in a term infant 4) laterality defects (eg, situs inversus totalis)

Bubbly-appearing stool filling the bowel in newborn suggestive of...

meconium ileus and CF

Girl has multiple endocrine neoplasia type 2B (MEN2B). Her mouth lesions are mucosal neuromas. The mucosal neuromas; tall, thin body habitus; full lips; and joint laxity are all phenotypic features of MEN2B. Her intermittent constipation and diarrhea are typical symptoms of associated gastrointestinal tract ganglioneuromas. Alacrima from birth is also a feature of MEN2B. The best next step is to evaluate for

medullary thyroid carcinoma - occurs in 100% of individuals with MEN2B, is aggressive, and metastasizes early - other than resection, effective treatment options for MTC are limited - American Thyroid Association recommends prophylactic thyroidectomy in the first year after birth for those with MEN2B - evaluation for MTC includes thyroid ultrasonography and a serum calcitonin level test. Calcitonin is produced by the neuroendocrine C cells of the thyroid and is elevated in MTC.

A 12 yr old girl presents with edema and gross hematuria. Her evaluation reveals serum creatinine 1.4 mg/dL, serum albumin 2.3 mg/dL, 24-hr urine protein excretion 5.5 g, and C3 12 mg/dL. The most likely cause of her nephrotic syndrome is:

membranoproliferative glomerulonephritis - most common in the 2nd decade of life - Serum C3 complement levels are low in the majority of cases - diagnosis of MPGN is made by renal biopsy - Indications for biopsy include nephrotic syndrome in an older child, significant proteinuria with microscopic hematuria, and hypocomplementemia lasting > 2 mo in a child with acute nephritis

A 10 day old child manifests profound coma 1 day after an illness characterized by emesis, poor oral intake, and hypotonia. Laboratory findings reveal 4+ ketonuria; arterial blood gas, 6.9 mm Hg; PCO2, 19 mm Hg; PO2, 95 mm Hg; anion gap, 37 mEq/L; and absolute neutrophil count, 400/mm3. The most likely diagnosis is:

methylmalonic acidemia - lethargy, feeding problems, vomiting, a sepsis-like picture, tachypnea (from metabolic ketoacidosis), and hypotonia may develop in the 1st few days of life and may progress to hyperammonemic encephalopathy, coma, and death if left untreated - infants who survive the first attack may go on to develop similar acute metabolic episodes during a catabolic state such as infection or prolonged fasting or after ingestion of a high-protein diet - such acute events can cause a sudden injury of the basal ganglia (movement centers in CNS), a metabolic stroke, resulting in a debilitating movement disorder - between the acute attacks, the patient usually continues to exhibit hypotonia and feeding problems with failure to thrive, while other complications of the disease occur with age - in milder forms, patients may present later in life with hypotonia, failure to thrive, and developmental delay - Laboratory findings include ketosis, acidosis, anemia, neutropenia, thrombocytopenia, hyperglycinemia, hyperammonemia, hypoglycemia, and the presence of large quantities of methylmalonic acid in body fluids

A 12 yr old girl who recently visited Haiti with her family presents with signs suggestive of cerebral malaria. She did not take malaria prophylaxis. The confirmatory procedure that should be performed immediately is:

microscopic examination of blood films - The diagnosis of malaria is established by identification of organisms on Giemsa-stained smears of peripheral blood or by rapid immunochromatographic assay. - Giemsa stain is superior to Wright stain or Leishman stain. - Both thick and thin blood smears should be examined. - The concentration of erythrocytes on a thick smear is 20-40 times that on a thin smear and is used to quickly scan large numbers of erythrocytes. - The thin smear allows for positive identification of the malaria species and determination of the percentage of infected erythrocytes and is useful in following the response to therapy.

May be the ideal drug for all forms of orthostatic hypotension associated with normal blood volume.

midodrine - exerts its effects on alpha-adrenergic receptors of the arteriolar and venular vasculature, producing vasoconstriction and venoconstriction, respectively --> increases blood pressure through a combination of increased ventricular afterload and enhanced preload (venous return)

A 5-yr-old boy presents with a recurrent history of vomiting. Vomiting episodes last around 48 hr and usually end abruptly. There have been 4 episodes over the past 8 mo. Growth is normal, and the child is well between episodes. Which of the following is most likely to develop in this patient?

migraine equivalent - The onset of cyclic vomiting is usually between 2 and 5 yr of age but has been observed in infants and adults. - The frequency of vomiting episodes is variable (average of 12 episodes per yr) with each episode typically lasting 2-3 days and 4 or more emesis episodes per hour. - The episodes usually occur in the early hours of the morning or upon wakening. - Patients can have a prodrome of nausea, pallor, intolerance of noise or light, lethargy, and headache. - Several theories have been proposed as causative factors, including a migraine-related mechanism, mitochondrial disorders, and autonomic dysfunction. - More than 80% of affected children have a 1st-degree relative with migraines; many patients develop migraines later in life.

A 15-year-old adolescent girl with respiratory distress is brought to the emergency department. Her parents state that she started to have signs of a mild respiratory tract infection 2 weeks ago, but her condition suddenly worsened today. She appears mottled and in moderate respiratory distress. She has a temperature of 37.5°C, a heart rate of 155 beats/min, a respiratory rate of 20 breaths/min, a blood pressure of 105/65 mm Hg, and an oxygen saturation of 90% on room air. She is awake but somnolent. Her lung examination reveals diffuse rales and rhonchi but no wheezes. Auscultation of the heart reveals a quiet precordium with a grade 3/6 systolic murmur and a grade 2/6 diastolic murmur. Her abdomen is soft, and the liver edge is palpable 5 to 6 cm below the right costal margin. Her skin is mottled and cool with barely palpable distal pulses. Capillary refill time is 5 sec. There is 2+ edema in both legs up to the knee. The remainder of the examination is unrevealing. Oxygen is started, vascular access is established, and blood is drawn for laboratory evaluation. Of the following, the MOST appropriate medication infusion to start is

milrinone - The patient in the vignette has signs of acute heart failure and cardiogenic shock. - On the basis of the viral prodrome described, the most likely diagnosis is acute infectious or postinfectious myocarditis and heart failure. - Given the severity of the girl's symptoms, medical cardiac support is immediately warranted, and the best medication listed is milrinone. Milrinone - phosphodiesterase 3 inhibitor that increases cardiac contractility and decreases pulmonary and systemic vascular resistance, thus improving overall cardiac output - Phosphodiesterase 3 inhibitors prevent the breakdown of cyclic adenosine monophosphate, which ultimately results in increases in calcium availability to the myocyte sarcomere. The increased calcium availability results in increased cardiac inotropy, and the increased calcium reuptake into the sarcoplasmic reticulum also leads to enhanced myocardial relaxation and improved diastolic function - Increases systolic contractility, improves diastolic relaxation (lusitropy) and also increases both pulmonary and peripheral vasodilation. These effects, in total, result in increased cardiac output via improvement of ventricular performance and a reduction in afterload.

The most common abdominal mass in a neonate is:

multicystic dysplastic kidney - congenital condition in which the kidney is replaced by cysts and does not function - can result from ureteral atresia. Kidney size is highly variable. - usually is unilateral and generally is not inherited - most common cause of an abdominal mass in the newborn, but the vast majority are nonpalpable at birth. - in most cases it is discovered incidentally during prenatal ultrasonography

You suspect narcolepsy in an adolescent patient who complains of persistent daytime sleepiness and who is noted by his teacher to "stare off" in class periodically. A polysomnogram is normal. What is the next step in diagnosis?

multiple sleep latency test - Overnight polysomnography (PSG) followed by a multiple sleep latency test (MSLT) are strongly recommended components of the evaluation of a patient with profound unexplained daytime sleepiness or suspected narcolepsy. - The purpose of the overnight PSG is to evaluate for primary sleep disorders, such as OSA, that may cause EDS. - The MSLT involves a series of 5 opportunities to nap (20 min long), during which narcoleptics demonstrate a pathologically shortened sleep-onset latency as well as periods of REM sleep occurring immediately after sleep onset.

All children and adolescents with T1D should self-monitor blood glucose (SMBG) levels how often?

multiple times daily, including pre-meal, pre-bedtime, and as needed for safety in specific clinical situations such as exercise, driving, or for symptoms of hypoglycemia - Continuous glucose monitoring (CGM) should be considered in children and adolescents with T1D, whether using injections or continuous subcutaneous insulin infusion - • The majority of children and adolescents with T1D should be treated with intensive insulin regimens, either via multiple daily injections or continuous subcutaneous insulin infusion

If infant does not subsequently make respiratory efforts *and* if mother has received narcotics within 1 hour of delivery, give...

naloxone hydrochloride (0.1 mg/kg IV, IM, or SQ) - don't give naloxone if there is persistent bradycardia - duration of action is shorter than narcotics --> additional dose may be necessary

A 6-week-old infant girl is seen in the office for evaluation of a 4-day history of cough, posttussive emesis, and fussiness. The mother reports that the infant became apneic and limp for a brief period after a coughing episode at home. Her grandmother has had a chronic cough for more than 1 month. The infant has a temperature of 37.2°C, a heart rate of 148 beats/min, a respiratory rate of 37 breaths/min, a blood pressure of 82/44 mm Hg, and an oxygen saturation of 96% on room air. Findings of a respiratory examination are notable for bilateral coarse breath sounds. Several episodes of transient apnea are observed. Findings from the rest of the physical examination are unremarkable. Rapid viral antigen testing via direct fluorescent antibody is negative for respiratory syncytial virus. Of the following, the BEST next diagnostic step in the evaluation of this infant's illness is

nasopharyngeal swab for polymerase chain reaction - Pertussis in young infants can have an atypical presentation characterized by gagging, gasping, apnea, or bradycardia without the characteristic "whoop." - Children younger than 1 year have the highest reported rate of pertussis; infants younger than 6 months are at risk of experiencing severe disease and death. - Immunization is the key strategy to prevent pertussis.

A 17-year-old soccer player who was diagnosed with a concussion 3 weeks ago is evaluated for clearance to return to sport. She has had full resolution of symptoms and remains asymptomatic with walking and light strength training. She has returned to her previous level of academic achievement in school. This is her third concussion. Her first concussion was at 14 years of age, and her second concussion was 6 months ago. She recovered uneventfully from both of these injuries and is otherwise healthy. She is eager to return to practice and would like to be cleared for sports participation, but her mother is concerned about the risk of another concussion. Her neurologic examination findings are unremarkable. Of the following, the MOST appropriate advice for this patient is that she

needs to perform a trial of high-intensity exercise and report any return of symptoms - Return to sports after concussion requires resolution of symptoms at rest and with exertion, as well as return to baseline level of cognitive achievement. - Recommendations regarding retirement from contact sports after repeat concussion should be made on an individualized basis.

A bright 14 yr old boy has had persistent right leg pain for 6 mo after a minor soccer injury has healed. He describes the pain as sharp, shooting, and burning. Results of all imaging studies are normal. He is able to walk on the leg and is attending school. The type of pain the patient is experiencing is most likely to be:

neuropathic - caused by abnormal excitability in the peripheral or central nervous system that may persist after an injury heals or inflammation subsides. - can be acute or chronic, is often described as burning or stabbing and may be associated with cutaneous hypersensitivity (allodynia). N - may be responsible for >35% of referrals to chronic pain clinics, conditions that commonly include post-traumatic and postsurgical peripheral nerve injuries, phantom pain after amputation, pain after spinal cord injury, and pain due to metabolic neuropathies. - typically responds poorly to opioids. - in adults, evidence suggests the efficacy of TCAs (nortriptyline, amitriptyline) and anticonvulsants (gabapentin, pregabalin) for treatment of neuropathic pain.

An infant has pustules without surrounding erythema concentrated on the face and extremities and small round hyperpigmented macules, some of which are surrounded by scale. These findings are consistent with a diagnosis of transient neonatal pustular melanosis (TNPM). Microscopic examination of pustule contents would reveal

neutrophils but no organisms - self-limited disorder of unknown cause that occurs most often in African American infants - condition begins in utero as sterile pustules that may rupture or remain intact after delivery - when pustules rupture, the infant is left with round hyperpigmented macules (the bases of pustules) that often are surrounded by a rim or collarette of scale (the remnants of pustule roofs) - lesions of TNPM may be widespread but tend to be concentrated on the forehead, chin, neck, lower back, and shins - pustules resolve 24 to 48 hours after birth and hyperpigmented macules fade in several weeks to months

A 5-year-old previously healthy, developmentally normal boy is brought to the emergency department after having a first-time seizure. His mother reports hearing a strange noise from his room and finding him lying on the floor, unresponsive with generalized shaking. His eyes were open and deviated to one side. She called emergency medical services and by their arrival, 15 minutes later, the seizure had stopped. A capillary blood glucose level was 90 mg/dL (5.0 mmol/L). Lorazepam 0.1 mg/kg was administered intravenously en route to the emergency department with placement of an intravenous line. His current vital signs are a temperature of 37°C, heart rate of 90 beats/min, respiratory rate of 16 breaths/min, blood pressure of 90/75 mm Hg, and pulse oximetry of 97% on room air. He is obtunded and moans to sternal rub but does not localize painful stimuli. His pupils are equal, round, and reactive to light. His face is symmetric at rest and with grimace. He moves his arm and leg to noxious stimuli, vigorously on the right but minimally on the left. His deep tendon reflexes are 2/4 throughout, with toes downgoing bilaterally to plantar stimulation. Of the following, the BEST next step in his diagnostic evaluation is

noncontrast head CT - For the boy in this vignette with a persistent postictal left hemiparesis, the next most appropriate step is to obtain noncontrast head computed tomography to evaluate for a structural etiology and/or acute intracranial process. In children presenting with status epilepticus (SE), in particular with persistent focal deficits and encephalopathy, urgent head imaging is recommended once the patient has been medically stabilized.

measures fetal heart accelerations during a 20-minute period to assess fetal well-being

nonstress test - An abnormal nonstress test result should be followed by a biophysical profile. - Both the nonstress test and biophysical profile have low false-negative rates. - The most common cause of fetal distress is uteroplacental insufficiency.

A 4 yr old with severe vomiting due to suspected partial small bowel obstruction is moderately dehydrated and requires IV hydration. The appropriate bolus fluid is:

normal saline - Child with dehydration needs acute intervention to ensure that there is adequate tissue perfusion. This resuscitation phase requires rapid restoration of the circulating intravascular volume and treatment of shock with an isotonic solution, such as normal saline (NS) or lactated Ringer (LR). - The child is given a fluid bolus, usually 20 mL/kg of the isotonic fluid, over approximately 20 min.

A previously healthy, 13-year-old adolescent boy with an abrupt onset of emesis, abdominal cramps, and watery diarrhea is seen in the emergency department. One day ago, he visited a local park with his younger siblings. The public park has a picnic ground, lake, paddle boats, and a splash pad. The patient's siblings who swam at the lake with him are also ill with a similar gastrointestinal illness. He has a temperature of 38.1°C, dry mucous membranes, and sunken eyes. Capillary refill time is 3 seconds. The rest of the examination findings are unremarkable. Laboratory evaluation reveals a serum sodium concentration of 128 mEq/L (128 mmol/L). Of the following, the MOST likely cause of this illness is

norovirus - Enteric pathogens associated with untreated recreational water-associated outbreaks include norovirus, Escherichia coli, Shigella, Cryptosporidium, and other enteric pathogens. - Noroviruses are highly contagious and easily transmitted through fecal-oral or vomitus-oral routes, via consumption of contaminated water or food, or by direct contact with contaminated objects or surfaces. - Norovirus illness often begins with the abrupt onset of vomiting associated with watery diarrhea, abdominal cramps, and nausea.

A 6-week-old male infant born at 32 weeks' gestational age has been hospitalized in the neonatal intensive care unit since birth. He has multiple congenital anomalies including nonshunted hydrocephalus and agenesis of the corpus callosum. He has had 4 episodes of fever. With the first episode, results of a full evaluation for a serious bacterial infection, including cerebrospinal fluid evaluation, were negative. With later episodes, blood cultures, urine cultures, and respiratory viral panels have been negative. His vital signs today include a temperature of 38.5°C, heart rate of 145 beats/min, respiratory rate of 30 breaths/min, and blood pressure of 75/44 mm Hg. Physical examination reveals a well-appearing infant with macrocephaly; a soft anterior fontanelle; a nasogastric tube; a II/VI systolic murmur audible throughout the precordium, axillae, and back; a soft but protuberant abdomen with no masses or enlargement of the liver or spleen; and warm extremities with capillary refill of less than 3 seconds. Of the following, the BEST next step in the evaluation of this infant includes

observation - Has had 4 previous evaluations for serious bacterial infections, which did not reveal a bacterial source of fever. In addition, he is clinically stable and well-appearing. - The historical information and his current appearance, in the context of known central nervous system abnormalities, should raise concern for fever of central origin. - The hypothalamus is the brain's thermoregulatory center. The posterior portion of the hypothalamus controls heat conservation whereas the anterior portion controls heat dissipation. Thus, lesions in the posterior hypothalamus are associated with hypothermia and conversely, lesions in the anterior hypothalamus are associated with hyperthermia. - Shapiro syndrome is a rare disorder characterized by episodic hypothermia, hyperhidrosis, and agenesis of the corpus callosum. Reverse Shapiro syndrome, characterized by episodic hyperthermia in the setting of agenesis of the corpus callosum, is a rare cause of fever of unknown origin. Though poorly understood, the absence of the corpus callosum in both Shapiro and reverse Shapiro syndrome is believed to lead to hypothalamic dysfunction and thus altered thermoregulation.

A neonate with petechiae is being evaluated 15 hours after birth. She was born vaginally, at term, 20 hours after rupture of membranes, to a 26-year-old gravida 2, para 0 woman whose noninvasive prenatal screening was positive for trisomy 21. She had declined confirmatory testing. Group B Streptococcus screening was negative. On physical examination, the neonate has facial features consistent with trisomy 21, normal respiratory effort, mild generalized hypotonia, and 10 petechiae scattered over the abdomen. No murmur is heard. A complete blood cell count from a central sample shows a white blood cell count of 16,000/μL (16 × 109/L), hemoglobin of 23 g/dL (230 g/L), hematocrit of 69%, and platelet count of 125 × 103/μL (125 × 109/L). She has breastfed 3 times and taken 15 mL of formula. Her mother reports 1 stool and 1 wet diaper. Of the following, the BEST next management step for this neonate is to

observe closely Trisomy + polycythemia but clinically asymptomatic - neonates with polycythemia and Hct values of 65-69% who are clinically asymptomatic should be closely observed - polycythemia with Hct >70% should be treated with partial exchange transfusion

A 14 yr old girl has a 6-mo history of recurrent abdominal pain. The pain is characterized as periumbilical to epigastric, lasting 20 to 30 min, with a frequency of 2 or 3 times per week. The pain is relieved following the passage of stool. Her mother has had similar symptoms intermittently since the age of 12 yr. There has been no fever, rash, joint symptoms, or weight loss. No blood has been noted in the stool. The most appropriate next step in the evaluation or management of this patient would be:

obtain celiac serology Irritable bowel syndrome (IBS) - multiple diagnostic criteria with varying durations of symptoms ranging from ≥ 3 mo to > 2 yr - abdominal pain is episodic, cramping, or aching, usually in the lower abdomen, and often relieved by defecation. - it is important to rule out organic causes of abdominal pain and altered bowel patterns, especially celiac disease, even in the absence of the classic features of this disease

A 2-hour-old male neonate is being seen in the well-child nursery for feeding difficulties. He was born via normal spontaneous vaginal delivery at 40 weeks' gestation to a primigravida mother who has been healthy and had an uncomplicated pregnancy. His mother is reporting difficulty with latching and notes that his tongue seems to be anchored to the bottom of his mouth. Of the following, the BEST management approach for this infant-mother dyad is to

offer lactation support - For feeding problems in healthy breastfeeding infant-mother dyads, lactation support should be offered before any other intervention. - Evidence for procedural correction of shortened oral frenulum in healthy neonates, infants, and children is lacking; the decision to perform such a procedure should be made with open communication among the patient's parents, primary care physician, and specialists.

The leading cause of death among drug users is:

opiate overdose - The overdose syndrome is an acute reaction after the administration of an opiate. - It is the leading cause of death among drug users. - The clinical signs include stupor or coma, seizures, miotic pupils (unless severe anoxia has occurred), respiratory depression, cyanosis, and pulmonary edema.

A 2-year-old girl with a history of chronic neutropenia and a chief concern of fever of 41°C is being evaluated at the office. The parents ask what the best method of measuring her temperature is. Of the following, the PREFERRED method of thermometry in this girl is

oral - For non-neutropenic patients, rectal thermometry is the preferred method of measurement during the first few years of age, because it offers the best approximation of core temperature. However, accuracy may be affected by poor technique, stool in the rectum, or poor perfusion. - For children older than 3 to 4 years, oral thermometry is generally preferred because of the physical and psychological discomfort of rectal thermometry. Oral temperature is approximately 0.6°C lower than rectal temperature, but the relationship is not consistent. The accuracy of oral measurement may be affected by technique, recent ingestion of hot or cold foods or drinks, or tachypnea. Although axillary thermometry is the least discomforting and most convenient method, it is the least consistent with rectal thermometry.

The recommended treatment for Chlamydia trachomatis conjunctivitis is:

oral erythromycin for 2 weeks - erythromycin (base or ethylsuccinate, 50 mg/kg/day divided 4 times a day PO for 14 days) - failure rate with oral erythromycin remains 10-20%, and some infants require a 2nd course of treatment - an association between treatment with oral erythromycin and infantile hypertrophic pyloric stenosis has been reported in infants < 6 wk of age who were given the drug for prophylaxis after nursery exposure to pertussis

A 3-year-old boy with optic nerve hypoplasia is brought to the office by his mother for a health supervision visit. There has been no evidence of pituitary dysfunction and no additional abnormalities were noted on brain magnetic resonance imaging. The ophthalmologist documented bilateral visual acuity of 20/200. The boy had some initial mild delay in his language development, but is now meeting age-appropriate speech and language milestones. His other domains of development are also age appropriate. His physical examination is notable for nystagmus and small optic discs. The boy's mother asks which services or supports should be included in his Individualized Education Program. Of the following, the BEST response to the parent's question is

orientation and mobility training - essential to ensure that children with visual impairment are able to navigate their environment safely and effectively - children are taught to determine their body's position, develop motor skills to balance and move, protect themselves, use systematic search patterns, and plan routes - they are also taught to use their other sensory systems (eg, auditory, vestibular, tactile) to aid in their safe and independent mobility - this instruction is provided by a certified orientation and mobility specialist (COMS)

What kind of blood pressure abnormality is more common in fibromyalgia patients when compared to the general population?

orthostatic hypotension

A 23-month-old girl with fever is seen in the clinic in December. She had been in good health until 2 days ago when she developed rhinorrhea and a mild cough. Last night she woke up crying and had a temperature of 39°C. She received a dose of ibuprofen 3 hours ago. No one has been sick at home, but 2 children in her day care room have been diagnosed with influenza in the last week. She received her first dose of influenza vaccine 3 weeks ago. She has a temperature of 38°C, blood pressure of 98/56 mm Hg, heart rate of 105 beats/min, and respiratory rate of 26 breaths/min. She appears well nourished and is in no distress. There is clear drainage from both nares, and the lungs are clear to auscultation. Of the following, the BEST next step in management is

oseltamivir treatment - The American Academy of Pediatrics recommends antiviral treatment for confirmed or suspected influenza infection in any individual at high risk for complications. Children younger than 5 years, especially those younger than 2 years, are at increased risk of hospitalization and complications due to influenza.

A 10-year-old boy presents with a limp. He developed pain in his left thigh two days ago, and it has worsened since. He also developed a fever this evening. On examination, he has point tenderness of the proximal femur. He has full active range of motion of the hip. He has an antalgic gait that favors the right leg. Radiography of the hips and femur are normal. What is the most likely diagnosis?

osteomyelitis

A 15 yr old female basketball player sustained a knee injury 4 wk previously. Initial radiograph of the knee was normal. She has been receiving physical therapy for the past 3 wk with no relief of her pain. Repeat examination shows diffuse swelling around the knee, which is most prominent in the distal femur. Repeat radiograph demonstrates a sunburst pattern in the tibia. The most likely diagnosis is:

osteosarcoma - pain, limp, and swelling are the most common presenting manifestations - occur most often in active adolescents - initial complaints may be attributed to a sports injury or sprain - any bone or joint pain not responding to conservative therapy within a reasonable time should be investigated thoroughly - additional clinical findings may include limitation of motion, joint effusion, tenderness, and warmth - classic radiographic appearance of osteosarcoma is the sunburst pattern

A 17-year-old adolescent boy, who was pitching for his high school baseball team, is brought to the emergency department for evaluation after being hit in the face by a line-drive several hours earlier. After sustaining the trauma, he fell immediately to the ground but did not lose consciousness. There was bleeding from both nares, which resolved after 15 minutes of pressure to the nasal bridge. In the emergency department, he appears well, alert, and oriented and has normal vital signs. His pupils are equal, round, and reactive to light. There is no periorbital swelling or ecchymosis and no subconjunctival hemorrhages. There is mild to moderate swelling of the nasal bridge, but the nasal dorsum is not flattened. He can breathe through each nare when the contralateral nare is temporarily occluded. Protruding bilateral reddish masses are seen in the nares. There is no malocclusion or loose teeth. Of the following, the BEST next step in management is

otorhinolaryngologist consultation - The adolescent in this vignette has bilateral septal hematomas after sustaining midface trauma. - The hallmark finding of a septal hematoma on direct rhinoscopy is a reddish or bluish protuberance emanating from the nasal septum. Other common symptoms include pain and nasal obstruction. - Management of a nasal septal hematoma includes an emergent consultation with an otorhinolaryngologist for emergent drainage. Failure to expeditiously drain a septal hematoma may result in necrosis of the nasal septum in as little as 72 to 96 hours, secondary to obstruction of blood flow from the mucoperichondrium to the cartilage. This can lead to a permanent deformity (typically a saddle-nose deformity). - Nasal septal hematomas result from arterial injury between the cartilaginous nasal septum and the overlying mucoperichondrium. Adequate examination of the nasal cavity is crucial after any degree of midface trauma in children as septal hematomas can occur even after minor trauma. - If the antecedent trauma also caused epistaxis it may be necessary to suction the nasal cavity to ensure adequate evaluation of the nasal septum. - Use of a topical vasoconstrictor (eg, oxymetazoline) may facilitate visualization of the nasal septum. - In a cooperative adolescent, drainage of a nasal septal hematoma can be accomplished under local analgesia; however, younger patients may require general sedation and drainage in an operating room.

Initial laboratory studies to investigate for metabolic disease in an ill infant should include:

pH, bicarbonate, ammonia, lactate - elevated blood ammonia level is usually caused by defects of urea cycle enzymes. Infants with elevated blood ammonia levels from urea cycle defects commonly have normal serum pH and bicarbonate values; without measurement of the blood ammonia level their defect may remain undiagnosed and they may succumb to their disease. - In organic acidemias, elevated plasma ammonia is accompanied by severe acidosis caused by accumulation of organic acids, ketone bodies, and lactate in body fluids. - When blood ammonia, pH, and bicarbonate values are normal, other aminoacidopathies (e.g., hyperglycinemia) or galactosemia should be considered; galactosemic infants also may manifest cataracts, hepatomegaly, ascites, and jaundice.

Treatment of osteogenesis imperfecta (OI) is not curative. Nonetheless, improvement with decreased bone fractures and pain has been reported with:

pamidronate - A several-year course of treatment of children with OI with bisphosphonates (IV pamidronate or oral olpadronate) confers some benefits. - Bisphosphonates decrease bone resorption by osteoclasts; OI patients have increased bone volume that still contains the defective collagen. - Bisphosphonates are more beneficial for vertebrae (trabecular bone) than long bones (cortical bone). Treatment for 1-2 yr results in increased L1-4 DEXA and, more importantly, improved vertebral compressions and area, which can prevent or delay the scoliosis of OI.

A 10 yr old boy develops pancreatitis after a blow to the abdomen. During a 5-day hospitalization he is treated with intravenous fluids and analgesics and is discharged home on a low-fat diet. Four weeks later he has recurrence of abdominal pain and vomiting. The most likely diagnosis is:

pancreatic pseudocyst - suggested when an episode of pancreatitis fails to resolve or when a mass develops after an episode of pancreatitis - most common signs are a palpable mass in 50% of patients and jaundice in 10% - other findings include ascites and pleural effusions (usually left sided) - recommended that the patient with acute pancreatitis undergo an ultrasonographic evaluation 2-4 wk after resolution of the acute episode for an evaluation of possible pseudocyst formation

An 18 yr old girl presents with signs and symptoms of appendicitis. Her past medical history is significant only for sexual activity and placement of an IUD 3 yr previously. She undergoes an appendectomy, in which her appendix is found to be normal. One month postoperatively, she has local pain and has an irregular, hard mass in her ileocecal area. Biopsy of the mass shows branching, filamentous rods. The most likely diagnosis is:

pelvic actinomycosis - Patients often present with a history of gastrointestinal surgery, diverticulitis, or appendicitis. - The clinical course is indolent, with chills, fever, night sweats, and weight loss, and the presentation is similar to that of tuberculous peritonitis. - Women using IUDs are at risk for development of pelvic actinomycosis, which classically manifests as vaginal discharge, pelvic pain, abdominal pain, menorrhagia, fever, pelvic mass, and a history of pelvic inflammatory disease. - The risk is higher if the IUD has been in place for > 2-3 yr.

A 4-month-old male infant is referred to the emergency department by his pediatrician for weakness in his legs. He was born full term via vaginal delivery following an uncomplicated pregnancy. He was well with normal feeding, growth, and development until 3 weeks ago. At that time, his mother noticed that he would not push his feet against her hands as hard as he used to. A video from the mother taken before that time shows the infant jumping in a bouncer, using both legs to push off. The day after the video was taken, he received his 4-month vaccines in both legs, and since that time he stopped planting his feet on the ground when held up, and he has had decreasing spontaneous movement of his lower extremities. His upper body movements and strength have been normal. He has had no fever, weight loss, irritability, sick contacts, or exposure to honey. He has a temperature of 37.2°C, heart rate of 127 beats/min, blood pressure of 98/50 mm Hg, respiratory rate of 44 breaths/min, and oxygen saturation of 99% on room air. He appears happy and playful, reaching for and pulling his mother's hair. His physical examination is remarkable for flaccid paralysis of both lower extremities, with clonus in both feet. He has normal strength and activity in his arms, hands, and head. Other than the lower extremity neurologic examination findings, there are no other abnormal findings. Of the following, the MOST appropriate next step is to

perform emergent magnetic resonance imaging of the spine - The rapid recognition, diagnosis, and treatment of spinal cord compression is essential to provide the best opportunity for recovery of neurologic function. - The infant has flaccid paralysis of both legs with clonus but no other abnormal physical findings. - His mother has provided video evidence of previously normal lower extremity function, suggesting that the paralysis is acquired and of relatively recent onset. - The bilateral nature of the neurologic deficit strongly suggests a lesion of the spinal cord, likely in the thoracic cord. - The differential diagnosis includes diseases intrinsic to the spinal cord, such as transverse myelitis, as well as external compression. While the treatment of transverse myelitis is urgent, decompression of the spinal cord when impinged by an external lesion is emergent. Every minute that passes with the cord compressed decreases the likelihood of achieving a neurologic recovery. - When spinal cord compression is suspected, it is imperative to obtain emergent magnetic resonance imaging so that action can be taken to decompress the cord. Thus, the most appropriate next step for the infant in the vignette is emergent magnetic resonance imaging of the spine. - In an otherwise well infant with new-onset bilateral lower extremity weakness, a neuroblastoma should be suspected. Neuroblastoma is the most common extracranial solid tumor in children. It is a tumor of the sympatico-adrenal axis and can arise from the paraspinal ganglia. It can invade through the spinal neural foramina and create an epidural mass causing spinal cord compression. - While decompression of the spinal cord can be accomplished through surgery, paraspinal neuroblastomas in infants often span multiple spinal levels and would require a multi-level laminectomy to decompress. As neuroblastoma typically responds very quickly to chemotherapy, it would be appropriate to emergently treat a child with this type of tumor with emergent chemotherapy.

Which form of dialysis is the best option for an adolescent with chronic renal insufficiency and heparin allergy?

peritoneal dialysis - most commonly employed in neonates and infants with ARF, although this modality may be used in children and adolescents of all ages.

A 7-year-old boy with a history of renal dysplasia and chronic kidney disease is seen for a health supervision visit. He has a history of increased thirst and polyuria. His current medications include sodium bicarbonate, ferrous sulfate, calcium carbonate, and calcitriol. His weight is at the fifth percentile, and his height is at the third percentile. He has a heart rate of 89 beats/min, a respiratory rate of 16 breaths/min, and a blood pressure of 90/60 mm Hg. The rest of the examination findings are unremarkable. Of the following, the MOST appropriate dietary restriction for this child is

phosphorous CKD - can cause urinary phosphate retention and hyperphosphatemia - he needs dietary restriction of phosphorus to maintain a serum phosphorus level in the normal range and to control hyperparathyroidism - nutritional status should be evaluated on a periodic basis, because it affects growth and neurocognitive development

Weakest part of the growing skeleton

physis - forces that produce sprain or dislocation in adult are more likely to cause physeal fracture in child

For foreign-body aspiration in an infant, what do you do?...

place infant over arm or rest on lap --> give five back blows between scapulae if unsuccessful, turn infant over and give five back thrusts (NOT abdominal thrusts) after back, chest, and/or abdominal thrusts, open mouth using attempt blind finger sweeps - magill forceps may be used to retrieve objects in posterior pharynx ventilate if unconscious --> repeat sequence if needed

A neonate with micrognathia and a high arched palate has signs of respiratory distress. The initial management response should be:

place the neonate in prone position Pierre Robin syndrome - consists of micrognathia usually accompanied by a high arched or cleft palate - tongue is usually of normal size, but the floor of the mouth is foreshortened - air passages can become obstructed, particularly on inspiration, usually requiring treatment to prevent suffocation - infant should be maintained in a prone or partially prone position so that the tongue falls forward to relieve respiratory obstruction - some patients require tracheostomy - mandibular distraction procedures in the neonate can improve mandibular size, enhance respiration, and facilitate oral feedings

A one-day-old male infant is noted in the well-baby nursery to have bruising and bleeding at the site of a heel stick. He is afebrile and otherwise seems to be doing well. This is the family's first child. He was born full term and a prenatal infectious disease evaluation was negative. His birth weight was 3594 g. His vital signs are normal. On exam, he is sleeping comfortably. Heart and lungs are normal. Abdomen is soft with no organomegaly or tenderness. One heel shows significant petechiae and bruising. Complete blood count reveals a white blood cell count of 17.3 cells/mm3, hemoglobin 18.4 mg/dL, and platelets 21 cells/mm3. You strongly suspect a diagnosis of neonatal alloimmune thrombocytopenia. Which of the following is the best approach to manage this patient?

platelet transfusion Neonatal alloimmune thrombocytopenia - fetus or newborn inherits a platelet antigen, most commonly human platelet antigen 1a, from his or her father. The mother is negative for this antigen and in fact has antibodies to it. These antibodies attack the platelets of the fetus or newborn, sometimes causing severe thrombocytopenia. - feared complication of fetal alloimmune thrombocytopenia is intracranial hemorrhage, which correlates with the severity of the thrombocytopenia and can cause substantial morbidity or mortality. - may occur in a woman's first pregnancy. This patient has severe thrombocytopenia (< 30 cells/mm3). - Patients in the first week of life who have platelets < 50 cells/mm3 and have signs of bleeding or those with platelets < 30 cells/mm3 without signs of bleeding should receive a platelet transfusion, which, despite the alloimmune nature of the condition, has been shown to reliably raise the platelet count. - After the first week of life, the transfusion threshold for patients without signs of bleeding drop to <20 cells/mm3

A 5 mo old infant with gross motor delay, hypotonia, and cardiomegaly has a creatine kinase level of 860 IU/L. Of the following, which is the most likely diagnosis?

pompe disease - infants present in the 1st few months of life with hypotonia, a generalized muscle weakness, neuropathic bulbar weakness, feeding difficulties, macroglossia, hepatomegaly, and a hypertrophic cardiomyopathy - laboratory findings include elevated levels of serum creatine kinase, aspartate aminotransferase, and lactate dehydrogenase - chest radiography shows massive cardiomegaly

An 18-hour-old male newborn has not voided since birth. He was delivered at 38 weeks' gestation to a 25-year-old primigravida mother via cesarean delivery. Prenatal records indicate that she had oligohydramnios. The neonate's vital signs are stable. He has suprapubic swelling. The remainder of the physical examination findings are unremarkable. Renal ultrasonography shows bilateral hydronephrosis with ureteronephrosis and a distended urinary bladder. Of the following, the MOST likely diagnosis in this neonate is

posterior urethral valves - The neonate in this vignette has features suggestive of a distal obstructive uropathy. The history of delayed passage of urine and a distended urinary bladder with bilateral hydroureteronephrosis on renal ultrasonography in this male neonate favors the diagnostic possibility of posterior urethral valves (PUV). - A male infant with bilateral hydroureteronephrosis and a distended urinary bladder should undergo a voiding cystourethrogram to evaluate for posterior urethral valve. - Urinary retention, poor urinary stream, urinary incontinence, urinary tract infection, hematuria, and failure to thrive are clinical features in children with distal obstructive uropathy. - Delayed passage of urine and a distended urinary bladder with bilateral hydroureteronephrosis on renal ultrasonography favor a posterior urethral valve.

refeeding syndrome. what should you check in that child?

potassium, calcium, phosphate, magnesium - before starting feeds, administer thiamine 200-300 mg daily orally, vitamin B high potency 1-2 tablets 3 times daily (or full dose IV vitamin B), and multivitamin or trace element supplement once daily - start feeding 0.0418 MJ/kg/day - slowly increase feeding over 4-7 days - rehydrate carefully and supplement and/or correct levels of potassium (give 2-4 mmol/kg/day), phosphate (0.3-0.6 mmol/kg/day), calcium, and magnesium (0.2 mmol/kg/day IV or 0.4 mmol/kg/day orally) - monitor potassium, phosphate, calcium, and magnesium for first weeks and amend treatment as appropriate

Contraindications for MMR vaccine include

pregnancy immunosuppression severe allergy to neomycin or gelatin

A 15-year-old girl is brought to the emergency department with right lower quadrant abdominal pain for 48 hours. She reports intermittent, sharp pain that she has not experienced in the past. The adolescent denies vomiting, diarrhea, or fever, but has been feeling nauseated. She has had a decrease in her appetite, but is able to tolerate solids and liquids. The girl had menarche at age 12 years and her menstrual periods are regular. Her last period was 2 weeks ago and lasted for 5 days. She states that she has never been sexually active. Abdominal and pelvic ultrasonography demonstrates a 2.5 × 1.0 × 1.5 cm fluid-filled right ovarian cyst. There is no evidence of appendicitis or other abnormality. Of the following, the BEST next step in this adolescent's evaluation and management would be to

prescribe a NSAID - The adolescent in the vignette most likely has a simple functional ovarian cyst. Her pain can be managed conservatively with a nonsteroidal anti-inflammatory drug.

A 15-year-old adolescent boy is being evaluated for left knee pain of 4 months' duration. The pain began while playing on 2 different basketball teams, but he denies a specific injury. The pain is primarily anterior and medial to the patella, and worsens during and after activity. Several weeks ago he developed intermittent left knee swelling and reports that the knee will occasionally "get stuck" in a given position. He has no other joint complaints and is otherwise healthy. Findings on physical examination of his left knee include a mild effusion, mild tenderness over the anterior medial femoral condyle, and pain with flexion greater than 90 degrees and with resisted extension. The knee is stable to valgus and varus stress testing; Lachman and posterior drawer test results are negative. The adolescent's gait is antalgic. He feels pain with attempts to squat and jump. Of the following, the BEST next management step for this boy is to

prescribe crutches and order radiography of the knee - Imaging studies are warranted for pediatric patients with a knee effusion. - Osteochondritis dissecans must be ruled out in a child with joint swelling and painful mechanical symptoms (eg, locking, catching).

A 2-month-old boy is seen in the office for a sick visit. His mother is concerned that he is breathing fast and eating less. Over the last few days, while breastfeeding, he has tired out sooner, breathed a little faster, and become sweaty. He now seems to be breathing faster when he is at rest and not eating. He does not have rhinorrhea, cough, congestion, fevers, vomiting, diarrhea, or rash. He is afebrile. He has a heart rate of 158 beats/min, a blood pressure of 75/45 mm Hg, and a respiratory rate of 60 breaths/min. He appears comfortable. His oxygen saturation is 95%. His weight remains at the 50th percentile for age. He has a regular heart rate and rhythm with a normal S1 and physiologically split S2. He has a grade 2/6 holosystolic murmur at the left lower sternal border. He has good perfusion and normal pulses. His liver edge is palpated 3 cm below the right costal margin. Of the following, the BEST next step(s) in treatment is (are) to

prescribe furosemide and refer the infant to a pediatric cardiologist - Cardiac lesions with left-to-right shunting can include atrial septal defects, ventricular septal defects, atrioventricular septal defects, aortopulmonary window, and patent ductus arteriosus. - Hemodynamically significant lesions result in cardiac enlargement and pulmonary congestion and warrant repair.

Unexplained neonatal respiratory distress followed by recurrent sinopulmonary infections is a hallmark for

primary ciliary dyskinesia (PCD) - The preferred test for PCD in a patient five years of age or older with at least two of the four key clinical features and for whom cystic fibrosis (CF) has been ruled out, is measurement of exhaled nasal nitric oxide - Phase microscopy of cilia motility is no longer the best test for the diagnosis of PCD - Bronchiectasis in children is most often secondary to recurrent infection.

A 10 yr old white girl has a history of increasingly severe exercise intolerance and fatigability. The day of admission she had a syncopal event. On examination, she has a systolic ejection click and a loud, narrowly split 2nd heart sound. This is a soft systolic murmur. The chest radiograph demonstrates prominent pulmonary arteries and an enlarged right ventricle. The peripheral pulmonary vascular markings are greatly decreased. The most likely diagnosis is:

primary pulmonary hypertension - characterized by pulmonary vascular obstructive disease and right-sided heart failure - predominant symptoms include exercise intolerance and fatigability; occasionally, precordial chest pain, dizziness, syncope, or headaches are noted - heart is moderately enlarged, and a right ventricular heave can be noted - 1st heart sound is often followed by an ejection click emanating from the dilated pulmonary artery - 2nd heart sound is narrowly split, loud, and sometimes booming in quality; it is frequently palpable at the upper left sternal border - presystolic gallop rhythm may be audible at the lower left sternal border - systolic murmur is soft and short and is sometimes followed by a blowing decrescendo diastolic murmur caused by pulmonary insufficiency - in later stages, a holosystolic murmur of tricuspid insufficiency is appreciated at the lower left sternal border - chest roentgenograms reveal a prominent pulmonary artery and right ventricle - pulmonary vascularity in the hilar areas may be prominent, in contrast to the peripheral lung fields in which pulmonary markings are decreased

A term male neonate born to a mother with limited prenatal care has a positive newborn screening result for IgM antibodies specific for Toxoplasma gondii. Further testing in conjunction with an infectious disease specialist confirms congenital toxoplasmosis. The original source of the infection is determined to be undercooked meat consumed by the boy's mother and his 7-year-old sister. The sister has no medical problems and has remained well with no symptoms but is also found to have been infected with T gondii. Of the following, the BEST advice regarding the management of the sister's infection is that she should receive

prophylaxis if she becomes immunocompromised - The girl in this vignette is currently healthy and asymptomatic but is infected with Toxoplasma gondii; this acute infection will likely become latent. If she later becomes immunocompromised, her latent infection may reactivate and cause severe symptoms. She should therefore receive prophylaxis if she becomes immunocompromised.

A 2-year-old boy with no significant medical history is brought to the emergency department with a limp. The limp began 2 days ago and has been associated with fever. His parents are unable to localize the pain. One week prior to this illness, he had nasal congestion and rhinorrhea and fell, resulting in abrasions to his knee and elbow. He has a temperature of 39.1°C, heart rate of 162 beats/min, respiratory rate of 30 breaths/min, and blood pressure of 106/51 mm Hg. He appears ill and grimaces with movement of the right hip. Laboratory data are shown: White blood cell count 16,600/µL (16.6 × 109/L) Neutrophils 51% Bands 20% Lymphocytes 19% Monocytes 10% Hemoglobin 11.8 g/dL (118 g/L) Platelet count 237 × 103/µL (237 × 109/L) C-reactive protein 6.9 mg/dL (657 nmol/L) Erythrocyte Sedimentation rate 29 mm/h Radiographs of the hip are concerning for a right-sided effusion. Of the following, the clinical findings BEST support a diagnosis of

pyogenic arthritis - In the setting of pyogenic arthritis, fever and limited range of motion are almost always present, but external signs (erythema, swelling) vary depending on the site of disease. - High fever, leukocytosis, elevated inflammatory marker levels, and inability to ambulate help distinguish pyogenic arthritis from transient synovitis. - Empiric treatment of pyogenic arthritis in children should include an antimicrobial expected to have activity against Staphylococcus aureus and streptococcal species, and in young children, Kingella kingae should also be covered.

Three adolescents are brought to the emergency department 2 days after attending a political rally at which a small explosion occurred near them. None of the girls sustained any physical injuries, so they did not seek immediate medical care. Later that evening, all three began to have nonbloody nonbilious vomiting, diarrhea, mild headache, and fatigue, which has persisted. In the emergency department, all three adolescents appear well and have normal vital signs. On physical examination, each patient has mild diffuse abdominal tenderness and one appears to be mildly dehydrated. The remainder of their physical examination findings are normal. Intravenous lines are placed and a bolus of normal saline is administered to each adolescent. All three have a decreased absolute lymphocyte count, but laboratory findings are otherwise normal. Of the following, the MOST likely cause of the adolescents' symptoms is exposure to

radiation - Acute radiation syndrome can include gastrointestinal, neurologic, cutaneous, or hematologic signs and symptoms or a combination thereof. - Absolute lymphocyte counts decrease following known patterns after radiation exposure and can be used to deduce approximate timing and doses. - Ionizing radiation is present in nature; however, medical imaging, medical therapies, and certain occupations can increase the risk of exposure.

A 7-year-old boy is receiving a new patient preparticipation sports physical. He has no family history of heart disease. He has been growing and developing normally, eating well, active, and doing well in school. He and his mother have no concerns. His physical examination is remarkable for a low-pitched, musical, 2/6 systolic murmur heard best at the lower left sternal border. When the boy performs a valsalva maneuver the murmur is not heard. There is no click, rub, or gallop. The liver edge is palpable at the right costal margin. Of the following, the BEST management step for this boy is

reassurance - The boy in the vignette has a murmur consistent with the classic description of a Still or innocent murmur. These murmurs are typically low-pitched, located at the lower left sternal border, and are often described as being musical in character. They may change in character or disappear with position changes, and often are not heard during the Valsalva maneuver. These murmurs are common in healthy children aged 3 years to adolescence and are not associated with heart disease.

A 14-year-old previously healthy adolescent girl is brought for follow-up after sustaining a head injury the day prior. She was evaluated in the emergency department after a fall during cheerleading practice that was associated with a brief loss of consciousness. Computed tomography of the head showed no intracranial abnormality, but it did show a nodule in her right thyroid lobe measuring 1.5 cm in greatest diameter. There is no family history of thyroid disease. Physical examination of her thyroid is normal, and the nodule is not palpable. There is no cervical lymphadenopathy. The remainder of her physical examination findings are unremarkable. The results of thyroid function tests are normal. Thyroid ultrasonography confirms a hypoechoic 1.5-cm nodule in the right lobe. Of the following, the BEST next step in evaluating this patient's incidental finding is to:

refer her to a fine needle aspiration biopsy - According to current guidelines, a fine needle aspiration (FNA) biopsy with ultrasonography guidance is indicated to evaluate for thyroid cancer. - Indications for ultrasonography-guided FNA include a nodule of at least 1 cm in diameter or risk factors for malignancy on history, physical examination, or ultrasonography.

A 16-year-old adolescent boy is seen for evaluation 2 days after injuring his right hand. His finger got caught while trying to tackle another player in football practice. Since then, he has had difficulty bending his fourth finger. He is swollen, bruised, and tender on the volar aspect of the distal phalanx. He can actively flex the proximal interphalangeal joint but not the distal interphalangeal joint. The remainder of the hand examination and a radiograph of the fourth finger have normal findings. Of the following, the BEST next step is to

refer him to a hand surgeon for urgent evaluation - adolescent in this vignette has sustained a tear of one of the flexor tendons of his finger and requires prompt evaluation by a hand surgeon. flexor digitorum profundus and flexor digitorum superficialis both contribute to finger flexion, and early recognition of injury to these tendons is important for long-term maintenance of hand function - in adolescents, tears are often the result of sport-related injury to the flexor digitorum profundus during forced extension while the finger is actively flexed. This is commonly called "jersey finger" as it often occurs when attempting to grab the jersey of an opponent who is moving in the opposite direction. The fourth finger is most commonly affected - inspection of both hands in resting position is an important first step in evaluation, particularly in young children who may become uncooperative with direct examination. asymmetry of finger flexion in the resting position or with attempts at grasping or making a fist is a strong indicator of possible flexor tendon injury - torn flexor tendons have the potential to retract and lose their blood supply fairly quickly. if not surgically repaired, these injuries may result in permanent deformity and loss of function. outcomes improve with surgery within 1 week of injury

A 16-year-old basketball player is being evaluated for persistent dizziness. One week ago she collided with another player during a game. She continued to play, but within minutes, developed a significant headache. She was diagnosed with a concussion in the emergency department, and was advised to rest for several days and follow up with her primary care physician. Currently, she is feeling markedly better, other than persistent dizziness and a sense of being "off balance," particularly with positional changes. Her physical examination findings are notable for provocation of dizziness with head movement and position changes. Neurologic findings are otherwise nonfocal. The remainder of her examination findings are unremarkable, including full and pain-free range of motion of the neck. Of the following, the BEST next management step for this girl would be

referral for vestibular rehabilitation - Dizziness in a patient after a concussion is predictive of prolonged recovery. - Patients with persistent vestibular symptoms after a concussion should be identified and referred for rehabilitation. - A brief vestibulo-ocular assessment can be readily performed in the provider's office.

A 3 yr old boy is undergoing mechanical ventilation 12 hr after repair of an atrial septal defect. Opioids and benzodiazepines are being used for analgesia and sedation. The mandatory ventilatory rate has been decreased from 20 to 10 breaths/min in preparation for removal of the endotracheal tube. The PaO2 is 120 mm Hg and the PaCO2 is 75 mm Hg. The arterial pH is 7.13. The child has no spontaneous respirations. Auscultation of the chest demonstrates that the breath sounds are slightly reduced on the left side. Occasional crackles can be heard over both bases. The most likely reason for this child's acidosis is:

respiratory depression - This child has a rising CO2 with corresponding decreased pH in the setting of decreasing respirations. - He is experiencing respiratory acidosis.

A 4 yr old boy presents with urinary obstruction. Ultrasound study reveals a pelvic mass. There is no history of urinary tract infections or other illnesses. The most likely diagnosis is:

rhabdomyosarcoma - most common pediatric soft tissue sarcoma, rhabdomyosarcoma, accounts for approximately 3.5% of childhood cancers - tumors may occur at virtually any anatomic site but are usually found in the head and neck (25%), orbit (9%), genitourinary tract (24%), and extremities (19%); retroperitoneal and other sites account for the remainder of primary sites - involvement of the genitourinary tract can produce hematuria, obstruction of the lower urinary tract, recurrent urinary tract infections, incontinence, or a mass detectable on abdominal or rectal examination

An 8-year-old boy with a 3-day history of low-grade fever, sore throat, nasal discharge, cough, and malaise is seen in the office. He recently returned to school after summer break. He appears well and has a temperature of 38.2°C, a heart rate of 102 beats/min, a respiratory rate of 18 breaths/min, and a blood pressure of 108/67 mm Hg. There is redness of the pharyngeal mucosa without exudate. There is nontender, shotty, anterior cervical adenopathy. His right tympanic membrane is erythematous. The remainder of his physical examination findings are normal. Of the following, the MOST likely etiology of this child's illness is

rhinovirus - most frequently implicated community respiratory viral pathogen causing the common cold in children and adults - occurs throughout the year, with peak activity from autumn through spring. - in addition to the common cold, rhinoviruses can cause otitis media, bronchiolitis, and pneumonia in infants and children

Insert umbilical arterial and venous catheters and measure vascular pressures. Be certain UVC is where before diagnosing elevated CVP?

right atrium - pressure in portal circulation is higher than CVP by variable amount

A 2-year-old boy with trisomy 21 has persistent tachypnea. He has a history of oxygen desaturation with minor viral illnesses, with oxygen saturation as low as 80% on pulse oximetry. He sometimes snores at night, but does not have respiratory pauses or witnessed apneic events. On physical examination, his oxygen saturation in room air is 96%, respiratory rate is 45 breaths/min, and heart rate is 120 beats/min. His breath sounds are clear to auscultation, heart has normal rhythm with no murmur or gallop, and oropharynx shows 2+ tonsils in a small pharynx. Of the following, the MOST likely findings on echocardiography include a

right ventricular hypertrophy with tricuspid valve regurgitation - Down syndrome and pulmonary hypertension, likely related to the congenital pulmonary hypoplasia common to Down syndrome and sleep hypoventilation as manifest by his small pharynx and snoring. Children with pulmonary hypertension have signs of right ventricular overload and dysfunction, and thus the expected echocardiographic findings would include right ventricular hypertrophy and tricuspid valve regurgitation.

A 6-month-old unimmunized female infant is brought to the emergency department in January with a 6-hour history of fever, vomiting, and watery, foul-smelling nonbloody diarrhea. One month before presentation, she and her parents arrived in the United States with refugee status from Syria. There is no history of sick contacts. On physical examination, her temperature is 39°C with a heart rate of 130 beats/min, respiratory rate of 54 breaths/min, and a blood pressure of 75/50 mm Hg. She appears severely dehydrated, with dry mucous membranes and a capillary refill time of about 3 seconds. Stool examination is negative for occult blood. Of the following, the MOST likely pathogen causing this infant's illness is

rotavirus - The unimmunized infant in the vignette has acute gastroenteritis characterized by acute onset of emesis, watery diarrhea, and fever associated with severe dehydration and shock. Of the choices listed, rotavirus is the most likely cause of this infant's symptoms. - Infants and young children 6 months to 2 years of age are at highest risk for severe rotavirus infection. The infection is transmitted via the fecal-oral route from person-to-person contact with infected individuals or contact with contaminated fomites. - The mean incubation period ranges from 1 to 3 days. - The clinical spectrum of rotavirus gastroenteritis can range from asymptomatic infection to life-threatening disease because of shock and acidosis from severe dehydration. - The primary symptoms include abrupt onset of emesis, followed by watery diarrhea and fever. - Electrolyte abnormalities and acidosis can result from severe dehydration. - Unusual manifestations of rotavirus infection include hepatitis and meningoencephalitis. - The duration of rotavirus gastroenteritis is generally 3 to 8 days. - The laboratory diagnosis of rotavirus gastroenteritis can be made by detecting rotavirus antigen in stool specimens using an enzyme immunoassay.

Characterized by effortless and painless nonbilious, nonbloody regurgitation of food within minutes to hours of eating, after which partially digested food is then rechewed, re-swallowed or expulsed

rumination syndrome - whereas pain is not a predominant feature of rumination syndrome, dyspeptic symptoms such as epigastric or pharyngeal burning may exist - mild weight loss is relatively common in rumination syndrome, but other features indicative of an eating disorder—such as malnutrition, dental enamel erosion, electrolyte abnormalities, and vital sign abnormalities—are absent - medication therapy suppressing acid production, including the use of proton pump inhibitors, provides little, if any, relief for rumination syndrome - mainstay of treatment for rumination syndrome is behavioral modification consisting of habit reversal - diaphragmatic or abdominal breathing techniques may be effective treatment because they compete with the urge to regurgitate

Most common cause of oligohydramnios and must be ruled out if oligohydramnios is suspected, especially if a normal-sized bladder is seen on fetal ultrasound evaluation ...

rupture of membranes - Oligohydramnios causes fetal compression abnormalities such as fetal distress, clubfoot, spadelike hands, and a flattened nasal bridge. - The most serious complication of chronic oligohydramnios is pulmonary hypoplasia.

An 8-year-old girl is brought to the office to discuss recent behavioral changes. Her mother reports that her daughter has been having frequent dreams about getting lost in a forest and wakes up crying. Every morning before school, she will persistently ask if she can go to work with her mother instead of going to school. During the day, the school nurse has called to report that the girl refuses to do her assigned work and instead will suddenly cry for no apparent reason. At home, she behaves well with no emotional outbursts, enjoys outings with her mother, and completes her homework without problems. Her mother reports that the behavioral problems started after her husband was deported. Of the following, the MOST likely diagnosis for this girl is

separation anxiety disorder - The child in this vignette most likely has separation anxiety disorder given that she fulfills 3 of 8 diagnostic criteria: her symptoms worsen when separated from her mother and abate when she is physically near her, she is reluctant to go to school because she would be separated from her mother, and she has repeated dreams about separation.

Initial work-up for a patient with possible juvenile dermatomyositis should include:

serum CK level Diagnosis of dermatomyositis requires the presence of characteristic rash as well as at least three signs of muscle inflammation and weakness. - Elevated serum levels of muscle-derived enzymes (creatine kinase [CK], aldolase, aspartate aminotransferase, alanine aminotransferase, and lactate dehydrogenase) reflect muscle inflammation. - Not all enzyme levels rise with inflammation in a specific individual; alanine aminotransferase is most commonly elevated on initial presentation, whereas the CK level may be normal.

Healthy lifestyle changes that promote weight loss are the primary management for obesity and metabolic syndrome. Motivational interviewing can be used to help set SMART (specific, measurable, attainable, relevant, timely) goals. The 9-5-2-1-0 principle provides a framework for counseling and goal setting:

9 hours of sleep per night 5 servings of fruits and vegetables per day Less than 2 hours of screen time per day (television, computer, electronic devices) 1 hour of physical activity per day 0 sugary drinks (soda, juice)

Recommended hours of sleep for: 6-12 years

9-12 hours

Normal anion gap in infants

<15 mEq/L Increased anion gap - >15 in LBW infants (<2500 g) - >18 in ELBW infants (<1000 g) newborn infants normally have base deficit of 1-3 mEq/L

Normal anion gap in infants

<15 mEq/L Increased anion gap: - >15 mEq/L in LBW infants (<2,500 g) - >18 mEq/L in ELBW infants (<1,000 g)

VLBW infants:

<1500g

Who gets retinopathy of prematurity eye screen...

<30 wk or <1500 g - GA + weight

Suggested frequency of measurements of electrolytes, including calcium for infants receiving only IV fluids:

<750 g - q8-12 h x 3-4d, then daily 750-1,500 g - q12 h x 3-4 days, then daily >1,500 g - daily

high voltage =

>1000 V - power lines = >14,000 V

Caffeine Citrate dose (used for apnea of prematurity and CLD prevention):

20mg/kg loading dose x1, then 10mg/kg/dose q day (or divided bid)

Age? - 25-50 words - can point to 5 body parts

21-month-olds

Target heart rate is calculated as ...

220 - age in years

When are there sufficient air sacs and pulmonary vasculature development so that the fetus is able to survive?

26-28 weeks gestation

When does stranger anxiety emerge?

6-12 mos

In newborn, saturation goal at 3 minutes...

70-75%

Mainstays of treatment for amphetamine intoxication.

Benzodiazepines and supportive care

What is oxygen delivery

CO x O2 Content - HR x SV = CO - preload, afterload, and cardiac contractility ~ SV - afterload = wall tension heart pumps against -oxygen content = (hgb x 1.34 x SaO2) + (0.0031 x PaO2)

At what lung volume is pulmonary vascular resistance at its lowest?

FRC - At FRC, the pulmonary vascular resistance is at its lowest. - With increasing lung volumes, septal vessels get compressed leading to increased PVR. At lower lung volumes, corner vessels get compressed leading to increased PVR. Hence, the aggregate resistances of septal and corner vessels is the lowest at FRC (after a normal tidal breath exhalation).

mean arterial pressure

MAP = diastolic pressure + (pulse pressure/3) = 1/3 systolic pressure + 2/3 diastolic pressure Preterm infants and newborns: Normal MAP = gestational age in weeks + 5

Nebulized albuterol higher doses than MDI. Doses for each in the ED in peds?...

Nebulized albuterol: 0.15 mg/kg per dose max 5mg Must use higher doses of MDI - 2-4 puffs young children - 4-6 puffs older children - 4-8 puffs adolescents

An 18 month-old child has 1 week history of cough and runny nose. The child has diffuse cyanosis and responsive only to painful stimulation with slow respirations and rapid central pulses. The child's respiratory has decreased from 65/min to 10/min, severe inspiratory intercoastal retractions are present, HR is 160/min, SPO2 is 65% on RA, and capillary refill is less than2 seconds. Which are the most appropriate immediate interventions for this toddler?

Open the airway and provide positive-pressure ventilation using 100% O2 and a bag-mask device.

Tidal volume is generated by the difference between

PIP and PEEP

Age: works for toy out of reach

6 mos

quick way for systolic BP in kid

70 + (age x 2)

Daily iodine requirement for children

90-120 mcg - infants = 50-60 mcg - pregnant = 200 mcg - adults = 150 mcg (=1/4 tsp salt) *about 125 mcg stored in thyroid gland

Calculating a meal or snack dose becomes simple when you know your I:C ratio. If each unit covers 10g and you have a modest 20g meal, you will need ...

Simply divide your carbs by your ratio --> 2 units of insulin (20 ÷ 10 = 2) - If you devour 120g, you will need 12 units (120 ÷ 10 = 12).

SMART therapy for asthma

Single Maintenance and Reliever Therapy - The use of ONE inhaler (ICS+LABA) for both control and rescue - LABA must be formoterol, not salmeterol - Off-label use - Logistical issues -> hesitancy and inconsistent adoption Global Initiative for Asthma (GINA) guidelines: https://ginasthma.org/gina-reports/ • National Heart, Lung, and Blood Institute (NHLBI) guidelines: https://www.nhlbi.nih.gov/health-topics/asthma-management-guidelines-2020-updates

How long should you place a 5-year-old in time-out?

Six minutes, the number of the child's age plus 1.

3 routine vaccines for adolescents ages 11-12

Tdap MCV4 HPV

Autosomal recessive inherited disorder characterised by brittle hair and intellectual impairment

Trichothiodystrophy - Tests show that the hair is lacking sulfur, an element that normally gives hair its strength - Mild cases may involve only the hair. More severe cases also cause delayed development, significant intellectual disability, and recurrent infections; severely affected individuals may survive only into infancy or early childhood

Nasolacrimal duct obstruction present in up to 5% of infants. The symptom of eye drainage without inflammation or pain occurs because of incomplete opening of the valve of Hasner, the valve between the nasolacrimal duct and inferior nasal meatus. Providers often encourage applying gentle pressure to the lacrimal sac (at the inner corner of the eye) to increase the pressure through the nasolacrimal duct and overcome the obstruction. Gentle pressure is thought to be sufficient, without downward massage. Using a clean washcloth to avoid the introduction of infection is important. When should an ophthalmologist be consulted?

When the obstruction does not resolve by 12 months of age --> ophthalmologist may probe the duct to open the obstruction

When baby is rearfacing, should shoulder straps be above or below shoulders...

a little below the shoulder - for >3, should be above the shoulder

For pediatric asthma, after inpt discharge, send pts home on what albuterol management ...

albuterol 2-4 puffs q4hrs with a plan for spacing albuterol treatments at home

Clinical presentation in neonate suggests meningitis, which is supported by the presence of gram-positive rods in her cerebrospinal fluid. Listeria monocytogenes is the most likely pathogen given this laboratory finding. The treatment of choice for Listeria monocytogenes infections is

ampicillin

Typically, RSV causes a cold, which may be followed by bronchiolitis or pneumonia. Symptoms generally last how long?

an average of 5-7 days

Blood type O (OO) makes...

anti-A and anti-B Ab

The primary treatment of IgA nephropathy is

appropriate blood pressure control - Fish oil, which contains anti-inflammatory omega-3 polyunsaturated fatty acids, decreases the rate of disease progression in adults - Immunosuppressive therapy with corticosteroids or more intensive multidrug regimens may be beneficial in some patients - Angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonists are effective in reducing proteinuria and retarding the rate of disease progression when used as single agents or in combination

Size of VSD is defined as relative to ...

are of the aortic valve - small = less than 1/3 the area - moderate = 1/3 - 2/3 of the area - large = more than 2/3 the area

tracheostomy placement

between tracheal rings 2 to 4

Acute respiratory distress syndrome (ARDS) is a common clinical diagnosis in pts with noncardiogenic pulmonary edema and has been defined as...

bilateral pulmonary infiltrates and severe hypoxemia in absence of evidence for cardiogenic pulmonary edema

full sepsis workup for baby

blood culture CBC UA routine CSF cx CMP - serum HSV - coag - PCR

Rabies postexposure prophylaxis consists of

both rabies immunoglobulin and rabies vaccine on day 0 in addition to doses of rabies vaccine on days 3, 7, and 14

Calcium is only in infants in the TPN if the line is ...

central - if only a peripheral line is available, add P to TPN and give Ca gluconate separately - max P = total cation x 0.6

Medication for anxiety / behaviors, like repetitive/stereotypical

citaloprim and other SSRIs

Gonadoblastoma is associated with what testicular abnormality?

cryptorchidism

What is an important secondary prevention in a child with a submersion injury?

early initiation of CPR

Children with sickle cell disease are predisposed to invasive bacterial infections from

encapsulated organisms such as S pneumoniae, Haemophilus influenzae type b (Hib), and nontyphoidal Salmonella due to functional asplenia - results in diminished antibody-mediated phagocytosis - of these organisms, infection with S pneumoniae occurs most frequently - prophylactic antibiotics and immunization are critical aspects of preventive care in patients with sickle cell disease

What gender has a higher incidence of choanal atresia

female

QT --> distance between Q & T more than half of R & R -->

flag! --> look at closer and hand calculate

When does arterial duct close?

functionally within 24 hours anatomically within 3-4 wks in most pts - may remain patent longer in some pts, especially infants born prematurely

In the NICU, mL/kg/day of IL =

g/kg/day * 5

What disorder of carbohydrate metabolism causes an increased risk of Escherichia coli sepsis?

galactosemia

Glutaric aciduria type II is treatd with...

high carb, low protein, low fat diet - eat often to avoid low blood sugar - dietary supplementation with riboflavin, carnitine, other supplements

Used to medically close PDAs in premature infants

ibuprofen and indomethacin - acetaminophen = studied as alternative

A boy with 4 hours of classic symptoms of exam findings of testicular torsion should undergo...

immediate surgery without delay for diagnostic imaging

Most common cause of failure to catheterize and umbilical artery is....

inadequate dilatation of artery

A 12 yr old boy sustains a nail puncture of the right foot through an old sneaker. Two days later, he limps and complains of pain and swelling in that area. The most likely diagnosis is:

osteomyelitis

Absolute contraindications to MMR vaccine

pregnancy immunocompromised status HIV+ pts - only if CD4+ count <15% and <200/mm^3

Formula for preterm infants....

premature enfamily or similac special care, 260 mOsm/L

premie bili calculator... term bili calculator...

premie = stanford bili calc term = NCN bili calc

Which of the following probably best predicts fluid responsiveness?

pulse pressure variability - Pulse pressure variability (with a rise in pulse pressure with delivery of a mechanical PPV breath) of >15% has been shown to be associated with fluid responsiveness, at least in adult patients.

In what seasons are enteroviral infections most common?

summer and fall

The umbilical vein of a newborn can be accessed how long?

up to about 2 weeks of life

Which inheritance pattern is seen in androgen receptor defects?

x-linked recessive

Juvenile polyp(s) should be suspected in

young child (typically ages 1-7 years) with intermittent and painless rectal bleeding

Due to the significant comorbidities associated with T2D in youth, these tests are recommended at diagnosis:

› Blood pressure measurement › fasting lipid panel › Albumin excretion assessment › Dilated eye examination

Amount of potassium to add to maintenance fluids if needed:

● Add if patient is urinating ● Patient < 10kg = 10mEq/L KCl ● Patient >/=10kg = 20mEq/L KCl

Most common cause of thymic enlargement in infants

Benign thymic hyperplasia

What tool is used to determine an infant's risk of developing clinically significant hyperbilirubinemia?

Bhutani nomogram.

Which of the following is the earliest and most consistent sign of juvenile myasthenia gravis?

Ptosis and extraocular muscle weakness - In the juvenile form, ptosis and some degree of extraocular muscle weakness are the earliest and most constant signs. - Older children might complain of diplopia, and young children might hold open their eyes with their fingers or thumbs if the ptosis is severe enough to obstruct vision. - The pupillary responses to light are preserved.

Recommended schedule for varivax (varicella vaccine)

Pts < 13 yo - first dose = 12-15 mos - second dose = at least 3 mos later (recommended combines MMRV at 4-6 yo) Pts 13+ yo - 2 doses (0.5 ml each) of single-antigen varicella vaccine subQ 4-8 wks apart - cannot use MMRV

Murmur - systolic - LUSB - 8-14 yo - grade 1-2/6 - soft, blowing

Pulmonary flow murmur

Recognize prolonged qt

- The QT interval—the start of the QRS complex to the end of the T wave—must be corrected to the heart rate by dividing by the square root of the preceding R-R interval. - Those with acquired or inherited prolonged QT syndrome are at risk of experiencing palpitations, syncope, seizures, or sudden death.

stimulants used for constipation

- senna - bisacodyl oral and suppositories

Recognize ecthyma gangrenosum, which is caused by pseudomonas aer.

- usually affecting immunocompromised hosts, including patients with severe PEM - in addition to malnutrition, risk factors for EG include severe burns, uncontrolled diabetes, and a compromised immune system (eg, HIV/AIDS, malignancies, immunosuppressive therapy). however, EG can also occur in children with a normal immune system; infants are at highest risk.

Main reason for pRBC transfusion in first week of life in low birth weight infants is...

anemia from phlebotomies for lab studies - preterm infants recover from anemia at 34-36 wks of gestation - endogenous erythropoietin is released when hct decreases to low 20 s --> reticulocytes increase 1 wk later

First-line medication therapy for hypertension includes

angiotensin-converting enzyme (ACE) inhibitors angiotensin II receptor blockers (ARBs) long-acting calcium channel blockers thiazide diuretics

RV and LV pressures in setting of massive VSD

equal

Inspiratory stridor suggests...

extrathoracic obstruction - crouple - laryngomalacia

Infants with documented cow's milk protein-induced enteropathy or enterocolitis often are also sensitive to soy protein and should not be given isolated soy protein-based formula. They should be provided ...

formula derived from extensively hydrolyzed protein or synthetic amino acids - soy formulas can contain phytoestrogens, which have increased caution for their use in infants

Good source of iron includes

fortified cereals legumes leafy greens meat

At what time post acute acetaminophen ingestion can you begin to use the Rumack-Matthew nomogram?

four hours post ingestion

Polycystic ovary syndrome is the most common cause of chronic anovulation and infertility in young women. The diagnosis of polycystic ovary syndrome in adolescent girls is often based on evidence of hyperandrogenemia and ovulatory dysfunction. The most sensitive test for diagnosis in women is

free and total testosterone level

All do bolus feeds if tip is placed...

gastrically

The clinical manifestations of the infantile form of GM1 gangliosidosis may be evident in the newborn infant as ...

hepatosplenomegaly, edema, and skin eruptions (angiokeratomas) - most frequently presents in the first 6 mo of life with developmental delay followed by progressive psychomotor retardation and the onset of tonic-clonic seizures - typical facies is characterized by low-set ears, frontal bossing, a depressed nasal bridge, and an abnormally long philtrum. - up to 50% of patients have a macular cherry-red spot. - hepatosplenomegaly and skeletal abnormalities similar to those of the mucopolysaccharidoses, including anterior beaking of the vertebrae, enlargement of the sella turcica, and thickening of the calvarium, are present

A full-term infant is born via spontaneous vaginal delivery and develops respiratory distress immediately after birth. On physical examination, it is noted that the lower jaw is small and set back in relation to the upper jaw. The baby's tongue appears large and is positioned further back in the pharynx than normal, causing airway obstruction. No cleft palate is noted. For initial management of this patient's airway obstruction, in what position should the infant be placed?

*prone* Baby with Robin sequence + labored breathing --> first-line = prone positioning

Cardiogenic shock may be distinguished from other causes of shock by the presence of ...

*rales, hepatomegaly, and jugular venous distention* - Respiratory distress and wheezing are common signs of cardiac disease in children. - Cardiogenic shock can be worsened by aggressive fluid resuscitation.

Prefixes are used to describe therelative direction of the misaligned eye's deviation,such as "exo" for

abducting (outward) tendency

Seizures that can be triggered by hyperventilation

absence seizures

Pediatric dyslipidemia screening

age 9-11 and 17-21 - non-fasting lipid panel - concurrent screening for NAFLD (AST/ALT), diabetes risk (A1c), vitamin D deficiency

What is a marker of impaired bone mineralization?

alkaline phosphatase

Medication for hyperactive/impulsivity associated with autism

alpha agonist: guanfacine

Blood type A (AA, AO)- makes...

anti-B Ab

Most common complication of PGE1

apnea - due to direct effect of PGE1 on CNS

On esophageal biopsy, how many eosinophils per high power field are required for diagnosis of eosinophilic esophagitis?

at least 15

First-line therapy for functional constipation is daily polyethylene glycol; this should continue for how long?

at least 2 months - should not stop unless symptoms have resolved for at least 1 month

Preferred location of UVC tip is ...

beyond the ductus venosus in IVC or low right atrium - must be localized using pressure measurements, measurements of PO2, and by radiography - advance UVC while continuously measuring pressure --> as tip passes through ductus venosus, pressure will decrease and wave form will resemble atrial pressure tracing

Labs to get during ingestions of toxins

blood gas glucose BMP utox salicylate tylenol level

The classic triad of botulinum toxin includes

bulbar palsy descending (not ascending) paralysis clear sensorium

The most serious manifestation of lead intoxication is:

cerebral edema - Headaches, change in mentation, lethargy, papilledema, seizures, and coma leading to death are rarely seen at levels lower than 100 μg/dL but have been reported in children with a BLL as low as 70 μg/dL. - The last-reported death directly attributable to lead toxicity in the USA was in 2006 in a child with a BLL of 180 μg/dL

Type 1 fractures are most common in what age group?

children less than 5 yo - shearing or avulsion stress = fracture through physis with separation of epiphysis from metaphysis

A 2 mo old infant presents with conjunctivitis, tachypnea, and a mild cough. There is no fever. Physical examination reveals the presence of crackles bilaterally. The most likely etiologic agent is:

chlamydia trachomatis pneumonia - onset usually occurs between 1 and 3 mo of age and is often insidious, with persistent cough, tachypnea, and absence of fever - auscultation reveals rales; wheezing is uncommon - absence of fever and wheezing helps to distinguish C. trachomatis pneumonia from respiratory syncytial virus pneumonia - most consistent finding on chest radiograph is hyperinflation accompanied by minimal interstitial or alveolar infiltrates

choice for paralysis in children with renal failure

cisatracurium - eliminated by Hofmann elimination, which is spontaneous degradation of the medication at physiologic pH and temperature

Natal teeth are associated with

cleft palate Pierre Robin syndrome Ellis-van Creveld syndrome Hallermann-Streiff syndrome pachyonychia congenita other anomalies - family history of natal teeth or premature eruption is present in 15-20% of affected children

Which of the following symptoms most often persist after prolonged opioid use?

constipation

High urine chloride levels and metabolic alkalosis are seen in

diuretic use Bartter syndrome Gitelman syndrome

Routine schedule of MMR of 2-dose series...

dose 1 at 12-15 mos dose 2 at 4-6 years Special circumstances - catch up: 2 doses, 4 wks apart

Most common symptomatic vascular ring

double aortic arch

Main disadvantage of CT is

effect of ionizing radiation

Most common complication of SLE

glomerulonephritis

Children with cystic fibrosis are at risk of experiencing what with the following electrolytes: - Cl - Na - K - Bicarb

hypochloremia, hyponatremic dehydration, hypokalemia, and metabolic alkalosis - loss of electrolytes with sweating - sodium chloride supplementation prevents the risk of electrolyte disturbances in the setting of fever, vomiting, diarrhea, strenuous activity, or exposure to heat

Athletes with what corrected vision need additional visual evaluation before clearance for contact/collision sports?

worse than 20/40

Severe RSV disease occurs primarily in

infants younger than 6 months during their first fall and winter season of life

Treatment for congenital hypothyroidism...

levothyroxine - should be initiated by 2 weeks of age - thyroid dysgenesis is the most common cause of congenital hypothyroidism in the United States and most often occurs sporadically

Severe persistent asthma in children 12 years of age or older requires the initiation of step 4 or step 5 therapy. The preferred treatment includes a

medium- to high-dose inhaled corticosteroid plus a long-acting beta agonist (LABA)

Site of greatest resistance in the upper airway​

nose - pharynx = site of the greatest increase in airway resistance during sleep

Soy milk-based formula is nutritionally inappropriate for preterm infants because of the risk of

osteopenia aluminum toxicity inadequate absorption of zinc, phosphorus, and iron

What is the most common physeal fracture?

salter harris II - fracture above physis

Normal course of UVC is through...

single umbilical vein to liver --> through ductus venosus --> into IVC --> tip placed in the IVC-RA junction

Premature infants, especially the very premature, are prone to wasting electrolytes and bicarbonate through their kidneys. We affectionately call this the "dumb preemie kidney" (DPK) effect. Especially in the first few weeks, you are constantly fighting against this effect. That's why their KVOs are run with

sodium acetate - instead of sodium chloride - because they need the additional buffer

Tx for opthalmia neonatorum via neisseria/gonococcal

- ceftriaxone 25-50 mg/kg/day IV - topical irrigation

Umbilical venous catheter on imaging

- right at diaphragm - straight - right side of body

An 11 mo old boy is treated for acute Kawasaki disease with 2 g/kg of intravenous immune globulin. No cases of measles have reported in his hometown in the last 8 yr. The 1st measles-mumps-rubella vaccine should be administered to this child at the age of:

22 months - Because IVIG may interfere with the immune response to live virus vaccines because of specific antiviral antibody, the measles-mumps-rubella and varicella vaccinations should generally be deferred until 11 months after IVIG administration. Other vaccinations do not need to be delayed.

A major milestone of object permanence (constancy), the understanding that objects continue to exist even when not seen, is achieved by...

9 mos

A specific chromosomal translocation, or a variant thereof, is found in most of the Ewing sarcoma family of tumors. What is it?

t(11;22)

For total fluids in the NICU, feeds are calculated by

taking the volume --> multiplying by 8 (if they are being fed every 3 hours) --> dividing by their weight

Your baby is ready for solids when

they have outgrown the tongue-thrust reflex they can sit up mostly on their own

Anterior mediastinum contains...

thymus vessels (including internal mammary) - 85% of anterior mediastinal masses are due to lymphoma, teratoma, and benign thymic enlargement

Most common congenital anomaly of kidney or urinary tract to cause prenatal hydronephrosis?

ureteropelvic junction obstruction

Adolescents: Substance use screening: Any alcohol, marijuana, other drugs in the past 12 mos? If yes, administer....

CRAFFT questionnaire C - Have you ridden in a CAR drive ben someone (of yourself) who was "high" or had been using alcohol or drugs? R - Do you ever use alcohol or drugs to RELAX, feel better about yourself, or fit in? A - Do you ever use alcohol/drugs while you are ALONE? F = Do your family or FRIENDS ever tell you that you should cut down on your drinking or drug use? T = Have you gotten into TROUBLE while you were using alcohol or drugs? Answering yes to two or more questions = positive screen

Ossification centers of the elbow

CRITOE - capitellum = 1 yr - radial head = 3 yr - internal epicondyle = 5 yr - trochlea = 7 yo - olecranon = 9 yr - external epicondyle = 11 yr *when they appear

Characteristically presents with recurrent episodes of gross hematuria concomitant with an upper respiratory illness. The serum complement levels (C3 and C4) are normal and helpful in differentiating it from other causes.

Immunoglobulin A nephritis

Most common endocrinopathy in women and most common cause of anovulation...

PCOS - impacts 5-15% of the female population

What method is considered safest and most effective for reduction of intussusception?

*Air enema with fluoroscopy*

Imaging for kid with.... visualized structural anomalies (chest wall, vascular, tracheobronchial tree, lung parenchyma)

multidetector CT scan

diaphragmatic hernia treatment if diagnosed prenatally..

plan delivery at place with ECMO --> let lungs mature 3-4 days then do surgery

1 week old baby becomes cyanotic when feeding but pinks up when crying.

*Choanal atresia* Also look for .... CHARGE associated anomalies... - coloboma - heart defects - retarded growth - GU anomalies - ear anomalies & deafness

INITIAL FLUID ADMINISTRATION: Guidelines for the first few days of life are:

- For larger infants (i.e., >1,250 g) start at 60 mL/kg/d of D10W. - If estimated ILW is high (e.g., extreme prematurity, abdominal wall defect), start fluids at higher rate, 80- 100 mL/kg/d. - Fluid requirements gradually decrease by day 5-6 as skin permeability decreases.

Treatment for Very-long chain acyl-CoA dehydrogenase deficiency (VLDCASD)...

- frequent feeding - glucose infusion - low fat formulas and increased medium-chain triglycerides

Goal cyclosporin trough if... - fresh transplant - patient is far out and stable numbers

- fresh transplant = 200-400 - stable = <100

What is a dacryocystocele?

- results from obstruction of both nasolacrimal duct and its canaliculi - produces bluish, firm mass at nasolacrimal sac

Recommended hours of sleep for: 3-5 years

10-13 years (including naps)

Recommended hours of sleep for: 1-2 years

11-14 hours (including naps)

When can MMRV be given

12 mos through 12 years

Recommended hours of sleep for: 4-12 months

12-16 hours (including naps)

Begin routine yearly screening with opt-out consent starting at age

13

The recommended intake goal for infants with congestive heart failure is

140 kcal/kg/day

2 rescuer compression to breath ratio?

15:2

Any intermittent strabismus after what age is considered abnormal?

4 mos - warrants referral

Suture choice for face

5-0, 6-0 - remove after 3-5 days

When to start talking to parents about kindergarten application process

4 year visit - application process should be started at least 10 months prior to entry year

Kocher criteria

1. non-weight bearing 2. fever >101.3 F 3. ESR >40 or CRP >2 4. leukocytosis *pediatric pts with >/= 3 criteria have high likelihood of septic arthritis* --> immediate joint aspiration performed for dx and decompression of joint

Currently accepted level for threshold of concern in blood lead level is greater than

*9 mcg/dl* - GI symptoms occur at appx 50 mcg/dl

Age: removes garment

2 years

Otitis media with effusion is typically self-limiting and usually resolves within

3 months

In resuscitation of newborn infants, set blender to what in most cases?

40% O2

Suture choice for hands or feet

5-0 - remove after 10-14 days

What is the typical inheritance pattern of urea cycle defects?

Almost all urea cycle defects are autosomal recessive, but ornithine transcarbamylase deficiency is X-linked.

What are the diagnostic criteria for ADHD?

At least six of the inattention and/or hyperactivity-impulsivity symptoms described in the DSM-5, lasting for six months or more and present in two or more settings.

What type of formula is contraindicated for use in administering levothyroxine?

Soy-based formulas, as these inhibit absorption of levothyroxine.

Form of cerebral palsy often secondary to structural abnormalities, such as brain malformations, stroke, or vascular malformations. Patients often achieve the ability to ambulate independently and have normal intelligence with increased risk for seizures.

Spastic hemiplegia

Hormone-free IUD. May be used up to 10 years.

Copper

Bag mask ventilation gas flow rate for infant should be....

~5-6 L/min - don't let mask to slide down from bridge of nose and to occlude nares

Survival of VLBW babies is directly related to

birth wt

What fluids are recommended for pediatric patients not at risk of ADH secretion?

D5 1/2NS and D5 NS are recommended

How to dose glucose in kids

D50 x 1 kk/kg D25 x 2 cc/kg D10 x 5 cc/kg

Which virus is most commonly associated with post-transplant lymphoproliferative disease?

EBV

In the NICU, intralipids have a fun shortcut -

Each g/kg/day of IL equals 5 ml/kg/day of IL

Indication of small-airway function. First affected in obstructive lung diseases such as asthma and CF.

FEF25-75 - flows in midportion of forced-exhalation maneuver - forced expiratory flows between 25% and 75%

The etiology of acute liver failure in children varies by age. It is idiopathic in greater than 50% of children across all age groups. Infectious causes are also common. What are the most common infectious agents leading to ALF across all age groups?

Herpes simplex virus Epstein-Barr virus adenovirus parvovirus

A 2 yr old presents to your office with black spots and missing enamel on the maxillary anterior teeth. The most likely etiology of this condition is:

High sucrose consumption and Streptococcus mutans infection

Immunizations for 18 yo

Meningitis ACWY (Menveo) Meningitis B (Bexsero) Influenza

Formula for term infants

Neo 19

Murmur - systolic - LUSB, back/axillae - newborns - grade 1-2/6 - low-medium pitch

Peripheral PA stenosis

Cellcept dosing pre- and post-transplant

Pre-transplant: = Cellcept 1200 mg/m2/day IV divided Q12hrs Post-transplant: = Cellcept 1200 mg/m2/day IV/PO divided Q12hrs for first 48 hrs post-transplant, then 900 mg/m2/day PO divided Q12hrs until week 2, then 600 mg/m2/day PO divided Q12hrs thereafter. We do not routinely check mycophenolic acid (cellcept) levels.

Patients who develop late, late-onset infection ( > 90 days of age) with group B Streptococcus often have which underlying medical conditions?

Prematurity ( < 28 weeks) or immunodeficiency

The diagnosis of brain death cannot be made in what age children?

Preterm infants less than 37 weeks gestational age

Most allergens are what type of molecule?

Proteins of molecular weight 10-70 kd - molecules < 10 kd do not bridge adjacent IgE antibody molecules on the surfaces of mast cells or basophils - most molecules > 70 kd do not pass through mucosal surfaces, a feature needed to reach antigen-presenting cells (APCs) for stimulation of the immune system - allergens frequently contain proteases, which promote barrier dysfunction and increase allergen penetration into host tissues

Defined as starting a method of contraception on the day of the visit (not waiting until a new menstrual cycle begins).

Quick start

What is the function of the Wiskott-Aldrich syndrome protein?

Reorganization of the actin cytoskeleton of hematopoietic cells.

Most common primary malignant germ cell tumor in anterior mediastinum

Seminomas

Reducing burdens of existing dz

Tertiary prevention

Can children who have the classic rash of fifth disease return to daycare?

Yes, once rash or arthralgias develop, viral shedding is typically complete and the patient is no longer infectious.

What translocation is associated with Burkitt lymphoma?

Translocation (8;14)

Causes of primary amenorrhea with delayed puberty distinguished by an abnormally high FSH ...

Turner syndrome androgen insensitivity ovarian failure - combination of vaginal examination findings and lack of withdrawal bleeding to progesterone challenge indicate a lack of estrogen

In a patient who is recovering from typical or atypical pneumonia, how long can it take for the cough to resolve?

Up to four months.

Where is the lens displaced in patients with Marfan syndrome?

Upwards and temporally.

VACTERL syndrome

Vertebral abnormalities Anal atresia Cardiac defects TE fistula Renal disease Limb abnormalities

Malabsorption of which micronutrients is most common in patients with short bowel syndrome?

Vitamins A, D, E, and K, and calcium, iron, and vitamin B12.

Goal feeds of infants based on age...

age in mos + 2 oz = feed amount - newborn ~ 2-3 oz q3-4 hours - 1 mo ~ 3-4 oz q4h - 2 mo ~ 4 oz q 6-7 hours/24 h - 4 mo ~ 4-6 oz = 5 feedings/24h

Most common reason for laparotomy in pediatric population

acute appendicitis

Gut bacteria synthesize vitamin K2, an important isoform of vitamin K. Disruption of the gut microbiome (eg, by broad-spectrum antibiotics) can result in vitamin K deficiency. For children on long-term antibiotic therapy or prophylaxis, vitamin K deficiency can be prevented through

administration of vitamin K orally once weekly

Most common site of origin of mediastinal masses in children

anterior and posterior mediastinum - 20% of all mediastinal masses originate in middle

Blood type B (BB, BO)- makes...

anti-A Ab

Most common cause of mortality due to congenital malformations

congenital heart defects

Features that often occur in people with chromosome 15q deletion include

developmental delay, intellectual disability, behavioral problems, and distinctive facial features

First line medication for hypotension in an infant is

dopamine gtt - if infant is requiring a dopamine gtt, BP must be followed more closely with arterial access (UAC or PAL)

Burp infant how often?

every 1-2 ounces of use "paced bottle feeding"

What is the male equivalent of the round ligament?

gubernaculum testis

Seizures arising from which lobe are most likely to be mistaken for psychogenic nonepileptic seizure?

frontal

Central access (TPN) line terminates where?

in SVC

myopia

nearsightnedness

A 1-unit per-10-grams-of-carb (1:10) ratio means that

one unit of insulin covers 10 grams of carbohydrate

Oxygen saturations of less than 93% predict a partial pressure of ...

oxygen of less than 70 mmHg

Pathologic apnea in infant is ...

prolonged respiratory pause of >/= 20 sec or one associated with bradycardia or color change - central = no respiratory effort - obstructive = via upper airway obstruction - mixed

Medication for hypersensitivity-anxiety from autism

propranolol

The most effective treatment for port-wine stains is with the

pulsed dye laser (PDL)

What happens when, in CHD lesions dependent on PDA, the PDA begins to close...

shock or cyanosis

Physical illness associated with reduction or cessation of opioid ingestion is termed:

spontaneous withdrawal

In NICU, feed volume in ml/kg/day =

{(Volume per feed) x 8} / patient weight

Most full-term infants establish normal ocular alignment within the

first 8 weeks of life - by 3 months, SHOULD be completely resovled

A 4-year-old boy presents to clinic with left knee swelling. His parents report history of a bullseye rash several weeks ago. Aspiration of synovial fluid reveals white blood cells. Which of the following is the most appropriate therapy for this boy?

oral amoxicillin Lyme arthritis, a late finding of Lyme disease. - White blood cells are seen on synovial fluid aspiration. - The treatment of choice for Lyme arthritis in children under 8 years of age is oral amoxicillin.

Button batteries in esophagus contain...

- toxic heavy metals: NaOH, KOH --> can cause esophageal burns within 6 hours - less dangerous in stomach --> usually pass within 2 days BUT is symptomatic or battery still before pylorus in 48h --> repeat XR + remove it

General recommendations for weight training are

2 or 3 sets of 8 to 15 repetitions per exercise up to 3 sessions per week - Adult guidance to assure correct form and safety should be encouraged.

Laboratory data for neonate shows hyperammonemia with anion gap acidosis; in the presence of urine ketones, what should be suspected?

organic acid disorder - caused by disordered catabolism of certain amino acids, which leads to accumulation of organic acids - decreased feeding and somnolence is based on clues seen in laboratory data

Total body deficit of Na+ can be calculated by: Na+ deficit (mEq) =

(desired [Na+ ] - current [Na+ ]) x 0.8 x body weight (kg) (0.8 x body weight is the volume of distribution for Na+ ) Symptomatic hyponatremia: (e.g., seizures or [Na+ ] <120 mEq/L). - Calculate Na+ deficit to raise [Na+ ] to 125 mEq/L and give as 3% NaCl (0.5 mEq/mL) over 3-6h. Correct remaining deficit over next 24h. Asymptomatic hyponatremia: - Calculate total deficit of Na+ and give ½ over 6- 8h and the rest over the next 24h, as Na+ added to IV fluids.

3 yo girl. Height 85 cm. Father height 165 cm. Mother height 155 cm. Mid-Parental height?

*153.5 cm* MPH = [(parental height - 13) + maternal height]/2 = [(165 - 13 cm) + 155]/2 = 153.5 cm

Age? - No head lag when pulled to sit - Rolls front to back - Plays with rattle - Mouths objects - Laughs out loud

*4 mos* Also... - sits with trunk support - props on wrists - hands held predominately open - clutches at clothes - reaches persistently - briefly holds onto breast or bottle - reaches for ring/rattle - smiles spontaneously at pleasurable site - to and fro alternating vocalizations - orients head in direction of voice - stops crying to soothing voice - vocalizes when alone

Initial management of undifferentiated hyperammnoemia

If NH3 is elevated, re-check given high rate of false positives due to testing errors. If >100 and patient is symptomatic, try to stop catabolism and nitrogen breakdown (make NPO, start D10 containing fluids) and recheck ammonia q4H. - Complete workup for acute liver failure and inborn errors of metabolism (coags, serum and urine amino acids, carnitine profiles etc). - Other adjuncts may be used based on etiology to enhance NH3 metabolism (discuss with consultants). - Consider hemodialysis if >250 umol/L and not improving after 4 hours or for levels >500 umol/L.

5 stages of lung growth

*E*very *P*art *C*ome*S* Through *A*ge Embryonoic Pseudoglandular Canalicular Saccular Alveolar

Regional anesthetic technique used as an alternative or adjunct to general anesthesia for foot and ankle surgery

*sural nerve block*

Visual screening for 6-12 mos

- ocular history - external inspection of eyes/lids - red reflex testing - pupil exam - ocular motility assessment - instrument-based screening when available - visual acuity: fixate and follow response

acute flaccid myelitis

- rare neuro disorder - acute paralysis of 1 or more limbs - preserved sensation - normal mental status - typically within week of onset of viral symptoms - MRI = extensive longitudinal centrally located inflammation affecting gray matter of cord - CSF = lymphocytic pleocytosis

You need to provide rescue breaths to a child victim with a pulse. What is the appropriate rate for delivering breaths?

1 breath every 3 to 5 seconds

Normal plasma Na level

135-145 mEq/L

Viral upper respiratory tract infections typically improve within

14 days

Age: crawls up stairs

15 mos

Age: puts block in cup

15 mos

Referral age for intermittent strabsimus

6 mos

Abnormal sweat test in infant...

>60 mmol/L

•Dose of alkali for treatment of metabolic acidosis can be calculated by:

Dose of alkali (mEq) = base deficit x 0.3 x body weight (kg) - Administer alkali IV at a rate not exceeding 1 mEq/kg/min. - The usual alkali used in newborns is NaHCO3 and the concentration is 0.5 mEq/mL, so it is hyperosmolar (900 mOsm/L)

To which antibody class does the antibody in neonatal alloimmune thrombocytopenia belong?

Immunoglobulin G, which crosses the placenta to affect the fetus.

Dental caries (cavities) in children are due to:

Metabolism of Streptococcus mutans - bacteria have the ability to adhere to enamel, produce abundant acid, and survive at low pH - once the enamel surface cavitates, other oral bacteria (lactobacilli) can colonize the tooth, produce acid, and foster further tooth demineralization - demineralization from bacterial acid production is determined by the frequency of carbohydrate consumption and by the type of carbohydrate - sucrose is the most cariogenic sugar because one of its by-products during bacterial metabolism is glucan, a polymer that enables bacteria to adhere more readily to tooth structures - dietary behaviors, such as consuming sweetened beverages in a nursing bottle or frequently consuming sticky candies, increase the cariogenic potential of foods because of the long retention of sugar in the mouth

Clinicians must ensure that insulin therapy is initiated for children and adolescents with T2DM: who are ketotic or in diabetic ketoacidosis and who have venous or plasma blood glucose level > 250 mg/dl, whose Hemoglobin A1c is > 9% or in whom the distinction between T1D and T2D is unclear. In all other instances, clinicians should start what as first-line therapy for children and adolescents at the time of diagnosis with T2DM, in addition to initiating a lifestyle modification program including nutrition and physical activity?

Metformin

Preferred preoxygenation method for intubation

NRB facemask during preoxygenation (more FiO2) - can place NC while intubation (~30% FiO2)

In an otherwise well infant with new-onset bilateral lower extremity weakness, a what should be suspected?

Neuroblastoma - most common extracranial solid tumor in children - tumor of the sympatico-adrenal axis and can arise from the paraspinal ganglia - can invade through the spinal neural foramina and create an epidural mass causing spinal cord compression - while decompression of the spinal cord can be accomplished through surgery, paraspinal neuroblastomas in infants often span multiple spinal levels and would require a multi-level laminectomy to decompress - as neuroblastoma typically responds very quickly to chemotherapy, it would be appropriate to emergently treat a child with this type of tumor with emergent chemotherapy

Cat-scratch disease (CSD) classically presents as lymphadenopathy, sometimes accompanied by fever. The most common clinical manifestation of atypical cat-scratch disease is:

Oculoglandular syndrome of Parinaud - Cat-scratch disease due to Bartonella henselae is usually a self-limited infection that spontaneous resolves within a few weeks to months. - The most common atypical presentation is Parinaud oculoglandular syndrome, which is unilateral conjunctivitis followed by preauricular lymphadenopathy and occurs in 2-17% of patients with CSD. - Direct eye inoculation as a result of rubbing with the hands after cat contact is the presumed mode of spread. - A conjunctival granuloma may be found at the inoculation site. - The involved eye is usually not painful and has little or no discharge but may be quite red and swollen. - Submandibular or cervical lymphadenopathy may also occur.

In ventilation, this is used to prevent alveolar collapse at end of inspiration, to recruit collapsed lung spaces or to stent open floppy airways.

PEEP

Detecting disease early

Secondary prevention

loss of visual acuity due to active cortical suppression of the vision of an eye and can occur from a variety of causes

amblyopia

most common cause of vision loss in children

amblyopia

S1 heard best at...

apex or left lower sternal border - S3 also heart best at apex or LLSB - S4 heard best at apex

nonstimulant meds in tx of ADHD

atomexetine (strattera) - GI side effects (take after largest meal of day) a-adrenergic adonists - clonidine - kapvay

There are several inflammatory papules and open comedones in a teenager with acne. No scarring is evident, but there are several resolving inflammatory lesions. These lesions may remain erythematous or violaceous for months and are often confused with scars. The most appropriate treatment, therefore, is

benzoyl peroxide (BPO)/clindamycin applied to the face each morning and a retinoid (eg, tretinoin or adapalene) applied at bedtime

Kidney transplant is more common in what gender in kids?

boys

beta-blockers in children have side effects of ...

bradycardia hypotension *hypoglycemia* hypoglycemia-induced seizures hyperkalemia bronchospasm

The hallmark of puberty in girls is usually

breast development - followed by the development of pubic hair, more rapid growth in height, and then menarche. - menarche occurs approximately 2 to 2.5 years after breast development, at an average age of 12 to 12.5 years.

Tx for ophthalmia neonatorum via staphylococcus, streptococcus, and other bacteria

broad spectrum abx (i.e polymyxin B-trimetropim 1 gtt q4hours for 7 days)

What pathogen has been associated with increased mortality in cystic fibrosis?

burkholderia cepacia

When has the bronchial tree developed?

by 16 weeks gestation

Young children with these food allergies have at least a 50% chance of outgrowing them by kindergarten:

cow's milk and eggs - Most children "outgrow" milk and egg allergies, with about 50% doing so within 3-5 yr. - In contrast, about 80-90% of children with peanut, nut, or seafood allergy retain their allergy for life.

A 16-month-old girl presents to the office with her mother who is concerned about a lazy eye. Her mother describes intermittent crossing of the baby's eyes. On exam, intermittent medial deviation of her left eye is appreciated. The corneal light reflexes are asymmetric with the left eye reflex being more lateral when the eye appears to deviate. During a cover test, her left eye shifts when the right eye is covered. What is the correct term for her diagnosis?

esotropia Strabismus - described for the direction the eye deviates. - When the eye turns inward or nasally, such as in the vignette, it is known as esotropia. Esotropia is the most common type of strabismus in children. - It is important to differentiate strabismus from pseudostrabismus. - If a patient has either constant strabismus at any age or intermittent esotropia noted past the age of four months, a referral to ophthalmology is warranted. - It is important to diagnose strabismus in children, because if left untreated, it can result in amblyopia. Amblyopia is the reduction in visual acuity secondary to abnormal visual development.

What adverse event are children 12 to 23 months of age at increased risk of when administered the combination measles, mumps, rubella, and varicella vaccine?

febrile seizure

Time of organogenesis

first 20 wks - diabetes = increased risk of heart malformations

In the first few days after birth, hyponatremia usually indicates...

fluid overload - dilutional hyponatremia - after first wk- it may be either dilutional or indicate true deficit of total body Na+

A burn wound characterized by the absence of painful sensation, bleeding, or capillary refilling is best classified as:

full thickness - or 3rd-degree - involve destruction of the entire epidermis and dermis, leaving no residual epidermal cells to repopulate the damaged area - absence of painful sensation and capillary filling demonstrates the loss of nerve and capillary elements - wound cannot epithelialize and can heal only by wound contraction or skin grafting

If never received TdAP, then comes in at 18 yo

get 1 dose - needs pertussis - then Td every 10 years

In Congenital Hyperinsulinism in cases in which medical management fails, a partial to near-total pancreatectomy is the remaining option. What is a temporizing medication and can be run through a PICC or a PIV?

glucagon 2-3 mcg/kg/hour - must be run with dextrose containing fluid; no chloride; no normal saline - make up concentration 1mg/25ml for 24 hours

In fetal life, pulmonary blood flow (Qp) is low (5-10% of cardiac output) due to ....

high pulm vascular resistance (PVR) & shunts - foramen ovale - ductus arteriosus --> permits blood to bypass pulmonary vascular bed *At birth, PVR normally falls dramatically (due to lung inflation and oxygenation), Qp increased to 100% of CO &, by 24 hours after birth, Ppa has fallen to about 50% of systemic arterial pressure*

Cannulation of ECMO is done by pediatric surgeon, and pt management is by neonatology. With both forms of ECMO, vent settings are decreased to allow recovery of lungs, but generally PEEP is maintained at ...

higher pressure (8 cm H2O) - to prevent atelectasis

Shunting of blood from left to right at the atrial level leads to ...

increased diastolic blood volume in the RV --> right-sided chamber dilation

Medical treatment of symptomatic PDA

indomethacin - if infant fails indomethacin and continues to be symptomatic, surgical ligation can be considered

Why are children given pedialyte during diarrhea which is a hypotonic solution instead of an isotonic solution that's given to adults ?

infantile diarrhea is hypotonic fluid loss, so you cannot give just plain water it would add to the hypotonicity, so we give pedialyte; whereas adult diarrhea is isotonic - infantile diarrhea is mostly by rota virus...which causes destruction of villi...therefore impairing...Na and h2o reabsorption <goljan>

IZZ for age 5

influenza vaccine

What is the main benefit of postpyloric vs. gastric feeds?

less interruption in feeds overall - studies in which feeds have been dyed with methylene blue and then tracheal aspirates examined to determine aspiration into the lungs have not revealed a significant difference in aspiration risk (Kamat et al, PCCM 2008). - Postpyloric feeds typically enter the duodenum, and thus should NOT be bolused like gastric feeds. - feeds are interrupted less when they are postpyloric vs. gastric.

For fat digestion, newborn depends on ...

lingual lipase - stimulated by sucking and swallowing and by nutrients in stomach but not small bowel

The best method to identify prenatal drug misuse is:

meconium testing - more accurate than neonatal urine drug testing

In 2008 in China, baby formula tainted by what product was associated with acute kidney injury from urinary obstruction in thousands of infants?

melamine

Samters triad

nasal polyps, asthma, and aspirin-sensitivity

A 2 day old infant is presented with bilateral conjunctival redness and purulent discharge. The most likely diagnosis is:

ophthalmia neonatorum - characterized by redness and chemosis (swelling) of the conjunctiva, edema of the eyelids, and discharge, which may be purulent

An 18-month-old boy presents to clinic with two days of nasal congestion and low-grade fever and now has developed a harsh cough. When he becomes agitated, a raspy inspiratory noise becomes apparent. Lung auscultation reveals normal air entry throughout all lung fields. No rales or wheezing are appreciated. What is the most likely cause of the child's symptoms?

parainfluenza virus Croup typically - between 6 and 36 months of age. - most prevalent in the fall and winter, mirroring the seasonal variation of parainfluenza virus. - course of croup typically begins with one to two days of nasal congestion and coryza. - child then develops a barking or "seal-like" cough and hoarse voice. - as upper airway inflammation increases, the child may also develop inspiratory stridor. - as inflammation progresses and upper airway obstruction increases, stridor may become apparent when the child is at rest. - there is no cure for croup, but children with stridor can be treated with dexamethasone and nebulized epinephrine to relieve upper airway obstruction. - symptoms of infection resolve within one week.

At 4 weeks old, stooling colors and patterns will change between 4-6 weeks of life. More noticeable in breast fed infant who will go from stooling 8-12 times a day at weeks 2-4 to ...

possibly going several days without a bowel movement - infant infant is feeding normally without increased spitting up and urinating well, parents can relax and expect "explosion" after several days

A toddler who drinks pasteurized milk and avoids fruit is at risk for what disorder?

scurvy - Children fed predominantly heat-treated (ultra-high-temperature or pasteurized) milk or unfortified formulas and not receiving fruits and fruit juices are at significant risk for symptomatic scurvy.

A 1 day old infant is cyanotic. Hyperoxia test shows a PaO2 of 160 mmHg on 100% FiO2. Of the following, which diagnosis is most likely?

meconium aspiration syndrome Hyperoxia test - one method of distinguishing cyanotic congenital heart disease from pulmonary disease - neonates with cyanotic congenital heart disease are usually not able to significantly raise their PaO2 during administration of 100% oxygen - if the PaO2 rises above 150 mm Hg during 100% oxygen administration, an intracardiac right-to-left shunt can usually be excluded, although this is not 100% confirmative, because some patients with cyanotic congenital heart lesions may be able to increase their PaO2 to > 150 mm Hg because of favorable intracardiac streaming patterns

Average time from initiation of toilet training to completion is

3-6 months

Permanent teeth should start to present at appx

6 yo

Most common mediastinal germ cell tumor

teratomas - arise from pleuripotential stem cells from third pharyngeal pouch which descend into thorax with thymus

Individuals with Turner syndrome can have congenital heart disease in the form of

- coarctation of the aorta - bicuspid aortic valve - aortic dilation - anomalous pulmonary venous drainage - persistent left superior vena cava - sinus tachycardia - prolonged QT

Hyperinsulinsm treatment

- continuous dextrose IV infusion to maintain euglycemia (know GIR) - trial diazoxide (can use for transient or congenital HI) = 10 mg/kg/day TID (range 5-15 mg/kg/day) - surgery

Timeout timing for kids

1 minute per year of age >3 - don't start until 3 (when they begin understanding)

Formula changes take how long to work?

1-2 weeks

Typical prednisone or prednisolone dosage for pediatric asthmatic

1-2mg/kg/day PO daily or divided BID

Four doses of DTaP should be administered during the 1st 2 years of life, generally at ages ...

2, 4, 6, and 15-18 mo of age - 4th dose may be administered as early as 12 mo of age, provided that 6 mo have elapsed since the 3rd dose - 5th dose of DTaP is recommended for children at 4-6 yr of age; a 5th dose is not necessary if the 4th dose in the series is administered on or after the 4th birthday

In the NICU, once baby is getting 60 ml/kg/day of enteral feeds, fortify to ...

24 kcal/oz

Suture choice for palms or soles

3-0, 4-0 - remove after 14 to 21 days

Re-screen for HIV how many months after the diagnosis of a STI?

3-4 months

For pts with hemodynamically significant ASDs, they are referred for intervention at what age?

3-4 yo

Referral thresholds for visual acuity

3-4 yo = 20/40 or worse 5 years and older = 20/32 or worse or 2-line difference between eyes

Age: balance for each foot for 2 seconds

48 mos

Speech all intelligible by what age?

48 mos

EKG 1 large square =

5 mm = 0.2 sec

In the United States, priming doses of what vaccines are all given at 2, 4, and 6 months?

DTaP, Hib, rotavirus, PCV13, and inactivated polio vaccine (IPV) - although some formulations of Hib and rotavirus are given at 2 and 4 months

14 days old, bili @ 12, direct is 0.5. Baby regained birth weight, otherwise healthy.

Breast milk jaundice - breast milk has glucuronidase and de-conj bili

6 months old born at 32 wks. What ASQ interval should be used?

Ex. 6 months old born at 32 wks - baby was 8 weeks premature - 24 - 8 wks = 16 weeks = *4 month ASQ interval used*

EKG left axis deviation

Hands don't shake = left axis deviation look at leads I & 3

What is the most common infectious agent associated with febrile seizures?

Human herpesvirus 6 - causes the childhood exanthem known as roseola or exanthem subitum.

Most common cause of hemoptysis in children

Infection with or without bronchiectasis

IZZ for age 4

Kinrix (DTaP #5 / IPV #4) Proquad (MMR #2 / VZV #2) Influenza vaccine

Often described as a dull ache at the time of ovulation, usually occurring 1 to 2 weeks before the onset of menses, and can last for a few minutes to several hours.

Mittelschmerz

Neural tube defects (NTDs) can best be prevented by:

Preconception ingestion of at least 400 µg/day of folic acid - Because the neural tube closes within the 1st 28 days of conception, periconceptional supplementation is needed for prevention. - It is recommended that women without a prior history of an NTD ingest 400 μg/day of folic acid throughout their reproductive years. - Women with a history of a prior pregnancy complicated by an NTD or a 1st-degree relative with an NTD should have preconceptional counseling and should ingest 4 mg/day of supplemental folic acid beginning at least 1 mo before conception.

Steps to calculating insulin dosing

Step 1: Calculate Total Daily Dose (TDD): - Pre Pubertal: 0.5 units/kg/day - Post Pubertal: 0.7 units/kg/day Step 2: Calculate Basal Basal = 0.5 x total daily dose Step 3: Calculate Bolus - Carbohydrate factor = 500/total daily dose - High BG factor = 1800/total daily dose Step 4: Establish a target BG - Safe target is generally 120

What is Seidel sign?

The presence of a stream of fluorescein dye (after application) secondary to globe rupture.

How is the absolute neutrophil count calculated?

The sum of the mature granulocytes plus the neutrophil band cells.

Characterized by a prominent white forelock, wide nasal bridge, sensorineural deafness, heterochromia iridis, and in some types, lateral displacement of the inner canthi.

Waardenburg syndrome - Waardenburg syndrome type 4 (Waardenburg-Shah syndrome) is always associated with Hirschsprung disease

For all of the lysosomal storage disorders, inheritance is autosomal recessive except for

X-linked Fabry disease

Within what time period should newborns exposed to human immunodeficiency virus receive prophylactic treatment following birth?

Within 6 to 12 hours.

the patient initiates the breath

assist breath

Typical duoneb dosage for peds asthmatic

duoneb = 2.5 mg albuterol + 0.5 mg ipratropium

The hallmark of puberty

girls = breast development boys = testicular enlargement

What procedure is used to remove thyroglossal duct cysts?

sistrunk procedure

The PALS systematic approach initial impression consists of

"Consciousness, Breathing, and Color"

Language of a 24 month old

"twos at two years" two word phrases more than 50-75 words (often 200 words) follows two step command listens to short stories uses pronouns (but often incorrectly) points to items in a book knows names of body parts

Endotracheal tubes and laryngoscope - size for infant vs preterm

#1 blade for term infant #0 for preterm - use Miller #1 blade for term infants and Miller #0 for smaller infants

In the NICU, glucose infusion rate (GIR) =

(ml/kg/day) x (Dextrose percentage as decimal) x 0.69 (time constant) or (rate ml/hour) x (% dextrose) / (weight in kg) x (6)

The biggest risk factor for isolated neural tube defect is folic acid deficiency. Which of the following is also a risk factor?

*maternal obesity* - Screening for open neural tube defects should be offered to all pregnant women, and ultrasound screening at 18 to 20 weeks is recommended. - Open neural tube defects are often the result of folic acid deficiency and recent fortification of foods with folic acid and supplementation of pregnant women with folic acid has shown that many of these defects are preventable. Additionally, women who are obese have double the risk of having an infant with an open neural tube defect.

How to place a TST/PPD

- Inject 0.1 ml of tuberculin - If a wheal doesn't form, repeat - Record time and location of placement - Review instructions to return for reading

The first step of neonatal resuscitation asks three questions to determine if the baby is ok by asking what questions:

- Is this a term gestation - Is there good tone - Is the baby breathing or crying?

Start-out mid-tier acne regimen

- OCPs can also help with acne but.... - benzoyl peroxide wash 1x/day - light moisturizer, like cetaphil 1x/day - tretinoin cream with 5 dot method 1x/day after light moisturizer --> if too irritating, can transition to every other day (counsel things may get worse before getting better at to expect result appx in 6 weeks)

Narrow pulse pressure differential

- aortic stenosis - pericardial effusion - pericardial tamponade - pericarditis - significant tachycardia - cold shock narrow pulse pressure = <25 mmHg pulse pressure = systolic pressure - diastolic pressure

An expectant mother presents to clinic for a prenatal visit. Her father, the baby's grandfather, has Marfan syndrome. She is unaffected, as is the father of the baby. What is the likelihood that the baby will inherit Marfan syndrome?

0% Marfan syndrome - autosomal dominant disease usually caused by mutation in the FBN1 gene. - Thus, if the expectant mother is unaffected, she did not inherit the mutation from her father and does not have a mutation to pass to her unborn child. - The same is true for the father of the baby—if he does not phenotypically have Marfan syndrome then he also does not have a mutation to pass to his children. Because of this, the likelihood that their child will inherit Marfan syndrome is 0%.

The only 2 premeasured fixed doses of epi currently available in EA formulations in US and Canada...

0.15 mg and 0.3 mg - pts weight 15-30 kg = 0.15 mg dose - children 30 kg and over = 0.3 mg

Typical methylprednisolone dosage for pediatric asthmatic

0.5-1mg/kg IV Q6 - 12hr

Start photoscreens at what age?

1 year

Birth weight triples by ....

1 year - 7 lbs --> 21 lbs

Klippel-Trenauney Syndrome classic triad

1) limb PWS; 2) varicose veins; 3) hyperplasia of soft tissues and/or bone (limb overgrowth)

UCSF IDMP guidelines for pyelo treatment in pediatrics

1. cipro BID 2. levofloxacin 3. bactrim + ceftriaxone x1 dose

For all local anesthetics, 1% solution =

10 mg/dL

Recurrence rate of intussusception

10% - most common in first 2 days following reduction

Standard insulin concentration is

100 units/mL (round to nearest 0.5 unit) - Toujeo is a concentrated insulin with 300 units/mL - For younger children, you may see diluted insulin in order to give smaller amounts

Age: stoop and recover

15 mos

Age: wave bye-bye. Protodeclarative pointing.

15 mos

Age at which kids walk backwards

18 mos

Growth between 3 and 4 mo of age is best characterized as slowing to a rate of...

20 g/day - by 4 mo, birthweight has doubled

If you take a total of 25 units of insulin in a typical day, each unit of insulin should cover approximately

20 grams of carbohydrate (500 ÷ 25 = 20)

Usual dose of iNO in infants...

20 ppm - produces maximal pulmonary vasodilation in vast majority of infants PPHN

Age? - have greater than 50 words

24-months

A nurse calls to request evaluation of a 12-hour-old newborn whose mother did not receive prenatal care, but who is estimated to be full-term and was vigorous upon vaginal delivery, with Apgar scores of 8 and 9 at one and five minutes, respectively. Since delivery, the mother has tested positive for human immunodeficiency virus. She was previously unaware of having this infection. Of the following, what is the infant's approximate risk of acquiring human immunodeficiency virus if prolonged breastfeeding is not performed?

25% - In the absence of maternal treatment with appropriate antiretroviral therapy, the risk of maternal-to-child transmission of human immunodeficiency virus (HIV) from infected mothers to their infants is 25% to 30%. - In resource-rich settings such as the United States, it is also appropriate to counsel mothers infected with HIV to avoid breastfeeding in order to decrease the risk of viral transmission through breast milk.

Turnover of red blood cells in babies

30 days - vs 100-120 days in adults

Suture choice for legs

4-0, 5-0 - remove after 10-14 days

Cephalosporins that penetrate the CSF and are effective for treatment of pediatric meningitis, has activity against P. aeruginosa, and retains good activity against methicillin-susceptible staphylococcal infections.

4th generation cephalosporins (e.g., cefepime)

WHO/CDCACIP /AAP recommends routine vaccination of tetanus, diphtheria, and pertussis for: Infants and young children with

5 dose primary series of diphtheria and tetanus toxoids and acellular pertussis (Dtap) vaccine

Children who have EIB experience cough, shortness of breath, and rarely, wheezing, which begins during vigorous activity, reaches a peak when?

5 to 10 minutes after stopping exercise - resolves 20 to 30 minutes later

Example: Assume your total daily insulin dose (TDI)= 160 lbs ÷ 4 = 40 units In this example: Carbohydrate coverage ratio =

500 ÷ TDI (40 units)= 1 unit insulin/ 12 g CHO

Accommodations in school for diabetic child with no other learning difficulties...

504 accommodation - such as ability to check his own blood sugar level and have sugar candy during class time

Flow rates used during application of CPAP in infant...

6 L/min

Who needs a flu vaccine booster?

6 months - 8 years for their first ever flu vaccine - at least 28 days apart

The probable age of a developmentally normal child who is just able to sit without support, can transfer objects from hand to hand, and speaks in a monosyllabic babble is:

6 mos

Minimum age for MMR vaccine

6 mos - usually 12 mos though - doesn't count towards actual 2 doses of vaccine

Avoid juice in babies. If juice is introduced, do so at what age?

6-9 months - limit consumption to 4-6 ounces

A 10 mo old infant presents with vomiting and diarrhea, tachycardia, normal blood pressure, dry mucous membranes, a capillary refill time of 2 sec, deep respirations, and irritability. The percent dehydration for this patient is:

6-9% Clinical signs of moderate dehydration - tachycardia - little or no urine output - irritable/lethargic - sunken eyes and fontanel - decreased tears - dry mucous membranes - mild delay in elasticity (skin turgor) - delayed capillary refill (>1.5 sec) - skin cool and pale. Infants with moderate dehydration are typically 5-10% dehydrated.

Initial fluid admin: for larger infants (>1250 g), start at ....

60 mL/kg/d of D10W - if estimated ILW is high (extreme prematurity, abdominal wall defect), start fluids at higher rate, 80-100 mL/kg/d

In newborn, saturation goal at 2 minutes...

65-70%

Travuda can be used as PREP in adolescents <18 yo if....

>35 kg

Normal FEF 25-75% (marker of peripheral airway obstruction) =

>80%

What is the recommended daily total dose of elemental iron needed for treatment of iron deficiency anemia?

A daily total dose of 3-6 mg/kg of elemental iron is needed.

A 1-year-old boy is brought to the emergency department for evaluation of poor feeding, irritability, and sweating. The child is lethargic but arousable. He has labored breathing, very rapid pulses, and dusky color. His RR is 68/min, HR 300/min, and BP 70/45 mmHg. He has weak brachial pulses and absent radial pulses, a capillary refill of 6 seconds, SPO2 85% on RA, and good bilateral BS. You administer high-flow O2 and place the child on a cardiac monitor and see the rhythm (SVT). The child has no history of congenital heart disease IV access has beeb established. Which therapy is most appropriate for this child?

Adenosine 0.1 mg/kg IV rapid push

Which type of severe combined immunodeficiency may be treated with enzyme replacement?

Adenosine deaminase (ADA) deficiency - has been treated with polyethylene glycol-modified bovine adenosine deaminase (PEG-ADA)

Accounted for 74 of the 108 poison-related pediatric deaths in 2008 reported to the National Poison Data System ...

Adolescents (ages 13-19 yr)

Epinephrine for mild symptoms:

Adults: 0.3 to 0.5 mL 1:1000 (1 mg/mL) solution IM q15 minutes Children: 0.01 mg/kg 1:1000 (1 mg/mL) solution IM q15 minutes

Epinephrine for severe symptoms:

Adults: 1-5 mL 1:10,000 (0.1 mg/mL) solution IV over 10 minutes Children: 0.01 mg/kg 1:10,000 (0.1 mg/mL) solution IV prn

Available LABA + ICS

Advair = fluticasone + salmeterol - MDI inhaler = >6 yo - Diskus = >6 yo Symbicort = budesonide + formolerol - MDI = 5-12 yo Dulera = mometasone + formoterol - MDI inhaler

After exposure to mumps - unvaccinated children 12 months of age and older should be given: - children who have already received 1 dose of the vaccine at least 28 days prior should receive: - high-risk individuals who have already received the recommended 2 doses of mumps vaccine should receive:

After exposure - unvaccinated children 12 months of age and older should be given 1 dose - children who have already received 1 dose of the vaccine at least 28 days prior should receive a second dose - high-risk individuals who have already received the recommended 2 doses of mumps vaccine should receive a third dose

Most common syndrome with intrahepatic bile duct paucity ...

Alagille syndrome (arteriohepatic dysplasia) - Bile duct "paucity" designates an absence or marked reduction in the number of interlobular bile ducts in the portal triads, with normal-sized branches of portal vein and hepatic arteriole

Holt-Oram - common heart defect

Also ASD malformation

Girl with clinical pic suggestive of cellulitis of the left forearm after a cat scratch. In the setting of cat scratch or bite, the most frequent pathogen is Pasteurella multocida. What is the antimicrobial agent of choice for the treatment of infected cat bite wounds, given its excellent coverage against Pasteurella and other aerobic-anaerobic bacteria often found in animal bites?

Amoxicillin-clavulanate - duration of therapy for bite infection is determined by location, severity, and isolated pathogen - cellulitis is usually treated for 10 to 14 days - extended treatment up to 3 weeks is recommended for tenosynovitis, 4 weeks for septic arthritis, and 6 weeks for osteomyelitis

What are the most common maternal antibodies to cause neonatal lupus?

Anti-Ro and anti-La antibodies

Premedication for Non-emergent Intubation For routine, non-emergent intubations in the NICU, premedication should be used in almost all cases ...

Atropine - 0.02mg/kg - Give over 1 minute. Cisatracurium (paralytic) - 0.2mg/kg- Give rapid IV push. - As soon as it appears to start having physiologic effects, administer the Fentanyl. Fentanyl - 2mcg/kg- Given IV push - Chest wall rigidity will not be an issue if the paralytic has taken effect

Is indicated in the evaluation of patients with recurrent pneumonia, persistent cough of undetermined cause, stridor, or persistent wheezing.

Barium swallow - The technique can be modified by using barium of different textures and thicknesses, ranging from thin liquid to solids, to evaluate swallowing mechanics, the presence of vascular rings, and tracheoesophageal fistulas, especially when aspiration is suspected.

Benign vs malignant bone tumors on radiographic imaging

Benign bone lesions on radiographic imaging = solitary, discrete lesions with sclerotic margins Malignant tumors = ill-defined margins, periosteal reactions, or soft-tissue extension - Pain related to osteoid osteomas typically responds well to nonsteroidal anti-inflammatory drugs, worsens towards the end of the day, and is not related to activity. - Resection of the lesion has been the historical standard of care for osteoid osteomas, although techniques such as percutaneous radiofrequency or thermal ablation have become the approaches of choice.

Usually considered irreversible localized or diffuse bronchial dilation secondary to bronchial wall destruction.

Bronchiectasis - 3 types = cystic, cylindrical, saccular

Most common lower respiratory tract infection in first 2 years of life

Bronchiolitis - RSV = most common

What is the mechanism by which growth hormone stimulates linear growth?

By triggering cartilage growth at the epiphyseal plate.

What is the most common location for pediatric pseudosubluxation?

C2-C3

40 and 70 mm Hg (85-95% saturation) in order to maintain normal tissue oxygenation while minimizing the risk of oxygen toxicity. If oxygen saturation cannot be kept > 85% at inspired oxygen concentrations of 40-70% or greater, applying what is indicated?

CPAP at a pressure of 5-10 cm H2O via nasal prongs i - CPAP prevents collapse of surfactant-deficient alveoli and improves both FRC and ventilation-perfusion matching

Posterior mediastinal masses better evaluated by...

CT or MRI

What could an abdominal X-ray show in a patient with factitious diarrhea from prolonged laxative use?

Cathartic colon - dilation of the large bowel absent haustrations

A persistent thumb-sucking habit in a 7 yr old child will cause...

Cause the maxillary permanent incisor teeth to protrude - Prolonged digit sucking can cause flaring of the maxillary incisor teeth, an open bite, and a posterior cross bite. - The prevalence of digit sucking decreases steadily from the age of 2 yr to ≈10% by the age of 5 yr. - The earlier the habit is discontinued after the eruption of the permanent maxillary incisors (age 7-8 yr), the greater the likelihood that there will be lessening effects on the dentition.

Cephalosporin that is highly active against most strains of P. aeruginosa, making this a useful agent for febrile, neutropenic oncology patients.

Ceftazidime

You are evaluating an irritable 6-year old girl with mottled skin color. The patient is febrile (40C/104F), and her extemities are cold with capillary refill of 5 seconds. Distal pulses are absent and central pulses are weak. HR is 180/min, RR is 45/min. and BP is 98/56 mmHg. How would you categorized this child condition?

Compensated shock associated with tachycardia and inadequate tissue perfusion

The mother of a 4-month-old boy was recently diagnosed with shingles on her lower abdomen. The boy was born at term and has been growing and developing normally with no intercurrent illnesses. His mother is wondering if she can continue to breastfeed. Of the following, the MOST accurate response is that she should

Continue breastfeeding without interruption - Virus can be transmitted from direct contact with herpes zoster lesions. However, because the mother can and should cover the lesions to prevent her breastfeeding infant from coming into contact with them, she may continue to breastfeed without interruption. At 4 months of age, this infant is too young to receive the varicella vaccine. Active shingles is not an indication for a mother to stop breastfeeding or pump and discard her milk.

Goal feedings for first 3 days of life

Day 1: 15 cc q3h Day 2: 30 cc q3h Day 3: 2 oz q3h

Your 60kg patient with DKA is on an insulin gtt at 0.1 u/kg/hr, is receiving D12.5NS + 20KCl and 20 KPhos @200 ml/hr. The RN comes to you with a blood sugar of 70. What do you do?

Decrease the insulin gtt - While you do not want to shut off the insulin infusion, it is reasonable to decrease the rate of the infusion (ie 0.1 u/kg/hr to 0.05 u/kg/hr). - D15 cannot be given peripherally and hence, would not be a good option here. - While you could increase the rate of the infusion to 250 ml/hr, the patient is already receiving 2X maintenance fluids and additional fluid may have some association with increased risk of cerebral edema, although this remains controversial. - Generally patients are NPO and may not tolerate PO intake when they are in the PICU with DKA, making giving them orange juice not the best option.

An 18-month-old girl presents to clinic with a history of recurrent bacterial and viral infections. You are suspecting an immunodeficiency. A medical student rotating in your clinic inquires about how to evaluate the different components of the infant's immune system. Which of the following is the best diagnostic test to evaluate a defect in cell-mediated immunity?

Delayed cutaneous hypersensitivity test - Initial screening tests include a complete blood count with differential, electrolytes, glucose, blood urea nitrogen, creatinine, albumin, and a urinalysis. If screening tests are abnormal or if there is a convincing history of immunodeficiency, further diagnostic tests can be performed. - A diagnostic test that can detect defects in cell-mediated immunity is delayed cutaneous hypersensitivity testing using candida antigen. The test qualitatively evaluates T-cell response to an intradermal injection of an antigen to which an individual has already been exposed over a period of time. Therefore, it should be used in children over 1 year of age. Induration of more than 5 mm within 48 to 72 hours is positive in children and can exclude all primary T-cell defects. Other antigens used for delayed-type hypersensitivity testing include tetanus, tuberculin and Trichophyton. If cutaneous delayed-type hypersensitivity testing results are negative, confirmatory tests such as lymphocyte counts, quantitative testing of T-cell subpopulations by flow cytometry, and lymphocyte stimulation testing should be performed.

Tx for croup

Dexamethasone -Dose: 0.6 mg/kg/dose po (IVform orally, can also give IM) - max: 16mg-give alone for mild croup for decreased sx within 6 hours, reduced return to / time in ED, improved time to sx resolution Racemic epinephrine -use when stridor at rest or severe upper airway obstruction -dose: 0.05-0.1ml/kg up to 0.5ml max-observe up to 4 hours Discharge if steroids given, no stridor at rest, intact mental status -mist therapy-tent or 40-100% humidified O2 for severe croup

Characterized by small amounts of blood and mucus in the stools of otherwise healthy infants.

Dietary protein-induced colitis Treatment = elimination of cow milk and soy protein from the mother's diet for breastfed infants and feeding an amino acid-based formula for formula-fed infants.

A mother requests early discharge of her neonate, who is 36 hours of age, so she can be home with her 2-year-old daughter and husband. Her mother is also staying at her home to help. The boy was born at 38 weeks 6 days in a normal spontaneous vaginal delivery without any complications after an uneventful pregnancy. He has been nursing well, voided four times, and passed stools three times. His temperature is 37.2°C, his heart rate is 120 beats/min, his respiratory rate is 40 breaths/min, and his oxygen saturation is 98% on room air. His physical examination findings are normal. Of the following, the BEST next management step for this neonate's care is to

Discharge now and f/u within 28 hours - Neonates at low risk of experiencing complications can be safely discharged 24 hours after birth. - Neonates discharged before 48 hours of age should follow up with a pediatric provider within 48 hours.

Most frequently identified infection triggering HLH

EBV - followed by CMV

This morning an 8 yr old boy experienced the abrupt onset of fever, irritability, and headache, followed by lethargy, confusion, seizures, and coma. He had accompanied his parents on a picnic and boating outing in the saltwater marshes along the New Jersey coastline 1 wk earlier, on September 3. The peripheral white blood cell count demonstrates a marked leukocytosis. Which of the following is the most likely diagnosis?

Eastern equine encephalitis - very low incidence disease, with a median of 8 cases occurring annually in the Atlantic and Gulf States from 1964-2007 - result in fulminant encephalitis with a rapid progression to coma and death in one third of cases - in infants and children, abrupt onset of fever, irritability, and headache are followed by lethargy, confusion, seizures, and coma - high temperature, bulging fontanel, stiff neck, and generalized flaccid or spastic paralysis are observed. - unlike most other viral encephalitides, the peripheral white blood cell count usually demonstrates a marked leukocytosis, and the cerebrospinal fluid (CSF) may show marked pleocytosis - pathologic changes are found in the cortical and gray matter, with viral antigens localized to neurons - there is necrosis of neurons, neutrophilic infiltration, and perivascular cuffing by lymphocytes

The hallmark of duodenal obstruction is bilious vomiting without abdominal distention, which is usually noted on the 1st day of life. The diagnosis is suggested by the presence of a "double bubble" sign on a plain abdominal radiograph. The initial treatment of infants with duodenal atresia includes nasogastric or orogastric decompression and intravenous fluid replacement. What should be performed to evaluate for associated anomalies?

Echocardiography Renal ultrasound Radiology of the chest and spine

Adequate nutritional support is required for optimal wound healing in pediatric patients with burns. What route is preferred?

Enteral route - to ensure positive nitrogen balance and reduce gastrointestinal bacterial translocation - for children with large total body surface area burns, caloric needs will be higher than usual resting energy expenditure - titamins C and A and zinc sulfate should be prescribed in burn management to ensure adequate wound healing

In children, this can present as steatorrhea, severe diaper rash, failure to thrive, and/or fat-soluble vitamin deficiency.

Exocrine pancreatic insufficiency - While cystic fibrosis is the most common cause of exocrine pancreatic insufficiency in children, other causes include: chronic pancreatitis, Shwachman-Diamond syndrome, Pearson syndrome, and Johanson-Blizzard syndrome. - Exocrine pancreatic insufficiency is managed with pancreatic enzyme replacement therapy and supplementation of fat-soluble vitamins.

There are 3 infection phases for Dengue:

Febrile Critical - where patients will deteriorate and have symptoms of plasma leak and hemorrhage occurring for about 24-48 hours often (but not always) as the fever starts to subside (often day 3-7 but not always) Convalescent - when patients improve with resolution of laboratory values and generally without health problems but they may have post-viral fatigue syndrome

insensible water loss (IWL) can be calculated by

Fluid intake - Urine output + weight loss (or - weight gain) e.g., 24-hour totals = intake 90 mL, urine output 60 mL, and weight loss 55 g. Therefore, IWL = 90 - 60 + 55 = 85 mL

You are supervising a student who is inserting an IO needle into an infant's tibia. The student asks you what she should look for to know that she has successfully inserted the needle into the bone marrow cavity. What do you tell her?

Fluids can be administered freely without local soft tissue swelling

What to ask when called by the OB when called for a delivery...

GA infa risk factors GBS status chorio fever +/- max

Indications for intubation of preterm infant during resuscitation...

GA </= 27 wks and no maternal steroids Infants with any of the following - apnea - requires FiO2 >0.4 - pH <7.25 - unable to maintain adequate airway - increased work of breathing (grunting, retractions, flaring) - PaCO2 >60 mmHg

Hallmarks of salicylism

Gastrointestinal and neurological symptoms - causes a mixed acid-base disorder with a respiratory alkalosis and an elevated anion-gap metabolic acidosis - management of salicylism consists of intravenous fluids and urinary alkalinization to facilitate salicylate excretion

AR disease whose main enzyme defect is acid beta-glucosidase which causes accumulation of glucosylceramide, an indigestible lipid.

Gaucher Disease - occurs in macrophage-lineage organs - primary systems affected: liver, spleen, bone marrow, lungs, CNS

You diagnose measles in a patient today and realize he has shared the waiting room with the following patients: a well 4 mo old infant, a 2 yr old immunized child, and a 12 moh old unimmunized child. Which postexposure prophylaxis strategy is appropriate?

Give the 4 mo old infant measles immune globulin to prevent infection within 48 hr and Immunize the 12 mo old child with MMR within 48 hr - The vaccine is effective in prevention or modification of measles if given within 72 hr of exposure. - Immune globulin may be given up to 6 days after exposure to prevent or modify infection. - Immunocompetent children should receive 0.25 mL/kg intramuscularly, and immunocompromised children should receive 0.5 mL/kg (maximum dose in both cases is 15 mL/ kg). - Immune globulin is indicated for susceptible household contacts of measles patients, especially infants < 6 mo of age, pregnant women, and immunocompromised persons.

What medications can be used to reduce sialorrhea in patients with cerebral palsy?

Glycopyrrolate Scopolamine Benztropine Trihexyphenidyl

Grade murmur: audible with stethoscope off of chest

Grade VI

What bacteria classically causes concomitant acute otitis media and conjunctivitis?

Haemophilus influenzae.

Air throughout bowel to rectum in newborn suspicious for...

Hirschsprung's disease

An adolescent with a history of spelunking presents with weight loss, fatigue, dyspnea, and fever with onset 2 wk previously. Chest radiograph shows evidence of pneumonia. The most likely causative organism is:

Histoplasma capsulatum - Acute pulmonary histoplasmosis follows initial or recurrent respiratory exposure to microconidia - The median incubation time is 14 days - Hepatosplenomegaly occurs more often in infants and young children - Symptomatic infections may be associated with significant respiratory distress and hypoxia and can require intubation, ventilation, and steroid therapy - Acute pulmonary disease can also manifest with a prolonged illness (10 days to 3 wk) consisting of weight loss, dyspnea, high fever, asthenia, and fatigue - Most children with acute pulmonary disease have normal chest radiographs - Patients with symptomatic disease typically have a patchy bronchopneumonia; hilar lymphadenopathy is variably present

Treatment for Congenital Adrenal Hyperplasia

Hydrocortisone and fludrocortisone (increased doses in times of stress)

Most common complication of refeeding syndrome

Hypophosphatemia, - Can result in cardiac arrhythmia, respiratory failure, weakness, hemolysis, and mental status changes - Hypophosphatemia, hypomagnesemia, and hypokalemia can occur due to intracellular electrolyte shifts as refeeding begins

Hypoglycemia in NPO patient. How to treat?

IV dextrose fluids = 0.2 - 0.5 g/kg bolus of dextrose Rule of 50's (5/2/1) - D10 = 5 ml/kg - D25 = 2 ml/kg - D50 = 1 ml/kg

Preferred treatment Guillain Barre for most cases given its tolerability and favorable adverse effect profile.

IVIG

Phototherapy can increase insensible water loss (IWL) by 25-50%. IWL may exceed urinary output in smaller infants but, unlike urine output, IWL cannot be measured directly. However, IWL must be estimated in order to plan appropriate fluid management. IWL can be estimated by....

IWL = Fluid intake - Urine output + weight loss (or - weight gain) Ex. 24-hour totals = intake 90 mL, urine output 60 mL, and weight loss 55g. IWL = 90 - 60 + 55 = 85 mL

The mother of an 8-yr-old boy with acute streptococcal tonsillitis calls to report that now, within 15 min after the first dose of oral penicillin V that you prescribed, he is complaining of itching and has developed hives. Which of the following should you recommend?

Immediate return to your office or the nearest emergency department - In anaphylaxis, the onset of symptoms may vary somewhat depending on the cause of the reaction. - Reactions from ingested allergens (foods, medications) are delayed in onset (minutes to 2 hr) compared with those from injected allergens (insect sting, medications) and tend to have more gastrointestinal symptoms - Cutaneous symptoms may be absent in up to 20% of cases, and the acute onset of severe bronchospasm in a previously well asthmatic person should suggest the diagnosis of anaphylaxis.

How to use vaginal ring?

In 3 weeks; out 1 week Continuous use: change first of each month - estrogen and progestin - 4 weeks of medication in ring - inhibits ovulation

Recognize nevus sebaceous, which is a tan, hairless plaque with a velvety texture that was present at birth. Families should be advised that there is a small risk of a growth developing within the lesion after puberty.

In most cases, these are benign neoplasms, but rarely, a malignant lesion, like basal cell carcinoma, may occur.

How quickly can you drop blood pressure in a patient with hypertensive emergency, particularly in a case when you don't know how long the patient has had hypertension?

In terms of rate, the goal is typically to drop the blood pressure by 25% over 6-8 hours, ideally to a level where symptoms of hypertensive emergency improve. You can then reduce to a normal blood pressure range over the next 24-72 hours. Most useful to choose a fast acting, titratable medication such as nicardipine as the first-line agent to decrease blood pressures - Might use a dose or two of labetalol or hydralazine while waiting for the drip but ultimately want to start the drip and admit to the PICU. - If a nicardipine drip is insufficient in controlling blood pressure, you can also add an esmolol and/or nitroprusside drip.

Definition of pulmonary hypertension

Increased PA pressures 1) mean PA pressure >25 mmHg at rest or >30 mmHg with exercise 2) Echocardiogram indicating systolic PA pressure >1/2 systemic systolic pressure with or without acute and/or chronic right ventricular failure

oxygen-challenge test

Infants with cyanotic heart lesions have intracardiac right to left shunting, resulting in low PaO2 ... - Provide 100% oxygen for 10-20 minutes. Obtain arterial blood gas to assess PaO2. - Infants with cyanotic lesions typically cannot achieve a PaO2 of >100mmHg on 100% oxygen - Terrible T's: Truncus Arteriosus, Transposition of the Great Arteries, Tricuspid Atresia, Tetralogy of Fallot, Total Anomalous Pulmonary Venous Return

The highest incidence of retinoblastoma has been noted in which of the following age groups?

Infants/toddlers 0-2 yr of age - The median age at diagnosis is approximately 2 yr, and over 90% of cases are diagnosed in children < 5 yr of age - Overall, about two thirds to three fourths of children with retinoblastoma have unilateral tumors, with the remainder having bilateral retinoblastoma - Bilateral involvement is more common in younger children, particularly in those diagnosed < age 1 yr

Aside from mumps, what other viruses are known to cause parotitis?

Influenza, parainfluenza, cytomegalovirus, and enteroviruses.

Standard of care for all categories of asthma except for mild intermittent asthma

Inhaled corticosteroids - increased beta2 receptor affinity

Septic Shock: Initial Diagnostics - Initial Treatment -

Initial Diagnostics - Find source - Can source be removed? = pre-op labs, surgery consult, image (US, MR, CT, ex-lap) - Can source be cultured? Blood and Urine - Labs - think: CBC, Lactate, CMP, Lipase, UA Treatments - IV access (labs at same time) - DFT-NO: Dilaudid, Tylenol, Fluids, NPO, Oxygen - Abx

A 4 mo old infant with mild stridor since the 1st week of life is diagnosed with laryngomalacia. He spits up often but has been growing well. What is the most appropriate treatment recommendation?

Initiate ranitidine or pantoprazole therapy - Laryngopharyngeal reflux is managed aggressively. - For the few patients who have such severe obstruction that surgical intervention is unavoidable (patients with apparent life-threatening events, cor pulmonale, cyanosis, failure to thrive) endoscopic supraglottoplasty can be used to avoid tracheotomy.

In cases of suspected Kingella infection, what increases the odds of culture positivity?

Injecting samples such as synovial fluid into a blood culture medium. Polymerase chain reaction for Kingella is being used with increased frequency.

Includes data from all patients who were randomly assigned to a group even if they did not complete the study.

Intention-to-treat analysis - preserves sample size and improves the power of a study - can help reduce bias

What is the antimicrobial prophylaxis for close contacts of patients with diphtheria?

Intramuscular benzathine penicillin G or oral erythromycin.

What is atrovent?

Ipratropium bromide - trade name Atrovent among others - opens up the medium and large airways in the lungs - used to treat the symptoms of chronic obstructive pulmonary disease and asthma - used by inhaler or nebulizer - onset of action is typically within 15 to 30 minutes - lasts for three to five hours - metered dose inhaler

The recommended treatment for active pulmonary tuberculosis in children is:

Isoniazid and rifampin for 6 mo, with pyrazinamide and ethambutol added during the 1st 2 mo - The standard therapy for intrathoracic tuberculosis (pulmonary disease and/or hilar lymphadenopathy) in children recommended by the CDC and AAP is a 6-mo regimen of isoniazid and rifampin supplemented in the 1st 2 mo of treatment by pyrazinamide and ethambutol. Several clinical trials have shown that this regimen yields a success rate approaching 100%, with an incidence of clinically significant adverse reactions of < 2%.

Why is incision and drainage contraindicated in cases of suspected nontuberculous mycobacterial lymphadenitis?

It may create a draining sinus tract.

Patients with severe forms of reactive airways disease (e.g., asthma) who require mechanical ventilation may benefit from which of the following initial ventilator parameters?

Low rates, prolonged inspiratory/expiratory times, and moderate tidal volumes (10-12 mL/kg) - In a patient with relatively normal lungs, an age-appropriate ventilator rate and a tidal volume of 7-10 mL/kg would be appropriate initial settings. - Diseases associated with decreased time constants (decreased static compliance, e.g., ARDS, pneumonia, pulmonary edema) are best treated with small (6 mL/kg) tidal volume and relatively rapid rates (25-40 breaths/min). - Diseases associated with prolonged time constants (increased airway resistance, e.g., asthma, bronchiolitis) are best treated with relatively slow rates and higher (10-12 mL/kg) tidal volume.

Reminder of steps for troubleshooting during NRP

M: Adjust Mask on face R: Reposition head to ensure open airway. Re-attempt ventilation. If not effective... S: Suction mouth and nose O: Ventilate baby's mouth slightly Open and lift jaw forward. Re-attempt ventilation if not effective P: Gradually increase Pressure every few breaths (cautiously, and to max of 40 cm H2O), until bilateral breath sounds and visible chest movement present. If still not effective... A: Consider Airway alternative (endotracheal tube or laryngeal mask airway)

Bronchodilator tx for pediatric asthma

MILD: Albuterol single neb treatment MODERATE: Albuterol/Atrovent neb (Duoneb) back to back x 3 or continuous Albuterol 10-15mg/hr x 1 hr SEVERE: Albuterol/Atrovent neb x 3 until respiratory therapist starts continuous Albuterol 20mg/hr

Use nebulizers for in asthmatics for...

MODERATE to SEVERE exacerbation and for patients requiring supplemental oxygen - Start MDI once nebulized Albuterol is weaned to q3hrs (4 puffs q3hrs)

Imaging for kid with.... characterized vasculature of developmental lung malformations

MRI

A 10 yr old boy is under medical management for adrenal insufficiency. His mother reports he has complained that he is not as good at playing sports as previously and has trouble reading. The 1st step in the care of this boy is to:

Measure very long-chain fatty acids Adrenoleukodystrophy (ALD) - adrenocortical deficiency is associated with demyelination in the central nervous system - high levels of very long chain fatty acids are found in tissues and body fluids, resulting from their impaired β-oxidation in the peroxisomes - most common form of ALD is an X-linked disorder with various presentations - most common clinical picture is of a degenerative neurologic disorder appearing in childhood or adolescence and progressing to severe dementia and deterioration of vision, hearing, speech, and gait, with death occurring within a few years - milder form of X-linked ALD is adrenomyeloneuropathy (ALM), which begins in later adolescence or early adulthood - many patients have evidence of adrenal insufficiency at the time of neurologic presentation, but Addison disease may be present without neurologic symptoms or can precede them by many years

5 day old ex FT girl, progressive lethargy, tachypnea, poor feeding. Seizure upon arrival to ED. Na 135, K 4, Cl 99, bicarb 22, anion gap 18 BUN & Cr normal. Blood glucose <20, U-ketones negative. Ammonia 600. Lactate 6.4 Most likely diagnosis?

Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) - The patient has profound hypoglycemia in the context of negative urine ketones and lactic acidosis, suggesting a defect in fatty acid oxidation. - These patients can also have significant liver dsyfunction, producing hyperammonemia. Hence, MCADD is the best choice.

4 day old ex FT boy, born NSVD, poor feeding, lethargy. Initial labs: Na 140, K 4, Cl 96. Bicarb 14 BUN 26, Cr 0.3, BG 67. Ammonia 470. Lactate 2. Most likely diagnosis?

Methylmalonic Acidemia - The patient has an elevated ammonia level with an anion gap of 30 and a normal blood lactate and glucose level, making an organic acidemia the most likely cause. Hence, Methylmalonic acidemia is the best choice.

Which autoimmune disorder is highly associated with C1q deficient individuals?

More than 90% of individuals affected by C1q deficiency develop systemic lupus erythematosus.

Postinfectious arthritis should be managed initially with

NSAIDs

Total body deficit of Na+ can be calculated by...

Na deficit (mEq) = (desired [Na+] - current [Na+] x 0.8 x body wt (kg) - for symptomatic hyponatremia (seizures or Na <120 mEq/L), calculate Na+ deficit to raise [Na+] to 125 mEq/L and give as 3% NaCl (0.5 mEq/L) over 3-6h --> correct remaining deficit over next 24h

The most common bronchial foreign body is:

Nut fragments, particularly peanuts

Predictors of persistent asthma into adolescence and adulthood.

Obesity and vitamin D deficiency

Which treatment would be recommended for a 4-year-old child with a single erythema migrans and no other symptoms?

Oral amoxicillin or cefuroxime for 14 days.

What findings are characteristic of Pseudomonas aeruginosa on preliminary culture results?

Oxidase positive, nonlactose-fermenting gram-negative rods.

Signs of a symptomatic PDA ...

Oxygen requirement Low diastolic BP Palmar pulses Bounding pulses Metabolic acidosis Pansystolic murmur Respiratory instability

Alveolar gas equation

PAO2 = FIO2 (PBAR - PH2O) - PACO2/RQ - PH2O at 37 C = 47 mmHg - PBAR at sea level ~ 745 - 765 mmHg ~ assumed at 747 mmHg - Assume PACO2 = PaCO2 - Assume RQ = 1 *Simplified = FIO2 (700) - PaCO2* Example: Infant who is breathing 30% O2 (FiO2 = 0.3), has arterial CO2 of 40 mmHg, and has perfect matching of ventilation and perfusion --> has alveolar oxygen tension of: PAO2 = 0.3(700) - 40 = 170 mmHg

powerful objective tool to identify acute hypoxemic respiratory failure when supplemental oxygen has already been administered and no room air ABG is available, or pulse oximetry readings are unreliable

PF ratio

Earliest symptom of compartment syndrome

Pain - unremitting pain, even after appropriate analgesia, is the most sensitive sign - pain with passive muscle stretch = strong indicator - symptoms occur with intercompartmental pressures 20-30 mmHg

What are the cardiac anomalies most commonly associated with congenital rubella syndrome?

Patent ductus arteriosus and peripheral pulmonary artery stenosis.

What pediatric asthmatic pts are sent home on inhaled corticosteroids?

Prescribe for all admitted patients with asthma exacerbations requiring >12 hour stay including patients with first time wheeze. - should continue ICS until their next PCP or pulm appt

Pediatric inguinal hernias commonly present as painless, reducible groin mass that is most prominent with increased intraabdominal pressure (crying). Tx?

Prompt surgical correction - to prevent incarceration and bowel ischemia

A 12 yr old Hispanic boy with a history of a seizure disorder presents to his pediatrician with a complaint of leg cramps. He is of normal stature. His physical examination is unremarkable except for a Chvostek sign. His serum calcium is 6.1 mg/dL with a serum phosphorus level of 9.4 mg/dL. Serum level of intact PTH is 312 pg/mL (normal 10-60 pg/mL). The most likely diagnosis is:

Pseudohypoparathyroidism type 1B - parathyroid glands are normal or hyperplastic and they can synthesize and secrete parathyroid hormone (PTH) - serum levels of immunoreactive PTH are elevated even when the patient is hypocalcemic and may be elevated when the patient is normocalcemi - neither endogenous nor administered PTH raises the serum levels of calcium or lowers the levels of phosphorus - genetic defects in the hormone receptor adenylate cyclase system are classified into various types depending on the phenotypic and biochemical findings

Bulk-forming agents used for constipation

Psyllium (metamucil) - with lots of water - avoid in opioid-induced constipation

Difference between flovent and Qvar regarding age:

Qvar = redihaler = usually 7 and up because it requires quick inhalation to trigger medication, then hold for 10 seconds

Children younger than 24 months with UTI are often diagnosed with anatomic abnormalities like vesicoureteral reflux and obstructive uropathy. The AAP guideline recommends what for all children in the 2- to 24-month age group with a first febrile UTI?

RUS - In cases where clinical improvement is not shown in 48 hours, RUS can be performed early to evaluate for pyonephrosis or development of a renal abscess. If the results of RUS are normal, no further testing is required. However, if the results suggest hydronephrosis, scarring, or other features of urinary obstruction, VCUG is indicated to diagnose vesicoureteral reflux. The VCUG can be performed once the infection has cleared.

Considered the reference standard for prospective analysis of a specific treatment or intervention.

Randomized controlled trials

A mother brings her 6-year-old daughter for an evaluation for concerns about "knock knees." Her parents noticed that her knees seem to bump together when she runs. The girl denies any pain. She is otherwise healthy with normal growth and development. Physical examination reveals full range of motion and normal strength throughout both lower extremities. Genu valgum is apparent on stance and gait evaluation. Supine intermalleolar measurement is 7 cm. Of the following, the BEST next management step for this girl is

Reevaluation in 6 mos - Physiologic genu valgus ("knock knee") peaks in early childhood and then regresses to adult values over the next several years. - Radiography should be performed in children older than 7 years with intermalleolar distances greater than 8 cm.

Vision screening

Referral cuttofs: - 20/40 or worse for 4/5yo - anything 20/25 or higher for 6+ years old

Most common manifestation of Turner syndrome and may be the only presenting feature.

Short stature

Which pancytopenia syndrome is also associated with failure to thrive and fat malabsorption?

Shwachman-Diamond syndrome.

Criteria for starting supplemental O2 in pediatric asthma pts

SpO2 < 90%

There are very few contraindications to breastfeeding, including:

Specific maternal infections (untreated active tuberculosis, HIV in developed countries, human T-cell lymphotropic virus type I or II, untreated brucellosis, and ebola virus) A limited number of maternal medications (illicit drugs, chemotherapy, radioactive medications) galactosemia in the infant *The National Institutes of Health maintain a free, online database of medication safety during lactation, LactMed (https://toxnet.nlm.nih.gov/newtoxnet/lactmed.htm).*

Damages but does not destroy epidermis and dermis. Characterized by intense pain, blisters, pink to cherry-red skin, moist and weepy. Nails, hair, sebaceous glands, and nerves intact. Can progress. Spontaneous re-epithelialization in 2-3 weeks.

Superficial partial thickness

One type of hemophagocytic lymphohistiocytosis is known as macrophage activation syndrome (MAS). Which rheumatologic condition is commonly associated with MAS?

Systemic juvenile idiopathic arthritis (JIA)

Jimmy is a 6yoM with new-onset diabetes admitted to PICU in DKA and is now ready to transition to SQ insulin (weight=25kg). Calculate the following - TDD - Basal - Bolus

TDD: - 0.5U/kg/day * 25kg = 12.5 U/day Basal: - 12.5 U/day * 50% = 6.25U → Round to 6 units/day → give 3U Detemir BID Bolus: - Carbohydrate Factor: 500/12.5 = 40 → Give 1 unit for every 40g of carbs - High blood glucose factor: 1800/12.5 = 144 → round to 150 → give 1 unit of insulin for every 150 over 120

Best initial test in diagnosing primary hypothyroidism.

TSH - Autoimmune thyroiditis (Hashimoto thyroiditis) is the most common cause of acquired hypothyroidism. - Clinicians should avoid routinely ordering thyroid ultrasonography in children who have simple goiters or autoimmune thyroiditis.

Newborn screening for hypothyroidism is based on ...

TSH - positive = >25 mIU/L - misses 2ndary hypothyroidism (low T4 and low TSH)

An 18 yr old adolescent presents to the emergency department with a nail puncture wound of his foot. The injury occurred 1 hr ago in a school play yard. His wound is cleansed and debrided. His last tetanus immunization was at age 12 yr, as confirmed in his medical records. Which of the following measures is recommended?

Td - A tetanus toxoid booster (preferably Td or Tdap) is administered to all persons with any wound if the tetanus immunization status is unknown or incomplete. A booster is administered to injured persons who have completed the primary immunization series if (1) the wound is clean and minor but ≥ 10 yr have passed since the last booster or (2) the wound is more serious and ≥ 5 yr have passed since the last booster.

A 1 mo old infant is breathing supplemental oxygen from a hood at a measured concentration of 45% after developing respiratory distress. A PO2 of 60 mm Hg, a PCO2 of 50 mm Hg, and a pH of 7.30 are measured in a blood sample obtained from the left radial artery. Which of the following interpretations is most consistent with these findings?

The blood gas anomalies are caused by ventilation-perfusion inequality - This infant demonstrates arterial blood gas values consistent with a venous admixture V/Q mismatch (such as in intrapulmonary airway obstruction).

In the fetal transfusion syndrome, an artery from one twin acutely or chronically delivers blood that is drained into the vein of the other. Describe the twins born...

The latter becomes plethoric and large, and the former is anemic and small

Associated with poorer long-term renal prognosis in patients with HSP.

The presence of *nephrotic syndrome*, renal insufficiency (elevated blood urea nitrogen and serum creatinine), and hypertension (nephritic syndrome)

If the history and examination findings are consistent with GERD and no concerning signs are noted, what might be beneficial?

Thickening feeds with rice cereal (in formula-fed infants) or xanthan gum/carob bean thickeners (in breastfed infants) and avoiding overfeeding - If GERD continues, a 2- to 4-week trial of hydrolysate formula or elimination of cow milk in the maternal diet is recommended - If unsuccessful, referral to a pediatric gastroenterologist and/or a 4- to 8-week trial of acid suppression may be considered

Uncuffed ETT size

Think Weight or Gestational age <1000 g, <25 wks = 2.5 1000-2000, 25-30 wks = 3.0 2000-3000, 30-35 wks = 3.5

Timely administration of analgesia to children with acute pain and anxiety is critical, and can affect their reaction to future medical care. For children with severe pain seen in the emergency department and prehospital settings optimal pain management requires rapid pain assessment and administration of a systemic opioid pain medication. What has a similar analgesic action to intravenous opioids, providing rapid pain relief without the need for intravenous access?

Transmucosal fentanyl - ex. intranasal

Commonest form of CHD causing cyanosis in newborn period

Transposition of great arteries - post-ductal sat > pre-ductal

Most common type of CoA/Interruption of Aorta

Type B - interrupted between origins of left subclavian and left common carotid arteries - frequently associated with chromosomal 22 abnormalities

Neonates and young infants with anterior mediastinal mass may be evaluated with...

US - if normal thymic tissue seen, no further imaging may be necessary

Most useful modality to assess residual fluid or fibrinous exudates of empyema

Ultrasound

For pts with atopic dermatitis, new clothing should be laundered before wearing to decrease levels of formaldehyde and other chemicals. Residual laundry detergent in clothing may trigger the itch-scratch cycle; how can this be avoided?

Using a liquid rather than powder detergent and adding a 2nd rinse cycle - facilitates removal of the detergent

Minute ventilation calculation

VE = Respiratory rate x Tidal volume (VT)

Murmur - continuous - supraclavicular/infraclavicular - 3-6 yo - grade 1-2/6 - low-pitch

Venous Hum

Most important part of NRP!

Ventilation

Most common cause of pneumonia in all age groups

Viruses - diagnosis suggested by interstitial infiltrates on CXR and WBC count <20,000 cells/mm^3 with lymphocyte predominance

For a child who has had a syncopal event, physical examination with a full cardiac assessment is recommended, including:

Vital signs Peripheral pulses - symmetry, amplitude, and timing Cardiac auscultation when supine and standing - with or without the Valsalva maneuver

Better reflect "ideal growth" of breastfed infants and are superior to US Center of Disease Control and Prevention growth charts.

WHO growth charts - should be used to assess growth of children younger than 2 yo

Most common scoring system for croup

Westley Croup Score: Level of consciousness - normal/sleep = 0 - disoriented = 5 Cyanosis - none = 0 - with agitation = 4 - at rest = 5 Stridor - none = 0 - with agitation = 1 - at rest = 2 Air entry - normal = 0 - decreased = 1 - markedly decreased = 2 Retractions - none = 0 - mild = 1 - moderate = 2 - severe = 3 mild = </= 2 (no stridor at rest) moderate = 3-7 severe = >/= 8 (sig stridor at rest, severe retraction)

A 3-year old boy presents with multiple-system trauma. The child was an unrestrained passenger in a high-speed motor vehicle crash. On primary assessment, he is unresponsive to voice or painful stimulation. His respiratory rate is 5/min, HR and pulses are 170/min, systolic BP is 60 mmHg, capillary refill is 5 seconds, and SPO2 is 75% on RA. Which action should you take first?

While colleague provides spinal motion restriction, open the airway with jaw thrust and provide bag-mask ventilation

An 8-year-old boy is brought to your office for an initial visit after moving to the area. He was born by normal vaginal delivery at full term following an uncomplicated pregnancy and was discharged home with his parents after 2 days. He had normal growth and development without any serious illnesses for the first 6 months after birth. Around 6 months of age, he began to experience recurrent episodes of otitis media that have persisted. Despite bilateral tympanostomy tube placement last year, he has continued to experience ear infections. At 18 months of age, he was hospitalized for 10 days for severe pneumonia. He has been treated with antibiotics for 2 other episodes of pneumonia since that time. He has also experienced frequent paronychial infections. His immunizations are up-to-date. He is not currently taking any medications, and he has no known allergies to medications. He has 2 older sisters who are healthy and have had normal growth and development. Both of his parents are healthy and this is their first marriage. A male first cousin born to a maternal aunt died at 2 years of age from an infection. The boy's temperature is 38.4oC, heart rate is 102 beats/min, and blood pressure is 96/68 mm Hg. He is at the 5th percentile for weight and the 10th percentile for height. He is thin, but otherwise appears well. His right tympanostomy tube is draining a purulent fluid. His tonsils are very small, and you confirm that he did not undergo a tonsillectomy. There is erythema, swelling, and a purulent discharge at the medial margin of the nail of his right great toe. The remainder of his examination results are unremarkable. An initial laboratory evaluation reveals: Laboratory Test Result - White blood cell count 3,100/µL (3.1 x 109/L) - Hemoglobin 11.9 g/dL (119 g/L) - Platelet count 427 x 103/µL(427 x 109/L) - Neutrophils 60% - Lymphocytes 37% - Monocytes 3% Of the following, this child's presentation is MOST consistent with

a humoral primary immunodeficiency - history of multiple invasive bacterial infections that began at 6 months of age. - innate immune system is comprised of nonspecific barrier defense (skin, hair, mucosal barrier) and cellular defense (neutrophils, macrophages, natural killer cells). - The adaptive immune system is comprised of specific humoral immunity (B cells) and cellular immunity (T cells). - Agammaglobulinemia is an X-linked genetic defect of the adaptive humoral immune system that presents with invasive bacterial infections in the first year after birth. Patients with agammaglobulinemia require treatment with replacement immunoglobulin subcutaneously or intravenously every 3 to 4 weeks for life, unless they undergo a hematopoietic stem cell transplant.

The stage of hypertension determines the urgency of further evaluation and management. Children with elevated BP are recommended to have

a BP check at a second visit in 6 months - If the BP is again high, a third measurement is done in 6 months and diagnostic evaluation is initiated

ASDs are typically discovered when ....

a murmur is heart at a regular 4- to 6- mo infant well-child visit - loudest over pulmonic region - associated with fixed splitting of S2 during different phases of respiration and a loud S1 - murmur heard = increased blood flow across the pulmonary valve bc of greater volume of blood in right side of heart (relative pulmonic stenosis)

A 16-year-old adolescent girl is seen in the clinic for a pregnancy test. The limits of confidentiality are discussed. She reports that she recently met her older boyfriend on a social media site. She sneaks out of her house to see him every night; her family does not know about him. He frequently takes her to parties at which she participates in sexual activities with his friends when he asks her to. He rewards her with clothes and jewelry. Her last sexual encounter with his friend was 1 week ago and was unprotected. Physical examination findings are unremarkable. The result of her pregnancy test is negative. She reports no abdominal or genitourinary symptoms. Results of testing for sexually transmitted infections are pending. Concerns for her safety are discussed. The patient requests that her parents not be told. Of the following, the BEST next step in treatment is to contact

a national trafficking organization hotline - Children and adolescents who have experienced sexual exploitation, including sex trafficking, human trafficking, and commercial sexual exploitation, seek medical attention for a variety of reasons but seldom self-identify. - When responding to cases of suspected commercial sexual exploitation, a national trafficking organization (such as the National Human Trafficking Resource Center Hotline at 1-888-373-7888, sponsored by the Polaris Project) should be contacted for assistance. - Providers should also provide anticipatory guidance to pediatric patients about protecting themselves from online exploitation.

A shift of potassium from the intracellular space can maintain a normal or even an elevated serum potassium concentration, despite massive losses of potassium from the intracellular space. This is dramatically seen in diabetic ketoacidosis, in which a state of significant potassium depletion is often masked because of...

a transmembrane shift of potassium from the ICF to the ECF. - For potassium and phosphorus, electrolytes with a high intracellular concentration, the serum level may not reflect total body content. - Similarly, the serum calcium concentration does not predict the body content of calcium, which is largely in bone.

First line study in context of acute abdomen when intestinal obstruction or perforation is suspected

abdominal films

Any constant strabismus is considered...

abnormal

A 6 mo old boy is brought to the emergency department and is afebrile but responds poorly to tactile and auditory stimuli. He becomes apneic and unresponsive after a generalized seizure. The parents state that he was perfectly well in the car on the way to the hospital and that they only brought him to the emergency department because of constipation. He requires 10 min of cardiopulmonary resuscitation (CPR), after which he is noticed to have a bulging fontanel and bilateral retinal hemorrhages. A chest radiograph reveals two posterior rib fractures. The most likely diagnosis is:

abusive head trauma - results in the most significant morbidity and mortality. - poor neck muscle tone and relatively large heads of infants make them vulnerable to acceleration-deceleration forces associated with shaking - signs and symptoms may be nonspecific, ranging from lethargy, vomiting, changing neurologic status or seizures, and coma. - retinal hemorrhages are an important marker of AHT. - whenever AHT is being considered, a dilated indirect ophthalmologic examination by a pediatric ophthalmologist should be performed - hemorrhages that are multiple, involve more than one layer of the retina, and extend to the periphery are very suspicious for abuse - mechanism is likely repeated acceleration-deceleration due to shaking

In 2012, the World Health Organization published the Guidelines on the Pharmacologic Treatment of Persisting Pain in Children with Medical Illnesses, which outlines a "2 step approach":

acetaminophen and nonsteroidal anti-inflammatory medications for mild pain, with the addition of an opiate for moderate to severe pain

When should Tdap be given to preteens and adolescents?

age 11-12 Td booster (no pertussis coverage) should be given every 10 years - Give Tdap as booster if pt did not previous receive Tdap - pregnant women should recieve booster during every 3rd trimester of pregnancy

Head-banging is a repetitive motor behavior (stereotypy) that usually resolves by

age 4 years in typically developing children - Persistence after age 5 years is associated with developmental disorders such as autism or intellectual disability. - In the context of a child with significantly impaired communication skills, self-injurious behaviors such as head-banging should prompt evaluation for painful medical conditions such as otitis media, sinusitis, or constipation, particularly when the behavior is acute.

A previously healthy 6 mo old child develops paroxysmal colicky abdominal pain. The infant has occasional vomiting. Over the next 12 hr the infant passes stool containing blood and mucus and becomes progressively lethargic. After fluid resuscitation, the most appropriate next step in management is:

air-contrast enema - Air, hydrostatic (saline), and, less often, water-soluble contrast enemas have replaced barium examinations. - Contrast enemas demonstrate a filling defect or cupping in the head of the contrast media where its advance is obstructed by the intussusceptum. - A central linear column of contrast media may be visible in the compressed lumen of the intussusceptum, and a thin rim of contrast may be seen trapped around the invaginating intestine in the folds of mucosa within the intussuscipiens ("coiled spring" sign), especially after evacuation. - Retrogression of the intussusceptum under pressure and visualized on radiography or ultrasound documents successful reduction. - Air reduction is associated with fewer complications and lower radiation exposure than traditional contrast hydrostatic techniques.

A 4 wk old female infant is presented to the emergency department with hyponatremia and hyperkalemia. Other than dehydration, the physical findings are normal. The most informative laboratory examination would be:

aldosterone Adrenal insufficiency - cortisol levels are sometimes at the low end of the normal range but are invariably low when the patient's degree of illness is considered - ACTH levels are high in primary adrenal insufficiency but can take time to be reported by the laboratory - Similarly, aldosterone levels may be within the normal range but inappropriately low considering the patient's hyponatremia, hyperkalemia, and hypovolemia - Plasma renin activity is elevated. Blood eosinophils may be increased in number, but this is rarely useful diagnostically

Blood pressure checks are recommended at all health care visits for children at what age?

all children 3 years or older - An increased risk of hypertension occurs in children who were small for gestational age, had very low birth weight, or were born at less than 32 weeks of gestation, as well as children with a history of umbilical artery catheterization. In these children, BP should be measured even when they are younger than 3 years.

In situations in which brain death is not achieved, patients may be eligible for organ donation via

alternative pathway known as donation after cardiac death - This may be an option for patients who have a severe neurological injury and irreversible brain damage but still have minimal brain function and do not meet brain death criteria - It is important to note that donation after cardiac death is an option only for patients whose families have opted for withdrawal of life support - In cases of donation after cardiac death, all life support is withdrawn in a controlled setting and the patient must then progress to cardiopulmonary arrest; death is determined by lack of cardiopulmonary function for at least 5 min - Once death is pronounced by a physician who is not a part of the transplant team, the organs for transplantation are surgically removed by the transplant team - In the event that the patient does not die after a period, donation is no longer permitted.

Delayed removal of a congenital and complete unilateral cataract may lead to:

amblyopia - Persistent amblyopia is the most common cause of poor visual recovery after cataract surgery in children

The most common cause of viral meningoencephalitis is:

an enterovirus - more than 80 serotypes of these small RNA viruses have been identified - severity of infection caused by enteroviruses ranges from mild, self-limited illness with primarily meningeal involvement to severe encephalitis resulting in death or significant sequelae

What head circumference is considered macrocephaly?...

an occipitofrontal circumference or head circumference, OFC, of > 2 standard deviations above the mean or above the 97th percentage for a given age, and gender, or when serial measurement shows progressive enlargement, crossing of one or more major percentiles, or when there is an increase in OFC > 2 cm/month in the first 6 months of life. - The OFC should be measured using a non-elastic tape which surrounds the head along the line of the glabella and posterior occipital protrusion.

Tools for ear block for embedded earring removal, hematoma drainage, or abscess drainage

antiseptic solution 10 mL syringe for lidocaine 1.5 inch 25 or 27 gauge needle 1% lidocaine without epinephrine

"dead space" refers to ...

any area in which the alveoli and capillaries do not line up This dead space can be: Anatomical dead space - due to an obstruction of either the airways or the blood supply, such as with pulmonary emboli (obstructing a blood vessel) or a foreign body inhaled into an airway (obstructing an airway) Physiological dead space - occurs when the structures of the airways or blood vessels are normal, but there are other reasons why the alveoli and capillaries don't match up such as a shunting of blood away from one area of the lungs (physiological obstruction) With dead space, you are essentially bringing blood to an area in which it is unable to pick up oxygen, or bringing air into a region that is unable to extract oxygen

Murmur - systolic - supraclavicular/carotids - any age - grade 2-3/6 - harsh, medium to high-pitched

arterial supraclavicular murmur

The most important manifestation of hydrocarbon toxicity is

aspiration pneumonitis - The propensity of a hydrocarbon to cause aspiration pneumonitis is inversely proportional to its viscosity. - Compounds with low viscosity, such as mineral spirits, naphtha, kerosene, gasoline, and lamp oil, spread rapidly across surfaces and cover large areas of the lungs when aspirated. - Only small quantities (<1 mL) of low-viscosity hydrocarbons need be aspirated to produce significant injury. - If hydrocarbon-induced pneumonitis develops, respiratory treatment is supportive.

The most common cause of pneumomediastinum in pediatric patients is:

asthma Pneumomediastinum - usually caused by alveolar rupture during acute or chronic pulmonary disease - acute asthma is the most common cause of pneumomediastinum in older children and teenagers

Moderate to severe encephalopathy in newborn based on presence of...

at least 3 of 6 of Sarnat criteria present between 1-6 hours after birth - reduced consciousness - decreased spontaneous activity - posture - tone - primitive reflexes (such and Moro) - autonomic abnormality (pupils and respiration)

Medications for irritability and self-injurious behaviors/aggressiveness associated with autism

atypical antipsychotics - risperidone - aripiprazole - high risk for side-effects - increases growth curve, hyperlipidemia, insulin resistance

hypoxic-ischemic encephalopathy occurs when...

baby experiences reduced blood flow and oxygen delivery to the brain near the time of birth - standard treatment = hypothermia or cooling therapy - cooling baby's body temp for 3 days reduces risk of brain injury after HIE

Common causes of bloody stool in infancy include

bacterial enteritis milk protein allergy intussusception swallowed maternal blood anal fissure lymphonodular hyperplasia

alkali substances/ingestions

bleach dishwashing detergent drain cleaner - dangerous - pH >11 - liquefaction necrosis --> ulceration and perforation - NO ipecac or gastric lavage = reexposure of esophagus to chemical - poison control = 1-800-222-1222

When to get carboxyhemoglobin level in ingestion of toxins...

c/f carbon monoxide poisoning

Young children with isolated Horner syndrome should undergo careful examination for

cervical and abdominal masses measurement of urinary HVA and VMA levels radiologic imaging of the head, neck, and chest

Majority of ingested foreign bodies pass through GI tract spontaneously without consequence. Those that fail to pass frequently impact in ...

cervical esophagus, between cricopharyngeus muscle and level of thoracic inlet

A 2 mo old infant has difficulty with feeding. She tires quickly and sweats with feeds. Hepatomegaly is appreciated on examination, and there is a harsh systolic murmur. Pulse oximetry is 97%. Initial evaluation should include:

chest radiograph and EKG - Chest radiograph can show evidence of increased, normal, or decreased pulmonary vascular markings. - - Electrocardiogram can be used to determine whether right, left, or biventricular hypertrophy exists.

Individuals with choanal atresia may have other anomalies, such as

craniosynostosis

Potential symptoms of Congenital Heart Disease

cyanosis CHF poor pedal pulses failed newborn CHD pulse oximetry screen prior to discharge

What is the effect of positive pressure ventilation on LV transmural pressure?

decreases - PPV decreases the transmural pressure (pressure in the ventricle - pressure out of the ventricle) where pressure out of the ventricle is the intrathoracic pressure. Hence, by increasing intrathoracic pressure with PPV, you reduce transmural pressure, which is essentially decreasing LV afterload.

High serum chloride levels and metabolic acidosis are seen in

diarrhea and renal tubular acidosis

Indications for bolus feeds

dysphagia anorexia supplement to oral intake *Disadvantages* = increased aspiration risk, gastric feedings only, poorly tolerated with reflux and delayed gastric emptying *Goals* - infants = 8-12 feedings/day - older infants = 4-6 feedings/day - children/adolescents = 3-6 feedings/day

Of the following, the best method for diagnosis of myasthenia gravis is:

electromyogram - Myasthenia gravis is one of the few neuromuscular diseases in which electromyography (EMG) is more specifically diagnostic than a muscle biopsy. - A decremental response is seen to repetitive nerve stimulation; the muscle potentials diminish rapidly in amplitude until the muscle becomes refractory to further stimulation. - Motor nerve conduction velocity remains normal. - This unique EMG pattern is the electrophysiologic correlate of the fatigable weakness observed clinically and is reversed after a cholinesterase inhibitor is administered.

Type of formula to use if milk-protein allergy

elemental - ex. elecare

For first-aid management of anaphylaxis in health care settings, traditionally what dose of epinephrine is injected?

epi dose of 0.01 mg/kg injected IM - max of 0.3 mg in prepubertal child - up to 0.5 mg in teenager - Epinephrine autoinjectors (EAs) can be used in health care settings to delivery 0.15 mg dose in young child and 0.3 mg dose in child or teenager - If response to first epi injection is inadequate, it can be repeated once or twice at 5-15 minute intervals

Imaging for kid with.... suspected foreign body

expiratory radiograph

Protein-losing enteropathy should be suspected in children with hypoalbuminemia and edema. What is recommended to diagnose protein-losing enteropathy?

fecal α1-antitrypsin level - Elevated fecal α1-antitrypsin levels should prompt further testing for a cause of protein-losing enteropathy, based on the clinical presentation

Similarly, in perinatal asphyxia, what are criteria for use of cooling therapy in term newborn infants?

fetal acidosis Apgar score of 0-3 after 5 min neurologic dysfunction and/or abnormal EEG findings

Precordial catch syndrome localized to...

fingertip to one interspace at left sternal border or cardiac apex - increases with inspiration

Water treatment with what has been associated with a decreased incidence of poststreptococcal glomerulonephritis?

fluoride

hamburger sign

for appendicitis - if kid won't eat favorite food, more likely to have appendicitis

It is recommended that exclusively breastfed full-term infants start a daily iron supplement beginning when?

fourth month of life. - Once a child has initiated iron therapy, a complete blood count should be rechecked in four weeks to determine if the infant's anemia has improved. - The hemoglobin is expected to rise at least 1 g/dL within that time period. - The iron therapy is continued until several months after the hemoglobin reaches a normal level.

Irritant diaper dermatitis presents with erythema that spares the skinfolds. Management includes...

frequent diaper changes avoidance of excessively tight-fitting diapers exposure of skin to air application of barrier creams (*zinc oxide*, petrolatum)

On CT, pulmonary edema is most commonly associated with ...

ground glass opacities = increased density of pulm parenchyma without obscuration of vascular borders thickening of interlobular septa

Neonate with mild cyanosis at birth followed by rapid clinical deterioration on day of life 1 most likely has...

hypoplastic left heart syndrome - ductal-dependent - right ventricle responsible for supplying both pulm and systemic blood flow - mixing of oxygenated and deoxygenated blood in right atrium accounts for mild cyanosis immediately after birth

Will present during adolescence in the form of primary amenorrhea, cyclical abdominal or pelvic pain, a lower abdominal mass, or a blue discoloration of the hymen because of hematocolpos. It can also present with back pain, or difficulty with urination and defecation because of vaginal distention.

imperforate hymen

Because of the increased risk of bilirubin encephalopathy, therapy should be started at lower bilirubin concentrations. In general bilirubin should not be allowed to exceed....

infant's weight in kg x 10 - ex. 1 kg infant --> keep bilirubin <10 mg/dL

Clinicians should offer melatonin to children and adolescents with ASD if behavioral strategies have not been helpful and contributing coexisting conditions and use of concomitant medications have been addressed. Initiate dose at:

low dose 1 to 3 mg taken 30-60 min before bedtime and titrate to effect - not to exceed 10mg per night

Clinical factors associated with increased risk of IVH include

lower gestational age pneumothorax male sex bolus administration of normal saline or sodium bicarbonate

What malignancy are individuals with hyperimmunoglobulin E syndrome at risk of developing?

lymphoma

T2D diabetes management approach if: no symptoms, blood glucose <250, no ketones =

metformin +/- insulin + lifestyle changes

ET may be passed nasally or orally. The tip should be in ...

mid trachea at appx C7-T2, above the carina - tip of ET tube moves cranially with neck extension and caudally with flexion (hose goes with the nose)

During which part of adolescence are mixed-sex peer groups predominant?

middle adolescence

LTRA that can be used instead of ICS (Qvar vs Flovent/depending on insurance) if there is difficulty/preferred by parent for ease of therapy for asthma management

montelukast/singulair - leukotriene receptor antagonist - also helps reduce allergy symptoms - behavioral/mental health side effects (not black box warning though)

Sinus venosus ASDs are due to defects...

neart the SVC and right upper pulm vein, truly a form of partial anomalous pulmonary venous drainage

What is the difference between lotion and cream emollients?

oil content

The classic clinical triad of central nervous system depression, respiratory depression, and miosis should prompt clinicians to suspect

opioid poisoning, even in the absence of a history of opioid exposure.

A 14 yr old boy presents with a bony, nonpainful mass in the distal femur that has been slowly enlarging. There are no associated symptoms. The mass is most likely to represent which of the following tumors?

osteochondroma (exostosis) - one of the most common benign bone tumors in children - many are completely asymptomatic and unrecognized - most osteochondromas develop in childhood, arising from the metaphysis of a long bone, particularly the distal femur, proximal humerus, and proximal tibia - enlarges with the child until skeletal maturity - most are discovered at 5-15 yr of age, when the child or parent notices a bony, nonpainful mass - some are discovered because they are irritated by pressure during athletic or other activities

IV fluids in setting of moderate dehydration is also know as

parenteral nutrition

Use SIMV when ...

patient likely to breathe spontaneously

Determine SBP at end of exhalation and then during inhalation; if difference is >10 mmHg/pulsus paradoxus, consider...

pericardial effusion tamponade pericarditis severe asthma restrictive cardiomyopathies

The International Classification for Headache defines migraine with aura as an episodic, moderately severe, pulsatile, bilateral, frontoparietal headache with 2 of 4 associated symptoms:

photophobia phonophobia nausea vomiting - For diagnosis of episodic migraine there must be at least 4 lifetime attacks lasting 2 to 72 hours, not better explained by another condition. - An aura can include visual, sensory, motor, language, or brainstem symptoms which are reversible and often accompanied by a headache. - Concerning features, such as age less than 6 years, waking at night with headache, initiation of headache with valsalva maneuver, or associated focal neurologic deficit, would indicate a need for additional investigation. - The complete International Classification of Headache Disorders criteria can be found at: https://ichd-3.org.

Esophageal atresia may often be suspected prior to first feeding by hx of...

polyhydramnios or copious oral secretions that required frequent suctioning

Causes of painless rectal bleeding include

polyps Meckel diverticulum vascular malformations

MMR-V Vaccine is aka

proquad

Illusion of strabismus secondary to prominent epicanthalfolds or other facial features. Normal corneal light reflex diagnostic. Illusion resolves with growth of nose and face.

pseudostrabismus

In subgaleal hemorrhage in infant, earliest sign of expanding hemorrhage is ...

pushing of ears laterally and forward - treatment = difficult = may require vigorous therapy

A 7-year-old boy is having a follow-up visit for attention-deficit/hyperactivity disorder (ADHD). His parents ask about a newly publicized device for the treatment of hyperactive and impulsive symptoms in children with ADHD. They are excited about the possibility of an effective nonpharmacologic treatment and would like to know about the benefits and harms of this intervention. Of the following, the study design MOST likely to provide the desired information is a

randomized controlled trial - study design most likely to yield valid information about the benefits and/or harms of an intervention

most common vision disorders in children

refractory errors - occur when light is not focused on the retina, causing blurred vision

How long can breastmilk remain in refrigerator and freezer?

refrigerator = 4 days freezer = 6 mos - CDC states can remain in freezer for up to 12 mos

Commonest cause of neonatal hypertension in ICU is...

reno-vascular HT d/t thrombi from "high" umbilical arterial catheter (UAC) - maintain UAC so tip is below 3rd lumbar vertebrae (L3)

The most common cardiac tumor in pediatrics is:

rhabdomyoma - vast majority of tumors originating from the heart are benign - most common pediatric cardiac tumors and are associated with tuberous sclerosis in 70-95% of cases

Which lung lobe contains three lobes?

right

24 months old - gross motor dev't...

runs jumps in place walks up and down stairs holding on throws ball overhand kicks ball

Acute renal failure is defined as....

serum Cr >1.5 mg/dL - oliguric ARF = urine output <1 ml/kg/hr - non-oliguric ARF (urine output maintained despite decreased glomerular and tubular function)

Neonatal brachial plexus injuries (Erb-Duchenne palsy) most commonly associated with ...

shoulder dystocia in *macrosomic infants* - most infants recover spontaneously with supportive care

The prevalence of type 2 diabetes mellitus in children and adolescents has increased worldwide over the past three decades. Nutrition and exercise counseling should be started at the time of diagnosis and as a part of ongoing management. Metformin is the first-line therapy in conjunction with lifestyle changes. Insulin therapy should be initiated if there are ...

signs of ketosis or ketoacidosis or if the patient has significant hyperglycemia (A1C greater than 9% or a random plasma glucose level of 250 mg per dL or greater)

Consider what as an alternative to a 5-day course of prednisone/prednisolone in pediatric asthmatics?

single or 2-dose regimens of dexamethasone

A 6-year-old boy presents to the emergency department with new onset of tea-colored urine. His mother states that he was recently treated with a shot for a throat infection. Examination is significant for mildly elevated blood pressure and trace pedal edema. Urinalysis reveals red-brown urine and too many red blood cells to count. Serum creatinine is 0.65 mg/dL and blood urea nitrogen 16 mg/dL. What is the next best step in management?

sodium and water restriction - child's presentation of hematuria, pedal edema, and blood pressure elevation follows a recent episode of likely streptococcal pharyngitis = poststreptococcal glomerulonephritis, - results from deposition of immune complexes within the glomeruli after a cutaneous or pharyngeal streptococcal infection. - decreased levels of complement C3 and CH50 further support the diagnosis. - treatment of poststreptococcal glomerulonephritis is primarily supportive, sometimes including sodium and water restriction to decrease fluid overload and hypertension. Diuretics may be useful for volume overload that has not adequately responded to sodium and water restriction.

In establishing buprenorphine stabilization and maintenance the patient should be on what if concurrently treating pain?

split dose

When to transition from bottle to cup

start early! around 6 months - start with their formula and not just water - goal = to stop bottle at 1 year

diabetic drug under "1 pill can kill" list

sulfonylureas - ex. kid with seizures + low glucose even after boluses - give dextrose first = readily available - activated charcoal --> ~hour or loss + no lavaging --> symptomatic treatment

ipecac syrup

syrup that induces vomiting - old-school - not used as much anymore

Which is the most sensitive index of disease severity in patients with pneumonia?

tachypnea - Tachypnea is the most sensitive index of disease severity in pneumonia

Features of CHF in newborn

tachypnea increased work of breathing tachycardia hepatomegaly - hepatomegaly suggests cardiac inv't - normal newborn liver span = 4.5 - 5 cm

Why do we use cylosporins

tacrolimus induced hyperglycemia

A 17-year-old boy presents to clinic with penile discharge. He admits to recent sexual activity without protection. Diagnosis of infection with Neisseria gonorrhoeae is made by urine nucleic acid amplification test. In addition to notifying the patient, the physician is also mandated to report this patient's test results to which of the following entities?

the state public health department - Gonorrhea is a reportable disease in all 50 states; thus, this physician is required to report the boy's infection to the state public health department. State laws vary regarding other reasons for breach of confidentiality.

Majority of anterior mediastinal masses originate from...

the thymus - 85% are due to thymic hyperplasia, lymphoma, or germ cell tumors

Adults meet criteria for metabolic syndrome when

three of the following are present: - High waist circumference - High triglyceride level (≥ 150 mg/dL [1.7 mmol/L]) - Low high-density lipoprotein (HDL) cholesterol (< 40 mg/dL [1 mmol/L] in males, < 50 mg/dL [1.3 mmol/L] in females) - High blood pressure (≥ 130 mm Hg systolic and/or ≥ 85 mm Hg diastolic) - High fasting glucose level (≥ 100 mg/dL [5.6 mmol/L])

Imaging for kid with.... pleural effusion

ultrasound

Initial step in management of suspected septic arthritis of the hip.

ultrasound

Imaging for kid with.... complicated pneumonia

ultrasound (pleural effusion) CT scan

The absence of 2 normal-sized ventricles, absence of normal or repairable atrioventricle connection (in form of severe stenosis or atresia of mitral or tricuspid valves), absence of repairable outflow tract in some situations (severe stenosis or atresia of pulmonary or aortic valve), or any combo of these conditions indicates that pts should ....

undergo single-ventricle palliation - these lesions are dependent upon a PDA maintained by prostaglandin to ensure cardiac output into both systemic and pulm circulations

Hematuria frequently occurs with extremely strenuous exercise, and can occur in both contact and non-contact sports. Diagnosis should be considered one of exclusion, even with appropriate history, and follow up includes...

urinalysis in 1 wk - to ensure hematuria has resolved

The best screening test for Lesch-Nyhan syndrome is

urinary urate-to-creatinine ratio - will be greater than 2.0 in a child who is younger than 10 years of age

24 month old - fine motor dev't...

uses spoon and fork opens door stacks block draws vertical line, beginning circles

NDRI that can be used for ADHD

wellbutrin/bupropion - NE and dopamine reuptake inhibitor - inattention, hyperactivity, anxiety and depression

arc exposure

when body is part of electrical circuit - deep tissue thermal injury and internal organ injury

flash contact

when current hits skin but does not arc through the body (surface burns with soot)

Definition of whipple's triad-related hypoglycemia...

whipple's triad/may indicate insulinoma - signs/symptoms of hypoglycemia + - BG <50 mg/dl + - relief of symptoms with treatment to raise BG with normal range ** we usually consider hypoglycemia <70 mg/dl d/t symptoms and concern for neurodev'tal outcomes

How long after unprotected sexual intercourse can levonorgestrel be given for emergency contraception?

within 72 hours

A 2-year-old previously healthy girl presents with low-grade fever and refusal to walk for the past several days. She is complaining of pain around her left hip. She has mild upper respiratory symptoms as well. She has no vomiting, diarrhea, or significant rash. On exam, she is mildly ill appearing. Heart and lungs are normal. There is mild to moderate pain with palpation of the left femur. There is no swelling or redness. Laboratory values include a normal complete blood count, C-reactive protein of 1.8 mg/L and sedimentation rate of 23 mm/hr. Magnetic resonance imaging (MRI) of the hip reveals findings consistent with osteomyelitis of the femur. Which of the following is the most likely organism responsible?

*kingella kingae* - osteomyelitis, though her presentation and laboratory values are relatively mild = osteomyelitis due to Kingella kingae, a gram-negative bacterium which has become more recognized as a cause of musculoskeletal infections in children, especially those in daycare. - clinical course of osteomyelitis caused by Kingella may be less acute than that caused by Staphylococcus aureus. - very difficult to culture using standard techniques. - Treatment generally involves a cephalosporin such as cefazolin or ceftriaxone, though ampicillin-sulbactam may also be used.

A 7-year-old girl was diagnosed with acute B lymphoblastic leukemia 2.5 months ago. Her mother reports that her daughter had been quite ill during the first month of therapy (induction) and required multiple blood transfusions, as well as a 4-week course of parenteral antibiotics that finished 6 weeks ago. The girl's last transfusion of packed red blood cells was 3 weeks ago. She was in a complete remission at the end of induction therapy and is now in consolidation therapy. Five days ago, she started having loose stools that have progressed to watery, voluminous, non-bloody diarrhea occurring 5 to 6 times per day. Three days ago, she developed a rash that started on her upper chest and has worsened daily. She has a temperature of 37°C, a heart rate of 100 beats/ min, and blood pressure of 85/50 mm Hg. Her examination findings are remarkable for: scleral jaundice; an erythematous, papular, mildly pruritic rash on her trunk; a lacy, pink, macular rash extending down her arms and legs; and erythematous palms and soles. Her liver edge is palpable 3 cm below the costal margin. Her laboratory evaluation shows: Laboratory test Result White blood cell count 1,100/μL (1.1 x 109/L) Hemoglobin 9.1 g/dL (91 g/L) Platelet Count 89 x 103/μL (89 x 109/L) Neutrophils 10% Lymphocytes 85% Monocytes 5% Aspartate aminotransferase 675 U/L (normal, 0-31 U/L) Alanine aminotransferase 782 U/L (normal, 0-31 U/L) Bilirubin (total) 4.3 mg/dL (73.5 μmol/L), [normal, 0.2-1.2 mg/dL (3.4-20.5 μmol/L)] Of the following, the statement MOST consistent with this girl's presentation is that she:

*received a nonirradiated blood product* - The girl in this vignette is in the early months of treatment for acute lymphoblastic leukemia and can be expected to be immunocompromised because of chemotherapy. She has typical signs and symptoms of acute GVHD, which most commonly affects the skin (rash), liver (elevated liver function test results and bilirubin levels), and intestines (diarrhea). - Her history of being immunocompromised and receiving multiple transfusions, most recently 3 weeks prior to presentation, greatly raises the concern for transfusion-associated GVHD. Not all blood banks routinely irradiate their blood products prior to release for patient use; therefore, it is critical to order irradiated blood products for all immunocompromised individuals, such as newborns and patients with cancer who are receiving chemotherapy. - Leukodepletion of blood products removes neutrophils by filtration, a process that separates particles based on size. - Irradiation of blood products damages the DNA of donor lymphocytes, thereby rendering them replication incompetent and definitively preventing transfusion-associated graft-vs host disease. - Acute graft-vs-host disease presents with a rash, elevated liver function test results, elevated serum bilirubin levels, and profuse watery diarrhea.

Infants will gradually take in more milk with each feed. How much by: - 4-6 weeks - 2 mos

- 4 ounces by 4-6 weeks - 5-6 ounces at 2 mos

Spirometry for Restrictive - FVC - FRC - TLC - FEV1 - FEV1/FVC - FEF25-75

- reduced - normal or reduced - reduced - normal or decreased - normal or decreased - normal or decreased

Prehn's sign

- seen in epididymitis or inflammation from infection - GC = common causes - relief when lift inflamed teste

After the first day of life, urine output should be

>1 mL/kg/h

Placed into cecum or ascending colon for colonic cleaning and fecal evacuation in those children with problematic incontinence and soiling, such as myelodysplasia. Tubes may have appearance of pigtail or low profile "trap door" style or other button devices.

Cecostomy tubes - C tube check performed with water soluble contract to evaluate for position (such as malposition into small bowel, sigmoid) and with US or CT to look for other probz that may be associated (such as puncture of other viscus, inflammatory collection)

A 5 yr old boy presents with his third episode of painful cervical lymphadenitis. Each was treated with incision and drainage, and cultures grew Staphylococcus aureus. At the age of 2 yr, he required surgical aspiration of a liver abscess. The most important laboratory test is:

Fluorescence assay using dihydrorhodamine-123 Chronic granulomatous disease. - Any patient with recurrent pneumonia, lymphadenitis, hepatic or other abscesses, osteomyelitis at multiple sites, a family history of recurrent infections, or any infection with an unusual catalase-positive organism requires evaluation. - The onset of clinical signs and symptoms may occur from early infancy to young adulthood. - The most common pathogen is S. aureus, although any catalase-positive microorganism may be involved. - Other organisms frequently causing infections include Serratia marcescens, Burkholderia cepacia, Aspergillus, Candida albicans, Nocardia, and Salmonella. - The diagnosis is most often made by performing flow cytometry using dihydrorhodamine-123 (DHR) to measure oxidant production through its increased fluorescence when oxidized by H2O2.

The neck rule

The "neck rule" is commonly invoked in these decisions, where patients with conditions above the neck (eg, otitis media, sinusitis, nonspecific upper respiratory tract infections [URIs]) may participate in sport as tolerated. - Athletes with conditions that are below the neck (eg, pneumonia, vomiting) or systemic (eg, myalgia, malaise, fever) should be withheld from sport until the symptoms and underlying etiology have resolved. - Children and adolescents should not participate in vigorous physical activity while febrile or with evidence of acute systemic illness.

A 15 yr old previously healthy but obese girl presents in HHS/DKA overlap which has resolved on insulin infusion at 0.1 units/kg/hr. She is very hungry and wants to eat her breakfast now. The PICU is full and needs to get people out to the floor. She is post-menarche, has acanthosis nigricans, and weighs 90kg. Endocrinology expects a reasonable plan before you call for advice. What do you do?

Total Daily Dose (TDD) = 0.7 units x 90kg = 63 units 50% basal = 31.5 units glargine or detemir (daily or div BID) - TDD starting pt for older child/teen = 0.7 units/kg/day Insulin-to-Carbohydrate Ratio (ICR) = 500/63 = 7.9g - ICR = 500/TDD Insulin Sensitivity Factor (ISF) = 1800/63 = 28.6 mg/dL - ISF = 1800/TDD A reasonable starting plan: detemir 16 units BID aspart ICR 1:8g, ISF 30 mg/dL, BG target 125 mg/dL BG insulin dose = 1 unit:30 mg/dL > 125 mg/dL

Tx for ophthalmia neonatorum via herpes simplex virus...

acyclovir 60 mg/kg per day in 3 divided doses x2 weeks (3 weeks if CNS or disseminated disease) + topical gtts (1% trifluridine, 0.1% iododeoxyuridine, or 3% vidarabine) - gancyclovir gel

Irregularity in the shape of the cornea or lens that causes blurry vision at all distances if not corrected. Children who have myopia or hyperopia are more likely to have this.

astigmatism

ptosis + miosis -->

asymmetrically constricted pupil - may represent Horney syndrome - may need referral/referral for neuroblastoma

Hepatitis A vaccine is typically given when?

at 1 year of age, with a second dose 6 months after

Children with KD are at risk for coronary artery aneurysms. Echocardiography is the imaging modality of choice in suspected KD, and should be performed when?

at diagnosis, 1 to 2 weeks later, and again after 5 to 6 weeks

A 15-year-old girl presents to clinic for her annual well check. She is sexually active, and screening tests for sexually transmitted infections are sent. Test results reveal she is infected with a sexually transmitted pathogen that is intracellular. Which of the following is the most likely organism?

chlamydia trachomatis - causes intracellular infections.

Fever with temperatures < 39°C in healthy children generally does not require treatment. Antipyretic therapy is beneficial in high-risk patients who have ...

chronic cardiopulmonary diseases metabolic disorders neurologic diseases those who are at risk for febrile seizures - Hyperpyrexia (>41°C) indicates greater risk of hypothalamic disorders or CNS hemorrhage and should be treated with antipyretics

A child who returned 2 wk ago from equatorial Africa is admitted to the hospital in a coma, with high fever and a palpable spleen. The liver is not enlarged. Laboratory studies reveal hypoglycemia, but the cerebrospinal fluid is normal. The most likely diagnosis is:

falciparum malaria - Children with malaria often lack typical paroxysms and have nonspecific symptoms, including fever (may be low grade but is often greater than 104°F), headache, drowsiness, anorexia, nausea, vomiting, and diarrhea. - Distinctive physical signs may include splenomegaly (common), hepatomegaly, and pallor due to anemia. - Typical laboratory findings include anemia, thrombocytopenia, and a normal or low leukocyte count. The erythrocyte sedimentation rate (ESR) is often elevated. - Any child who presents with fever or unexplained systemic illness and has traveled or resided in a malaria-endemic area within the previous year should be assumed to have life-threatening malaria until proven otherwise.

Adverse rxns after MMR

fever rash low platelets LAD allergic rxn - ex rash - 5-12 days post-vaccine

What are two common treatments for immune thrombocytopenia?

intravenous immune globulin and steroids

Results from standardized aptitude (intelligence), achievement, and adaptive tests are typically reported as standard scores with means and standard deviations of ...

mean of 100 and standard deviation of 15 - The normal range is considered to be within 1 standard deviation (85-115) of the mean - Intellectual disability is present when both intelligence and adaptive functioning are more than 2 standard deviations below normal (scores <70) School districts may define a learning disability by 1) a discrepancy of at least 1 to 2 standard deviations between IQ scores and achievement scores 2) low achievement in a student with at least low average intelligence 3) a student's failure to respond to evidence-based educational interventions

Abnormal scoliometry must be confirmed on x-ray by measuring Cobb angle. Cobb angle <10 degrees is considered...

normal --> *requires no follow-up unless back pain or neuro symptoms develop*

In utero, homeostasis is maintained by the...

placenta - limited role for the kidney

A 10 mo old child presents to the emergency department with a 4-day history of fever, with temperature to 105°F, and watery diarrhea and has just experienced a generalized seizure. The most likely cause of this syndrome is:

shigella gastroenteritis - Ingestion of shigellae is followed by an incubation period of 12 hr to several days before symptoms ensue. - Severe abdominal pain, high fever, emesis, anorexia, generalized toxicity, urgency, and painful defecation characteristically occur. - Untreated diarrhea can last 1-2 wk; only about 10% of patients have diarrhea persisting for > 10 days.

In contrast to flexor tendon injuries, injuries to the extensor tendons on the dorsal aspect of the fingers often do well with conservative management. These injuries typically require

splinting in extension for 6 to 12 weeks

The most appropriate management of a splenic sequestration crisis is to

transfuse packed red blood cells in small aliquots

Ready to feed formula - stability prepared: - stability opened/unprepared:

- 48 hours (refrigerated) - 48 hours (refrigerated)

Changes regarding nutrition at 9 mo WCC

- Introduce solids - Introduce cup - Gradually decrease milk from bottle - No cow's milk until 1 year but can give milk products, like cheese, cottage cheese as protein has been denatured)

Sunscreen guidelines for kids

- SPF of 15 or greater - Re-apply q2 hours for children older than 6 months - consider using lip balm or lip cream containing sunscreen

Recognize *spiral fracture* in pediatrics, which can mean child abuse

- femur fracture in nonambulating child, especially spiral fracture = high suspicion

If you are measuring your body weight in kilograms: Assume your weight is 70Kg In this example: TOTAL DAILY INSULIN DOSE =

0.55 x 70 Kg = 38.5 units of insulin/day

How to use transdermal patch

3 weeks on/1 week off changed 1x/week - 9 days of medication in each patch - inhibits ovulation - estrogen and progestin

Regular BP monitoring, vision, and hearing screening starting at

3 y -4 y - also BMI instead of weight-for-length

At what age does a child typically master copying a circle?

3 yo

A 3-year-old girl is being admitted to the pediatric inpatient floor from the emergency department for a constellation of symptoms, including a high fever for the past six days in conjunction with dry, cracked lips, erythematous hands and feet, unilateral cervical lymphadenopathy, and nonexudative bulbar conjunctivitis. Which of the following therapies would be the most appropriate in the initial management of this patient?

Aspirin and intravenous immune globulin - Mainstay of initial treatment of Kawasaki disease during its acute phase is to give both aspirin and intravenous immune globulin. - The infusion of intravenous immune globulin, given as 2 grams per kilogram, is administered slowly over eight to 12 hours to help reduce the risk of a transfusion reaction. - High-dose aspirin is also given, in dosing of 30 to 100 milligrams per kilogram per day, with a maximum daily dose of 4 grams of aspirin initially, followed by 3 to 5 milligrams per kilogram per day. - Premedication with an antihistamine may help reduce adverse reactions to intravenous immune globulin infusion. - A second infusion of intravenous immune globulin is sometimes needed for children in whom the fever recurs within 48 hours following the initial infusion.

A 5 mo old girl presents with diarrhea and malabsorption and on initial laboratory testing has a neutrophil count of 900/mm3. Which of the following is the most likely diagnosis?

Shwachman-Diamond syndrome - autosomal recessive disorder characterized by pancreatic insufficiency and neutropenia - caused by pro-apoptotic mutations of the SBDS gene, which encodes a protein that may play a role in ribosome biogenesis or RNA processing - initial symptoms are usually diarrhea and failure to thrive because of malabsorption, which develops in almost all infants by 4 mo of age - some patients have respiratory problems with pneumonia and frequent otitis media, as well as eczema - virtually all patients with Shwachman-Diamond syndrome have neutropenia, with the ANC periodically <1,000/μL associated with hypoplastic myelopoiesis

The clinical presentation of a boy with acute onset of gastroenteritis after recent consumption of pork intestines (called "chitterlings" or "chitlins"), is suggestive of

Yersinia enterocolitica gastroenteritis - Yersinia enteritis may be complicated by bacteremia, especially in infants and older children who are at high risk of developing invasive infection, such as underlying conditions with excessive iron storage (eg, receipt of iron chelation therapy, sickle cell disease, β thalassemia, receipt of chronic transfusion therapy), and immunocompromised hosts - Transmission of Yersinia enterocolitica occurs mainly through the fecal-oral route, often via ingestion of contaminated pork products or unpasteurized or inadequately pasteurized milk products, contaminated water, and contaminated vegetables.

This severe disease of the liver, kidney, and peripheral nerve is caused by a deficiency of the enzyme fumarylacetoacetate hydrolase. When untreated, the affected infant appears normal at birth and typically presents between 2 and 6 mo of age. What usually heralds the onset of the disease and is usually precipitated by an intercurrent illness that produces a catabolic state?

acute hepatic crisis - Most hepatic crises resolve spontaneously but may progress to liver failure and death. - Between the crises, varying degrees of failure to thrive, hepatomegaly, and coagulation abnormalities often persist. - Cirrhosis and eventually hepatocellular carcinoma occur with increasing age.

When to give glucagon?

hypoglycemia + seizure, unresponsive, altered mental status, hypoglycemia x3 without returning back to normal BG - dose = 0.5 mg IM - main adverse effects = nausea, vomiting - if hypoglycemia is d/t hyperinsulinism, admin of glucagon will raise plasma BG by >30 mg/dl by 30 minutes (glycemic response) Test: - check POC glucose at baseline before giving - give glucagon 0.3 mg/kg (up to 1 mg) slow IV or IM - check BG at t=10, 20, 30, 40 minutes after by POCT

Postexposure prophylaxis with varicella-zoster immune globulin should be provided for

immunocompromised individuals pregnant women eligible newborns ideally - within 96 hours but up to 10 days after the exposure

Most common method of attempted suicide

ingestion

For bronchospasms in infant, give...

inhalations of bronchodilator, either metaproterenol (alupent) 5% (0.1-0.2 mL) or albuterol (ventolin) 0.5% (0.1-0.4 mL)

MMR, varicella, influenza - what type of vaccines

live

What is the most common physical exam finding in children evaluated for sexual abuse?

normal physical exam - only minority of sexually abused children have abnormal exam findings

The American Diabetes Association recommends that insulin should be initiated at diagnosis for youth with ...

type 2 diabetes if the blood glucose is greater than or equal to 250 mg/dL (13.9 mmol/L) or hemoglobin A1c is greater than or equal to 8.5% Also... - ketosis and if the distinction between type 1 and type 2 diabetes is not clear - Metformin, orally, would be the best pharmacologic therapy if her initial glucose and hemoglobin A1c levels were below the threshold for starting insulin. Once metabolic control is established, metformin may be added to her treatment regimen and the insulin dose lowered.

Age? - Turns head in supine position - Hands fisted near face - Follows face - Discriminates mother's voice

*4 weeks* Also... - chin up in prone position - sucks well - gazes at black-white objects - cries out in distress - startles to voice/sounds - throaty noises

A normal infant may cry for up to 3 hr/day during the developmental peak time of this behavior. This peak is typically at age:

6 weeks - Crying normally peaks at about 6 wk of age, when healthy infants may cry up to 3 hr/day, then decreases to 1 hr or less by 3 mo.

Over-the-counter cough and cold medications have not been shown to be effective in young children, and can pose a significant risk to children younger than

6 years

1st permanent molars = age

6 yo - 2nd permanent molars = 12 yo - 3rd permanent molars = 17-25 yo (sometimes never erupt or arent's present)

Children begin losing teeth at

6 yo - process finishes at 12 yo

Tracheobronchitis and bronchiolitis peak at ages...

6-12 months

Most common "mass" in chest in young child.

"Round pneumonia" caused by strep pneumoniae

2 yo M. Weight 10 kg (<5%ile). Height 85 cm (25-50%ile). Ideal body weight?

(Height (cm)^2x1.65)/1000 = (85^2 x 1.65/1000) = 11.9 kg Ideal body weight % = 10/11.9 x 100 = 84% = mild wasting

yuzpe method

- take birth control pills in two doses, 12 hours apart - works best up to 3 days after unprotected sex

Within 24 hrs post-kidney transplant, prograf dose should be...

0.07 mg/kg/dose PO Q12hours - Check QAM Prograf 12-hour trough levels in post-kidney transplant patients. Target prograf trough levels for all protocols: - Day 0 to 7 days post-tx: 12-14 ng/mL - Day 8 to week 8 post-tx: 10-12 ng/mL - Week 9 to week 12 post-tx: 7-10 ng/mL - After 12 weeks post-tx: 5-7 ng/mL

Drainage of purulent cellulitis is recommended with culture performed on the material obtained. In lesions less than 5 cm in a well-appearing child, drainage alone without antibiotic therapy is likely sufficient. In the face of significant surrounding erythema and fever to 39.2°C, as in this patient, antibiotic therapy should be initiated while awaiting culture results. What abx would be most appropriate?

*Clindamycin* would be the most appropriate to cover S aureus, including CA-MRSA. - Trimethoprim/sulfamethoxazole and doxycycline would also provide appropriate coverage for CA-MRSA in this type of infection, although doxycycline would not be routinely recommended in a child younger than 7 years of age.

Potassium channel opener, hence potassium efflux, hyperpolarization of the islet cell, and decreased insulin release. Side effect is Hyperglycemia. As this drug decreases insulin release, it is used in the management of insulinoma.

*Diazoxide*

Failure of the neural tube to close typically occurs at which gestational age?

*During the fifth and sixth weeks of gestation.*

Which of the following is the best treatment for histoplasmosis?

*Fluconazole* Fluconazole, itraconazole, and amphotericin B are the most commonly used medications.

What syndrome presents with torticollis due to congenital vertebral anomalies?

*Klippel-Feil syndrome*

A 12-year-old boy presents for a well-child check at his pediatrician's office. He is accompanied by his nanny since both parents are at work. The nanny confides to the pediatrician that she has noticed a pattern of the boy's father singling him out in punishment and calling him "stupid," "weak," or "worthless." Which of the following terms best describes this type of psychological abuse?

*spurning* - belittling or name-calling child - singling out child to punish - -humiliating child in public

In a child presenting with diarrhea, the physical finding of a left lower quadrant (fecal) mass suggests a diagnosis of

*functional fecal incontinence* - Encopresis occurs with an 80% male predominance. - In the child with encopresis, soiling episodes rarely occur during sleep.

What is considered normal for recurrent bacterial infections

- Viral URIs up to 8 times/year, lasting up to 14 days each - AOM up to 3 times/year - Pna/sinusitis up to once/year

rare disorder characterized by intrauterine growth restriction (IUGR), poor growth after birth, a relatively large head size, a triangular facial appearance, a prominent forehead (looking from the side of the face), body asymmetry and significant feeding difficulties

*Russell-Silver syndrome* - The majority of individuals with RSS are of normal intelligence, but motor and/or speech delay is common. - Abnormalities involving chromosomes 7 or 11 have been found in up to 60% of RSS patients.

Rare association of congenital cardiopulmonary anomalies consisting of a partial anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply to the right lung.

*Scimitar syndrome* - Combination of pulmonary hypoplasia and partial anomalous pulmonary venous return (PAPVR). - It almost exclusively occurs on the right side. Haemodynamically, there is an acyanotic left to right shunt. The anomalous vein usually drains into: - inferior vena cava: most common - right atrium - portal vein

Criteria for extubation to nasal CPAP in first week of life are:

- adequate respiratory drive, and - mean airway pressure </= 7 cmH2O, and - FIO2 </= 0.35

Type of pulmonary disorder: Stridor - loud Cough - barky Voice - hoarse Dysphagia/drooling - absent Fever - low to moderate (croup) Toxicity - absent, unless tracheitis is present Posture - normal

*Subglottic* disorders - bacterial tracheitis - viral croup

A previously healthy 4-year-old boy presents to the office with his concerned mother due to bruising all over his body. She noted a few bruises yesterday, but they are more numerous today. He also had an episode of epistaxis this morning. Daycare denied any injuries, and the family has not witnessed any injuries. On examination, the child has ecchymoses scattered over all four extremities and the abdomen. Two wet purpura are noted on the buccal mucosa, and petechiae are present on the tongue. Which of the following is also likely to be present?

*Thrombocytopenia* Immune thrombocytopenia - from immune-mediated destruction of platelets after circulating antibodies bind to surface antigens on platelets - most common among children aged two to five years. - severe cases may result in intracranial or gastrointestinal hemorrhage. - more commonly, the thrombocytopenia leads to mucocutaneous bleeding. - Mucous membrane bleeding is most commonly manifest as epistaxis, gingival bleeding, or wet purpura on the buccal mucosa. - When immune thrombocytopenia is suspected, the clinician should obtain a complete blood count, reticulocyte count, peripheral smear, and direct antibody test. - The reticulocyte is normal, and the direct antibody test is negative.

Which of the following is a risk factor for polycystic ovary syndrome?

*Use of antiepileptic drugs* - valproate most commonly

The classic phenotypic features of trisomy 18 include ...

*a clenched fist with overriding fingers, rocker bottom feet, characteristic facial dysmorphology, prenatal and postnatal growth deficiency, and hypotonia.* - Most newborns with trisomy 18 die within the first month after birth; only 5% to 10% will survive beyond 1 year of age.

A 4-year-old boy presents with swelling that has increased over the past week. The swelling is most noticeable around his eyes in the mornings but is most notable in his legs in the afternoon. On examination, he has pitting edema of the bilateral lower extremities. He is also hypertensive. Labs are significant for hypoalbuminemia and 3+ proteinuria. What is the most likely etiology of the child's hypoalbuminemia?

*idiopathic nephrotic syndrome* - Nephrotic syndrome results from urinary protein losses, which lead to hypoalbuminemia. The hypoalbuminemia leads to dependent edema. As such, the edema may be most noticeable around the eyes in the mornings with a shift to the lower extremities as the day progresses. - Idiopathic nephrotic syndrome is the most common cause of nephrotic syndrome in childhood, representing more than 90% of cases of nephrotic syndrome in children under age 10.

What immune cell is decreased in Shwachman-Diamond syndrome?

*neutrophils*

OSAS most common in what kids?

*preschool-aged children* - age at which tonsils and adenoids are the largest in relation to the underlying airway size

An 11-month-old male presents to the office for a complaint of constipation. The parents are concerned because they noted a mass in his abdomen during a recent bath time. Urine testing performed reveals elevated levels of homovanillic acid and vanillylmandelic acid. What factor is associated with a better prognosis for survival in this patient's most likely diagnosis?

*tumor cell hyperploidy* - abdominal mass, likely causing this patient's constipation due to mass effect, in the setting of elevated catecholamine metabolites in the urine is most consistent with a diagnosis of neuroblastoma. - originate from primitive sympathetic ganglion cells from the fetal neural crest that are supposed to become part of the adrenal medulla and sympathetic nervous system. - higher incidence of neuroblastoma amongst patients with Turner syndrome, and there seems to be an association between neuroblastoma and Hirschsprung's disease, central hypoventilation, and neurofibromatosis type 1. - third most common childhood cancer overall, is the most common extracranial solid tumor in the pediatric population, and is the most common malignancy in children under one year of age. - Pathologic analysis revealing tumor cells hyperploidy is associated with a better prognosis than hypoploidy.

A 6-day-old male child presents to clinic for a well-child visit after hospital discharge. On physical examination the baby has a smooth philtrum, midface hypoplasia, short palpebral fissures, and a thin vermillion border. Based on your suspected diagnosis, what finding is this infant most likely to also have?

*weight is small for gestational age* fetal alcohol syndrome - first trimester exposure --> facial and structural anomalies - Hyperactivity, cognitive impairment, learning disability, decreased tone, or seizures.

How to diagnose SC disease prenatally

- chorionic villus sampling - amniocentesis

The high blood sugar correction factor: Correction Factor =

1800 ÷Total Daily Insulin Dose = 1 unit of insulin will reduce the blood sugar so many mg/dl

FTT = drop by...

2%

Standard dose of oral ibuprofen

10 mg/kg

Babies have how long to get their spO2 up to 95?

10 minutes

The gut has formed and has completed its rotation back into the abdominal cavity by...

10 wks of gestation - by 16 wks, fetus can swallow amniotic fluid - GI motor activity present before 24 wks but organized peristalsis not established until 29-30 wks --> facilitated by antenatal corticosteroid treatment

Standard dose of oral tylenol

15 mg/kg

Definitive test for Congenital Adrenal Hyperplasia

17-OH progesterone before and after ACTH bolus

3 words by what age?

18 mos

Recurrent pneumonia is defined as

2 or more episodes in a single year or 3 or more episodes ever, with radiographic clearing between occurrences. - An underlying disorder should be considered if a child experiences recurrent pneumonia.

Typical dosing for magnesium in pediatric asthmatic pts

40-50mg/kg IV infused over 20-30min

Rule of 8s for determining need for referral in childhood vision screening

2: 2+6 = 8 (20/60) 3: 3+5 = 8 (20/50) 4: 4+4 = 8 (20/40) 5: 5+3 = 8 (20/30) 6: 6+2 = 8 (20/20)

In an otherwise healthy child, elevated temperature does not pose a threat unless it exceeds

41.7°C - at this level, an element of hyperthermia likely exists because of dehydration in addition to fever; hyperthermia should be promptly addressed

Healthy, well-nourished, lactating women are advised to increase their calories by

450-500/day. - That is a lot of calories which is turned into ~70 gallons or ~250 liters of breast milk/year.

Although discontinuous, growth during the years between 6 and 12 yr is characterized by annual weight and height increments of:

3.5 kg, 6 cm - Growth during the period averages 3-3.5 kg (7 lb) and 6-7 cm (2.5 in) per year. - Growth occurs discontinuously, in 3-6 irregularly timed spurts each year, but varies both within and among individuals. - The head grows only 2-3 cm in circumference throughout the entire period, reflecting a slowing of brain growth.

# of permanent teeth

32 - 20 deciduous teeth - count from top top right when reporting

Evidence demonstrates that in athletes and military populations, stress fracture rates are lower when 25-OH-D levels are greater than or equal to 30 ng/mL. Based on these findings, the current recommendation for athletes to minimize injury risk and optimize athletic performance is to maintain 25-OH-D levels between

32 and 50 ng/mL (80-125 nmol/L)

Most maternal immunoglobulins do not cross the placenta before...

32 weeks

Use UAC Insertion tray and appropriate sterile technique including cap, mask and gloves. Restrain infant's limbs. For infants >1500 g, use...

5 Fr catheter - for those </= 1500g, use 3.5 Fr catheter - attach stopcock to cath and fill the system with sterile heparinized flush solution (0.9% NaCl) - cleanse umbilical cord and adjacent abdomen with iodine solution --> drape area so only umbilical cord is exposed --> place cord tie around base of umbilical cord and tie loosely --> cut cord about *0.5 cm above skin line* --> if bleeding, tighten the tie

Birth weight doubles by ...

5 mos

Color vision by what age?

5 mos

MAP for infants...

5 wks + GA

Should use barrier method how long after inserting nexplanon?

7 days

Heart fully formed by

7 wks

Alveoli increase in number until what age?

8 years old

T2D diabetes management approach if: symptoms, blood glucose <250, positive ketones =

insulin +/- metformin +lifestyle changes

T2D diabetes management approach if: symptoms, blood glucose <250, negative ketones =

insulin +/- metformin +lifestyle changes

Most common type of ASDs

secundum ASD - due to deficiency in central part of septum primum that develops from middle of atrial cavity and grows inferiorly to meet endocardial cushion

Cardiac lesions with left-to-right shunting can include

atrial septal defects ventricular septal defects atrioventricular septal defects aortopulmonary window patent ductus arteriosus - Hemodynamically significant lesions result in cardiac enlargement and pulmonary congestion and warrant repair

What ventilation rate should be used during positive pressure ventilation of a newborn?

Breaths should be given at a rate of 40 to 60 breaths per minute.

Bacterial vaginosis can be diagnosed clinically using the ...

Amsel criteria by the presence of 3 of the following: - homogeneous, thin, white discharge that adheres to the vaginal walls - greater than 20% clue cells on microscopy - vaginal pH greater than 4.5 - release of a fishy amine odor with the addition of 10% potassium hydroxide to a drop of vaginal discharge (ie, positive whiff test)

Arising from the intestinal wall or the mesentery, intra-abdominal desmoid tumors are often associated with what mutations?

APC mutations - carry high risk of mortality - grossly, are firm and non-encapsulated masses arising from regions of connective tissue - margins can be poorly defined and difficult to identify - microscopically, are bland, appearing with well-differentiated cells and bands of fibrous tissue

Best initial imaging modality to confirm the diagnosis in an infant with nonbloody, nonbilious emesis.

Abdominal US

General averages for occipitofrontal circumference or head circumference (OFC) increase are:

Average at birth = ~35 cm 0-3 months = 2 cm/month (average 3 month old is ~ 41 cm) 0-1 year = 1 cm/month (average 1 year old is ~ 47 cm) > 1 year = only another 8 cm total (average adult is ~ 55 cm)

Refers to outline of airway made visible by filling the alveoli with cells or fluid

Bronchogram - air bronchogram best seen in respiratory distress syndrome (RDS) in neonate

The classic presentation of congenital or early-onset glaucoma is

corneal clouding photophobia chronic or intermittent tearing

What is pulmicort?

Budesonide (BUD) - brand name Pulmicort - available as an inhaler, pill, nasal spray, and rectal forms - inhaled form is used in the long-term management of asthma and chronic obstructive pulmonary disease (COPD) - nasal spray is used for allergic rhinitis and nasal polyps - pills in a delayed release form and rectal forms may be used for inflammatory bowel disease including Crohn's disease, ulcerative colitis and microscopic colitis

Boy has cellulitis that needs treatment with antibiotics. He has risk factors for methicillin-resistant Staphylococcus aureus (MRSA), including a history of skin trauma, a parent in the health-care field, and a family member with a history of skin abscesses. Therefore, antibiotics must be chosen that provide coverage for common skin flora (eg, β-hemolytic Streptococcus, methicillin-sensitive Staphylococcus aureus), as well as MRSA. What is the most appropriate treatment?

Clindamycin - For hemodynamically stable children with cellulitis and risk factors for MRSA, initial antibiotic choice should include coverage for MRSA and β-hemolytic Streptococcus. Clindamycin is the preferred first-line agent. - In communities where the prevalence of clindamycin-resistant MRSA is greater than 15%, an alternative antibiotic strategy such as combination therapy (eg, amoxicillin or cephalexin plus trimethoprim-sulfamethoxazole or doxycycline), vancomycin, or linezolid must be considered. - For hemodynamically stable children with cellulitis and no risk factors for MRSA, antibiotic coverage to treat methicillin-sensitive Staphylococcus aureus and β-hemolytic Streptococcus such as cephalexin, cefadroxil, or cefuroxime should be initiated.

A premenarcheal 12-year-old girl presents for evaluation of left lower quadrant pain for the past two days. She describes the pain as intermittent, dull or occasionally squeezing, and progressively worsening over time. She has had three episodes of nonbilious vomiting associated with episodes of pain today and also complains of persistent nausea with decreased appetite. She denies diarrhea, dysuria, and vaginal discharge. She has been afebrile throughout this period. On examination, her vital signs reveal a T of 98.6°F (37°C), HR of 124 bpm, RR of 16 breaths/min, and BP of 112/74 mm Hg. She appears uncomfortable but nontoxic. She is clinically well-hydrated with moist mucous membranes and capillary refill time is < 3 seconds. Her cardiopulmonary exam is normal. On her abdominal exam, she is noted to be nondistended, has normoactive bowel sounds, and has no organomegaly. She is exquisitely tender to deep palpation of the left lower quadrant with associated involuntary guarding. A urine pregnancy test is negative. An obstruction series is nonrevealing. A transabdominal pelvic ultrasound shows unilateral enlargement of the left ovary and pelvic free fluid. There is positive Doppler flow to the adnexal structures bilaterally. The pediatric hospitalist is consulted by the emergency room physician for possible admission. Which of the following is the most appropriate next step in the patient's evaluation?

Consult pediatric surgery for exploratory laparoscopy Ovarian torsion - many cases are associated with cystic or neoplastic ovarian pathology (especially if larger than 5 cm), but torsion can occur in normal ovaries, particularly among younger patients. - enlarged adnexa on ultrasound on the symptomatic side should raise significant clinical suspicion for torsion. - because the ovary may be torsing and detorsing intermittently, the presence of Doppler flow on the affected side does not rule out the diagnosis. - If there is clinical suspicion and ultrasound evidence of ovarian torsion, surgical intervention should not be delayed, and the physician should consult the surgical service for exploratory laparoscopy or laparotomy.

Ichthyosis vulgaris is the most common form of ichthyosis. The key physical findings in ichthyosis vulgaris are prominent scales on the extensor surfaces of the legs and hyperlinear palms. Treatment?

Emollient containing an α-hydroxy acid (eg, ammonium lactate)

A 10 yr old girl is being evaluated for new onset of school problems, obsessive-compulsive behavior, and occasional uncontrolled movements of the hands. She has been healthy and has not taken any medications. Further evaluation is most likely to reveal:

Evidence of streptococcal infection - Sydenham chorea (SC, St. Vitus dance) is the most common acquired chorea of childhood. - It occurs in 10% to 20% of patients with acute rheumatic fever, typically weeks to months after a group A β-hemolytic streptococcal infection. - Peak incidence is at age 8 to 9 yr, with a female predominance of 2:1.

There are significant variations in the incidence of type 1 diabetes mellitus worldwide, with what countries having the highest incidence?

Finland and Sardinia

Initial management of acute pancreatitis

Fluid replacement Pain control - hydromorphone or fentanyl (IV) - meperidine vs morphine

What is flovent?

Fluticasone - used to control and prevent symptoms caused by asthma - works by reducing swelling (inflammation) of the airways in the lungs to make breathing easier - good for uninsured - with plastic chamber - 2 puffs morning & 2 at night - inhaled steroid - lowest strength - if an asthma attack occurs, use your quick-relief inhaler (such as albuterol, also called salbutamol in some countries) as prescribed

Combination Therapies for Asthma --> name some...

Fluticasone + Salmeterol (Advair) Budesonide + Formoterol (Symbicort) Mometasone + Formoterol (Dulera) Fluticasone + Salmeterol (AirDuo Respiclick)

Leading cause of goiter and hypothyroidism in children and adolescents in countries with adequate iodine supplementation

Hashimoto's thyroiditis - TPO, thyroglobulin Ab - screen for high-risk population: family hx, DM type I, turner syndrome - goiter, slow linear growth, clinical signs, fam hx *Check TSH, FT4, TPO, anti-TG *levothyroxine therapy *goal TSH in upper half or normal *monitor q3-6 mos

Which condition should patients with osteogenesis imperfecta be screened for?

Hearing loss, which may be detected in 50 to 90% of patients with osteogenesis imperfecta, though it may not be present at birth.

What is the most common drug causing gynecomastia in males?

Marijuana.

Recognize myringosclerosis

Myringosclerosis - characterized clinically by asymptomatic calcium and phosphate crystal deposits within the tympanic membrane that appear as whitish plaques on otoscopy - typically asymptomatic and rarely requires treatment, because hearing loss that involves the ossicles is uncommon

Imaging for kid with.... pneumonia

PA and lateral radiograph

Imaging for kid with.... suspected vascular rings

PA and lateral radiograph

The term PC20 (provocative concentration 20) is defined as the exact concentration of methacholine that causes a 20% drop in FEV1 from baseline FEV1. A positive test is one in which ...

PC20 occurs at less than 4 mg/mL - less than 1 mg/mL = moderate-to-severe bronchial hyperreactivity

You are caring for a 5 yr old child with new onset seizures. MRI findings are consistent with neurocysticercosis, with multiple viable cysts. The most appropriate treatment strategy is:

Start valproic acid and prednisolone on day 1, followed by albendazole on day 3 - initial objectives of the management of cysticerosis are to diagnosis and manage hydrocephalus due to ventricular obstruction - if seizures are recurrent or associated with calcified lesions, treatment should be continued for 2-3 yr before attempting weaning from anticonvulsants - multiple lesions and complex presentations are typical of disease in adults - albendazole is the antiparasitic drug of choice (15 mg/ kg/day PO divided bid for 7 days; maximum 800 mg/day). It can be taken with a fatty meal to improve absorption. Praziquantel is an alternative (50-100 mg/kg/day PO divided tid for 28 days), but requires more complicated management due to an interaction with corticosteroids - worsening of symptoms can follow the use of either drug due to the host's inflammatory response to the dying parasite - patients should be medicated with prednisolone 2 mg/kg/day or 0.15 mg/kg/day oral dexamethasone, either concurrent with albendazole, or starting albendazole on the third day of corticosteroids

What is the next step in managing severe hyperbilirubinemia if the total serum bilirubin level continues to rise despite intensive phototherapy and if the total level is within 2 to 3 mg/dL of the exchange transfusion level?

The AAP guideline recommends administering IVIg at that point.

The general calculation for the body's daily insulin requirement is:

Total Daily Insulin Requirement (in units of insulin) = Weight in Pounds ÷ 4 Alternatively, if you measure your body weight in kilograms: Total Daily Insulin Requirement (in units of insulin) = 0.55 x Total Weight in Kilograms

What is idiopathic toe walking?

Toe walking past the age of 3 years.

ETT placement ...

Wt (kg) + 6 = cm at the gum line

For infections associated with long-term vascular access devices (Hickman, Broviac, totally implantable devices), antibiotic treatment is successful for most systemic bacterial infections without removal of the device, although tunnel infections (as suggested by the erythema over the catheter tunnel in this case) are often an exception. Antibiotic therapy should be directed to the isolated pathogen and given for a total of 7-14 days, depending on the organisms isolated. Central line associated bloodstream infections (CLABSIs) caused by Staphylococcus aureus may require a longer duration of therapy. Until identification and susceptibility testing are available, empirical therapy, based on local antimicrobial susceptibility data and usually including ...

a 3rd or 4th generation cephalosporin or aminoglycoside plus vancomycin - ex. cefepime + vancomycin

Always verify location of UAC tip radiographically; it should be in the...

abdominal aorta below the 3rd lumbar vertebrae (L-3) and above the aortic bifurcation (usually, bottom of L-4) - *do not feed an infant with a UAC*

When should the HPV vaccine be given?

age 11-12 - may be given as early as 9 - recommended for females through 26 yo - for males through 21 yo - pts require 3-dose series - 2nd dose given 1-2 months after 1st dose - 3rd dose given 6 months after 1st dose - not recommended for pregnant women

Precocious puberty = onset of secondary sexual characteristics before...

age 8 in girls age 9 in boys

Achondroplasia is characterized by disproportionate short stature with rhizomelic shortening, macrocephaly, and facial features of frontal bossing and midface retrusion. The inheritance pattern of achondroplasia is

autosomal dominant with complete penetrance

Inheritance of Hurler disease

autosomal recessive - hepatosplenomegaly, coarse facial features, corneal clouding, large tongue, prominent forehead, joint stiffness, short stature, skeletal dysplasia/dysostosis multiplex

The 1st-line treatment regimens recommended by the CDC for uncomplicated C. trachomatis genital infection in men and nonpregnant women include

azithromycin (1 g PO as a single dose) doxycycline (100 mg PO twice a day for 7 days)

This child has fever, chills, myalgias, and headaches in the context of a recent camping trip, making tick-borne illnesses an important consideration. His laboratory findings include thrombocytopenia and anemia, which appears to be hemolytic given elevated indirect bilirubin and appearance of schistocytes on blood smear. Most importantly, the peripheral smear shows periplasmic tetrads, which are concerning for

babesiosis

What class of antihypertensives is contraindicated in patients with hypertension and tachycardia in amphetamine intoxication?

beta-blockers

Intussuscecption is a common cause of acute abdomen in children of what ages?

between 6 months and 2 years

If your baby is teething, he may drool, become fussy, or put things in his mouth. You can use

cold teething ring - avoid Orajel and regular use of Tylenol

For research purposes, 1- to 4-month-old infants who cry ≥3 hours per 24 hours across a specified number of days are said to have

colic - derived from Greek word for intestine, implying that crying is caused by GI disorder and pain; controversial inference

Initial evaluation of an infant or child with hepatomegaly should include:

complete metabolic panel including liver function tests complete blood cell count coagulation panel evaluation for infectious causes abdominal ultrasonography with Doppler flow

Meningomyelocele is usually diagnosed how?

prenatally by ultrasound during 2nd trimester - positive screening for maternal serum alpha-fetoprotein may also prompt fetal US

BUN:Cr ratio of >20:1 suggests...

prerenal acute kidney injury

Pulmonary vascular resistance does what after birth when the fetal pulmonary and systemic circulations separate and the lungs ventilate for the first time?

decreases

Dental fractures classified based on ...

depth of fracture = Ellis system

Behaviors that are most concerning for suicide include

distributing possessions revealing suicidal thoughts on social media being preoccupied with death such as in drawings, play, music, and media

Factor VIII is produced by the

endothelium - often normal or elevated in ALF whereas it is consumed and thus low in DIC

Low serum chloride levels and metabolic alkalosis are seen in

hypertrophic pyloric stenosis

hyperopia

farsightedness

Myopia, or nearsightedness, is defined as a condition in which the visual images come to a focus where?

in front of the retina of the eyes = especially defective vision of distant objects

At what age does appendicitis most commonly occur?

in the second decade of life

The tip of the UVC should be where?

in the uppermost portion of the IVC or inferior cavoatrial junction.

When are most cases of posterior urethral valves first observed?

in utero

To increase oxygenation of conventional ventilator...

increase FiO2 increase PEEP

IZZ for age 3

influenza

Triad of veno-occlusive disease of liver (VOD)

jaundice weight gain painful (HSM)

EOS / sepsis risk calculator...

kaiser sepsis score - do TcB at 24h and 48h on all babies

Imaging for kid with.... suspected pneumothorax

lateral radiograph with pneumothorax side up

Presentation of hypopituitarism, 2nd most common cause of persistent neonatal hypoglycemia...

microphalus nystagmus prolonged jaundice persistent severe hypoglycemia - high GIR - despite glucagon - despite abx lethargy hypotonia hypothermia hypotension midline facial anomalies Diagnostics - critical sample = +++ ketones, very low GH, very low cortisol

Medications that can be used for mild OSA

montelukast flonase - alone with weight loss if needed

It can treat and prevent overactive parathyroid glands in patients with chronic kidney disease who are on dialysis.

paricalcitol/zemplar

IVH, the most common type of neonatal intracranial hemorrhage, occurs mainly in ....

preterm infants </= 32 wks of gestation

Antibiotic prophylaxis is recommended for procedures that have the greatest potential to cause bacteremia with an organism that can cause IE in patients who are at the greatest potential risk. Procedures involving the mucosa of the oropharynx, respiratory, skin, and musculoskeletal systems (but not the gastrointestinal or genitourinary systems) should be considered. Which patients are among those who are at highest risk and will need prophylaxis?

prosthetic material on a cardiac valve unrepaired cyanotic heart disease residual defects after repair

The organization of infant behavior into discrete behavioral states may reflect an infant's inborn ability to regulate arousal. Six states have been described:

quiet sleep active sleep drowsy alert fussy crying

hypocarbian in newborn, due to improved ventilation, can decrease cerebral and myocardial blood flow. manage by...

reducing ventilation rate or PIP

Which is thought of as the best marker to follow severity/progression of HLH?

sCD25 (soluble IL-2Ralpha) - thought to be the best marker to follow disease progression/course for HLH (hence, it is recommended to be obtained weekly during induction therapy)

Synesthesia, a state of altered perception where one sense is perceived as another (eg, hearing colors), is the most distinctive symptom indicating hallucinogen use. Hallucinogen use carries a risk for

serotonin toxicity - Treatment of acute hallucinogen intoxication is primarily supportive

Presenting symptoms for 95-99% of pts with gaucher disease

splenomegaly

Cervical spine radiographs in Down syndrome are warranted only when

symptoms or physical examination findings suggest myelopathy

A 15-year-old adolescent girl with a rash and difficulty breathing is seen in the pediatrician's office for evaluation. She has no known allergies. For long-term control of her asthma, she recently started using an inhaled medication newly approved by the US Food and Drug Administration. The medication has no specific black-box warnings and is approved for use in patients older than 12 years. She has urticaria throughout her body and diffuse wheezing. She has a temperature of 36.5°C, a heart rate of 130 beats/min, a respiratory rate of 28 breaths/min, and a blood pressure of 100/40 mm Hg. Her breathing is labored with audible stridor and wheezes and suprasternal retraction. The remainder of the physical examination findings are unremarkable. She receives intramuscular epinephrine and is transferred to the intensive care unit. A possible adverse drug reaction is suspected. Of the following, the MOST appropriate reporting entity is

the Food and Drug Administration MedWatch program - The US Food and Drug Administration encourages MedWatch submissions regarding safety issues related to medications, biologics, medical devices, dietary supplements, or cosmetics. - Online MedWatch forms are available, and physicians should complete them when they suspect serious adverse reactions. - Pediatric patients may be more prone to adverse drug reactions than are adult patients.

As a rule of thumb, between the ages of 2 and 5 yr, the number of words in a typical sentence equals

the child's age - 2 by age 2 yr, 3 by age 3 yr, and so on

Immediately obtain CXR to confirm proper position of ET tube in infant...

tip of ET tube 1 cm above carina - tip of ET tube should be between T1 & T2

Most common cause of fetal hydronephrosis

transient hydronephrosis

Apnea of Infancy

unexplained episode of cessation of breathing for 20 seconds or longer OR shorter respiratory pause associated with bradycardia, cyanosis, pallor, and/or marked hypotonia - usually ceases by 37 wks postmenstrual age but may persist for several weeks beyond term - extreme episodes usually cease at 43 wks postconceptional age

bupthalmos

unilateral or bilateral ocular enlargement

Children 1 mo of age or older with suspected pneumococcal meningitis should be treated with combination therapy using

vancomycin (60 mg/kg/24 hr divided q 6 hr IV) and ceftriaxone (100 mg/kg/24 hr divided q 12 hr IV)

The child has Varicella zoster virus, causing chickenpox. Prodrome symptoms of fever and malaise can precede the rash. The rash most often starts on the ...

*trunk, scalp, or face* - The rash has maculopapular, vesicular lesions that scab over. They range in size from 5 to 12 mm. - Most lesions are small, having an erythematous base with a diameter of 5 to 12 mm. The rash spreads to the arms and legs. - The patient is no longer contagious when all lesions have scabs. The crusts fall off in seven to 10 days. - The infection has become relatively uncommon due to the availability of a vaccine.

Hematuria is defined as

>/= 3 to 5 RBCs per highpower field (HPF) using a standard urinalysis technique on a centrifuged sample.

What level of EtCO2 generally indicates inadequate compressions during CPR?

<10-15 mmHg - An ETCO2 <10-15 mmHg indicates poor cardiac output with not much blood reaching the lungs. Hence, the quality of compressions may need to be improved. - An ETCO2 of >35-40 during CPR generally coincides with return of spontaneous circulation (ROSC)

For the girl in this vignette, the clinical feature that best supports the diagnosis of acute bacterial sinusitis is duration of symptoms. There are 3 clinical criteria that help discern acute bacterial sinusitis from its more common counterpart viral upper respiratory tract infection (URI):

- Persistence of symptoms beyond 10 days without improvement - Worsening of symptoms or new onset of symptoms after initial improvement - Severe symptoms at onset lasting for 3 consecutive days

What infants get head US?

<32 wk or <1500 g - after 1 wk

Encourage use of cup starting at what age?

6 months - According to the American Academy of Pediatrics, your little one is likely ready for you to begin introducing sippy cups to him or her between 6 - 9 months old.

Rare hereditary metabolic disorder caused by deficiency of enzyme glutaryl-CoA dehydrogenase...

Glutaric aciduria type I - usually well at birth but present during early infancy/childhood with acute event of vomiting, seizures, reduced consciousness often in association with infection (bad cold, gastro)

This is the most common tumor in children, affecting about 5% of the population.

Hemangioma - More common in premature infants (especially those weighing < 1 kg at birth), female infants, and white infants - Hemangiomas that are larger than 1 cm, located on the medial aspect of the periorbital area, or cause displacement of the globe or ptosis are more likely to lead to visual impairment

3 year old ex-26 preemie who is tracheostomy and ventilator dependent presents to the ER with 3 day history of increased secretions. She started developing rhinorrhea and cough 3 days prior. They are frequently suctioning. Her humidification is working at home. In the ER, her saturations are normal and she does not appear to be in distress. You obtain a CXR and there is no evidence of pneumonia. What is the next best step in management?

Increase airway clearance at home. The steps of airway clearance include 1. Bronchodilator such as albuterol to widen and relax the airways. Albuterol has been also shown to improve cilia movement, therefore this will help move the mucus out of the lungs 2. Hypertonic saline: if secretions are extremely thick, this can be given to loosen up the secretions to make it easier to be expectorated or suctioned 3. Chest Percussion- this helps loosen the mucus from the airway Goals of airway clearance is to clear the mucous from the lungs and prevent atelectasis.

A 1 day old newborn, born to a myasthenic mother, has generalized hypotonia and weakness. Which of the following best describes the prognosis?

No increased risk of myasthenia gravis - Infants born to myasthenic mothers can have respiratory insufficiency, inability to suck or swallow, and generalized hypotonia and weakness. They might show little spontaneous motor activity for several days to weeks. Some require ventilatory support and feeding by gavage during this period. - After the abnormal antibodies disappear from the blood and muscle tissue, these infants regain normal strength and are not at increased risk of developing myasthenia gravis in later childhood.

A 9-month-old boy presents to the office with three day history of fever. Temperatures have ranged from 102°F to 104°F. Along with fever, the patient has been acting "more irritable and crankier" than normal with a slightly decreased appetite. Today, the patient is afebrile but throughout the day developed a maculopapular, blanchable exanthem on his neck and trunk that seems to be spreading to his face and extremities. The rash does not seem itchy. On exam, the patient is active and alert and otherwise well appearing. Which of the following is the most likely diagnosis?

Roseola infantum - ninety percent of the cases are children under 2-years-old, with a large portion of those between 7 and 13 months of age. - most cases occur without known exposure. - classically present with 3 to 5 days of a high fever (often times exceeding 104℉) that abruptly resolves and is followed by the development of a rash. - rash is typically macular or maculopapular and blanchable, spreading from the trunk and neck out towards the face and extremities. - rarely pruritic. - irritability, lymphadenopathy, anorexia and erythematous tympanic membranes. - self limited - Treatment is supportive care for the fever and education for parents of the natural course of the virus.

A 5 yr old in your practice underwent screening for celiac disease because his mother was recently diagnosed with this condition. His screening test is positive, however he is asymptomatic. Intestinal biopsy is negative. How would you classify this patient, and what are your recommendations to the mother?

The child has potential celiac disease and should be monitored for the development of future symptoms; a gluten-free diet is not mandatory - Potential celiac disease is defined when patients are identified by positive screening studies but without documented celiac disease on small bowel biopsy. I - The nutritional risks, particularly osteopenia, are those mainly feared for subjects who have silent celiac disease and continue on a glutencontaining diet. - There are no guidelines concerning the need for a glutenfree diet in subjects with "potential" celiac disease (patients with positive celiac disease-associated serology but without enteropathy).

In an infant with atypical genitalia, palpable gonads are almost always testes, making a 46,XY karyotype likely. An infant indeed has a 46,XY karyotype which narrows the differential diagnosis to conditions associated with an undervirilized male, or 46,XY disorder of sex development (DSD). The relatively high luteinizing hormone, testosterone, and dihydrotestosterone (DHT) levels are consistent with

partial androgen insensitivity syndrome - caused by a defect in the androgen receptor *in complete androgen insensitivity syndrome, the external genitalia are phenotypically female.

Urolithiasis typically causes what presentation?

sharp, intermittent cramping pain in the back, with radiation to the groin

Underweight BMI for kid

<5%

A full-term male has a history of bilateral antenatal hydronephrosis and a thickened bladder wall. The findings were noted on second-trimester ultrasound and were again present on third-trimester ultrasound. In the newborn nursery, his renal ultrasound demonstrates a thickened bladder wall, bilateral hydroureter, and bilateral hydronephrosis. A voiding cystourethrogram demonstrates an elongated posterior urethra while the newborn is voiding. Which of the following is indicated to treat the newborn's diagnosis?

*ablation of posterior urethral valves*

A 3-month-old female infant is brought to your office for "excessive crying." Her mother reports that she cries consistently for 3 or 4 hours most evenings. She sometimes improves when being held and carried, but still "seems upset." Her mother reports that her belly feels "gassy" and that she has tried simethicone drops in the evening, but this has not helped. The infant is breastfeeding, and her mother has tried eliminating milk and soy from her diet. You diagnose infant colic. Of the following, the MOST appropriate step in the management of this infant's symptoms is to recommend

*swaddling, gentle rocking, and decreased stimulation* - Infant colic, as defined by an infant crying more than 3 hours per day, more than 3 days per week, for more than 3 weeks at a time, is common, occurring in up to one-quarter of all infants. It peaks around 6 weeks of age and typically resolves by 4 months of age. Periods of crying typically occur in the late afternoon or evening. In between periods of crying, colicky infants feed and act normally. The growth trajectory and the examination of infants with colic are typically normal. No laboratory or imaging evaluation is needed for the diagnosis, but a detailed history and examination should rule out other causes of excessive crying: gastroesophageal reflux disease, constipation, occult fracture, infection, and feeding disorders. - Since it is so common, as well as disruptive and troubling to parents, many interventions have been tested in an attempt to find an easy, scalable, effective way to quiet excessive infant crying. These efforts have been largely unsuccessful, and the prevailing advice to parents of colicky infants is to be assured that this is a self-limited problem and is best handled by swaddling the infant and maintaining a quiet, soothing environment. Encouraging parents to recruit other trusted caregivers to swaddle and rock the crying infant may also be helpful in order to decrease the stress from caring for a colicky infant.

Approach to umbilical vein catheterization

- Clean the cord and abdomen with chlorhexidine. - Prepare your umbilical line by flushing it with normal saline, and apply a 3 way stopcock (to prevent air embolization). - Tie umbilical tape loosely around the base of the umbilicus. (An alternative to this step would be to place a purse string suture around the base of the cord.) - Holding the cord clamp, use a scalpel to cut perpendicularly through the umbilicus directly below the clamp or about 2cm from the abdominal wall. - Gently grasp the edge of the cord with the forceps. Identify the larger, single, thin walled umbilical vein (as opposed to the smaller, double, thick walled umbilical arteries). - Gently remove any visible clots at the meatus of the vein. - Gently advance the catheter into the vein. In an emergency setting, only advance to the minimum depth required to get free flowing blood, or approximately 3-5cm. - Aspirate blood, then flush the line. - Tighten the umbilical tape to temporarily secure the line.

How to typically prescribe albuterol inhalers so can monitor symptoms too...

18g inhaler each --> prescribe 36g with no refills (x2) - call when out vs urgent care if symptoms so can reassess - approach: 2 inhalers with no refills - if goes through 2 inhalers fast, probably needs to be on controller - 200 puffs/inhaler - 1 inhaler for school vs 1 for home

Caloric Expenditure Method: 10 year old boy with injuries and fever, 30 kg ....

= REE + REE x (Mtn + Activ + Fever + Inj + Growth) = 40 + 40 x(0.2 + 0.1 + 0.13 + 0.4 + 0.5) = 40 + 40 x(1.33) = 93 kcal/kg/d = 2790 kcal/d Therefore, he needs 2790 cc water per day water needs parallel caloric needs 3 MEq Na/(100 kcals) = 84 MEq Na total per day 2 MEq K/(100 kcals) = 56 MEq K total per day

Gender dysphoria is defined as a marked difference between an individual's experienced gender and his or her natal sex. The difference must be present for at least ...

6 months and cause clinically significant distress - The Endocrine Society clinical practice guideline for the treatment of transsexual persons recommends that the suppression of pubertal hormones start when the adolescent first exhibits physical changes of puberty, followed by initiation of cross-sex steroids for pubertal development of the desired opposite sex at approximately 16 years of age

Routine surveillance for Beckwith-Wiedemann syndrome

Because of the risk for embryonal tumors, routine surveillance is recommended. - The highest risk is in young children through age 8 years --> abdominal ultrasonography is recommended every 3 months until age 8 years. - Serum α-fetoprotein concentration should be monitored every 2 to 3 months until age 4 years for early detection of hepatoblastoma. The estimated tumor risk for children with BWS is 7.5%. - Between age 8 years and mid-adolescence, annual renal ultrasonography is recommended to assess for renal disease; measurement of urinary calcium-creatinine ratio should also be considered.

An 11-year-old girl presents after getting hit in the eye with a golf ball. She complains of right eye pain and decreased vision. Examination reveals moderate periorbital swelling, conjunctival hemorrhage, and a teardrop-shaped pupil. Visual acuity is 20/30 in the left eye, and she has light perception only in the right eye. Which of the following is the most appropriate management?

Eye shield, CT scan of the orbit, and emergent ophthalmology consultation ruptured globe - blunt trauma to the eye causes a sudden elevation in intraocular pressure, leading to scleral rupture. - ocular pain and decreased vision. - chemosis or subconjunctival hemorrhage overlying the scleral defect. - if rupture occurs at the limbus, an irregularly shaped (teardrop) pupil may be seen. - management of suspected globe rupture involves protection of the eye (optimally with a curved eye shield that does not touch the globe), CT scan of the orbit, 30-degree head-of-bed elevation, and emergent ophthalmology consultation. - broad-spectrum intravenous antibiotics should be administered, as well, to reduce the development of endophthalmitis.

A 2 wk old infant girl has bilateral swelling of the breasts, with a milky discharge from one nipple. The best course of action is:

Reassure the parents that the swelling is due to maternal estrogen and should soon subside Neonatal breast hypertrophy - quite common in term infants and can occur as a result of elevated circulating maternal endogenous steroid hormones in late gestation - as maternal estrogen levels fall, prolactin levels can increase and the breasts can produce a clear or cloudy (milklike) nipple discharge ("witch's milk") - repeated manipulation of the breast can exacerbate the condition - on occasion, the hypertrophy is associated with mastitis caused by a staphylococcal or streptococcal infection; antibiotics should be administered

A 6 wk old infant presents with bilious emesis and has a nonspecific KUB. The most appropriate diagnostic test is:

Upper GI (barium swallow) with small bowel follow through - An acute presentation of small bowel obstruction in a patient without previous bowel surgery is usually a result of volvulus associated with malrotation. - Upper gastrointestinal series is the imaging test of choice and the gold standard in the evaluation and diagnosis of malrotation and volvulus. It is the best exam to visualize the malposition of the ligament of Treitz and can also reveal a corkscrew appearance of the small bowel or a duodenal obstruction with a "bird's beak" appearance of the duodenum. - A superior mesenteric vein located to the left of the superior mesenteric artery suggests malrotation. - Malrotation with volvulus is suggested by duodenal obstruction, thickened bowel loops to the right of the spine, and free peritoneal fluid.

A full-term infant boy was delivered at term to a human immunodeficiency virus (HIV)-positive woman who received no prenatal care and was not on antiretroviral medication. What is the recommended prophylaxis for this infant?

Zidovudine for six weeks and three doses of nevirapine - All HIV-exposed infants should receive zidovudine prophylaxis, preferably within six to 12 hours of delivery. - Additionally, newborns at high risk of perinatal infection, such as those whose mothers did not receive antiretroviral drugs during pregnancy, should be given three oral doses of nevirapine. - Therefore, zidovudine for six weeks and three doses of nevirapine would be the appropriate choice for this patient.

Fractional excretion of sodium above 2% is consistent with ...

intrinsic renal injury

A 9 yr old child is found to have a positive PPD test result on routine screening. There are no symptoms. Laboratory tests, including chest film, are normal. Which of the following is the best interpretation of this finding?

mycobacterium tuberculosis infection - Latent tuberculosis infection (LTBI) occurs after the inhalation of infective droplet nuclei containing M. tuberculosis. - A reactive tuberculin skin test (TST) and the absence of clinical and radiographic manifestations are the hallmark of this stage.

A 2-year-old girl with a history of chronic neutropenia and a chief concern of fever of 41°C is being evaluated at the office. The parents ask what the best method of measuring her temperature is. Of the following, the PREFERRED method of thermometry in this girl is

oral - The girl in the vignette has a fever in the setting of neutropenia, which is generally defined as an absolute neutrophil count of less than 1,500 cells/μL. - The preferred method of thermometry in neutropenic patients is oral, though axillary measurement is acceptable if a neutropenic patient is unable to tolerate an oral temperature.

Hyperglycemic hyperosmolar state is associated with

severe hyperglycemia hyperosmolality dehydration mental status change without significant ketosis or acidosis - associated with type 2 diabetes and antipsychotic medications (eg, risperidone)

An 18 mo old manifests pendular nystagmus, head nodding, and torticollis. Findings on cranial MRI are normal. This child is most likely to have:

spasmus nutans - special type of acquired nystagmus in childhood. In its complete form, it is characterized by the triad of pendular nystagmus, head nodding, and torticollis - nystagmus is characteristically very fine, very rapid, horizontal, and pendular; it is often asymmetric, sometimes unilateral - signs usually develop within the 1st 1 or 2 yr of life. Components of the triad can develop at various times. - in many cases, the condition is benign and self-limited, usually lasting a few months, sometimes years - some children exhibiting signs resembling those of spasmus nutans have underlying brain tumors, particularly hypothalamic and chiasmal optic gliomas

A 6-month-old infant presents to the emergency department with his mother for lethargy. She reports that the baby has been unusually sleepy and feeding poorly over the past few days. Today, the mother noticed that she had a very weak cry and that she hardly ate anything. The mother suggests that the baby may have caught a cold that has been going around the house. Birth history and medical history are unremarkable. The infant missed her most recent six month well child visit with the pediatrician but she reports that the baby has been developing normally. When the mother is at work, her boyfriend takes care of the baby at home. On examination, the child is lethargic with vital signs within normal limits for age. You note a bluish gray patch on the child's right lower extremity and buttocks, as well as a 2 cm circular burn on the left arm. Which of the following is the most appropriate next step in the evaluation of this infant?

*Order computed tomography scan of the brain* - Given the clinical history and initial presentation of the child in the emergency department, the best initial evaluation is to order computed tomography scan of the brain. Given the child's altered mental status, there is a concern for an intracranial hemorrhage, and further work up takes top priority. - Identification of child abuse is of utmost importance to the medical practitioner, both in order to appropriately treat the acute trauma as well as to initiate appropriate actions to protect the child from further abuse. In many cases, suspicions of abuse are based on physical exam.

A male neonate is born at 37 weeks gestation to a G1P1 woman via induced vaginal delivery secondary to concerning fetal heart tracings. Delivery is uneventful, with no evidence of meconium. The neonate is initially noted to be tachypneic and cyanotic, with subsequent development of significant respiratory distress. Apgar scores at 1 and 5 minutes are 4 and 6. Oxygen therapy and bag-mask ventilation are initiated, and the neonate is subsequently intubated and transferred to the neonatal intensive care unit. Pulse oximetry reveals an oxygen saturation of 96% in the right thumb and 85% in the right great toe. The neonate's vital signs include a T of 37.2°C, HR 190 beats/min, and RR 68 breaths/min. On physical exam, the baby is notably cyanotic, lung sounds are clear bilaterally, and a continuous machine-like murmur can be heard. A complete blood cell count with differential is unremarkable, and a blood culture is pending. Plain radiograph of the chest is obtained and appears normal with a normal-sized heart border. Echocardiography reveals normal structural cardiac anatomy with flattening of the ventricular septum. Doppler evaluation shows shunting from right to left through the ductus arteriosus. There is no impairment of the ventricles identified. Which of the following is the best initial treatment for this patient?

*Oxygen at 100% concentration* - Persistent pulmonary hypertension of the newborn most often affects infants ≥ 34 weeks gestation. Persistent pulmonary hypertension of the newborn typically occurs within the first 24 hours of life and should be suspected in all term infants with cyanosis without known risk factors. Cyanosis and respiratory distress are the most common presenting findings. These infants will usually have low Apgar scores and require interventions after delivery. Most often, there will be concomitant problems such as infection, respiratory distress syndrome, meconium aspiration, or congenital diaphragmatic hernia, however, persistent pulmonary hypertension of the newborn can be idiopathic. - In persistent pulmonary hypertension of the newborn, the expected fall in pulmonary vascular resistance, which normally happens during the transition to extrauterine life, fails to occur. This leads to persistent shunting of blood from right to left across the ductus arteriosus or foramen ovale in response to the high pulmonary vascular resistance and subsequent marked cyanosis and hypoxemia seen on evaluation. Initial evaluation generally includes evaluation for concomitant problems (infection, respiratory distress syndrome, etc.), as well as pulse oximetry screening, arterial blood gas monitoring, and chest radiography. - Initially, pulmonary vasodilation should be attempted with administration of oxygen at 100% concentration. This is a temporary measure, as prolonged exposure to hyperoxemia can be harmful to the infant, and generally, oxygen therapy is subsequently titrated to target a preductal saturation of > 90%.

3 day old boy in newborn nursery evaluated for emesis. He vomited after his last 2 feeds and second episode consisted of greenish liquid. Prior, pt had fed slowly but was tolerating formula. He has voided several times but has not yet had a bowel movement. Pt was born at 40 wks gestation by repeat c-section. There were no complications during pregnancy or delivery. Temp is 97 F, pulse is 124/min, and respirations are 22/min. Pt is awake but appears irritable and unconfortable. Anterior fontanelle open and flat, nares patent, no cleft lip or palate, and oral mucosa pmoist. S1 and S2 normal without murmurs. Lungs clear to auscultation bilaterally. Abdomen is distended and tense with hypoactive bowel sounds and no organomegaly. Rectal exam normal ROM of all extremities and no peripheral edema. No rash or jaundice present. What studies will most accurately yield diagnosis of this pt?

*Suction biopsy* Most likely Hirschsprung disease/congenital aganglionic megacolon - gold standard for diagnosis = rectal suction biopsy

A 37-year-old male presents with lower extremity numbness, weakness, and sexual dysfunction. His symptoms began 2 days prior, and has progressively worsened. Medical history is significant for pneumonia caused by Mycoplasma pneumoniae. He denies weight loss, night sweats, or recent vaccinations. Physical exam is notable for bilateral lower extremity sensory deficits and motor weakness. Both bulbocavernous and anal wink reflex are absent. MRI of the brain and spine are performed. There is no intracranial pathology. There is a gadolinium enhancing lesion on greater than two spinal cord levels in the lumbar spine. A lumbar puncture is performed, which shows cerebrospinal fluid pleocytosis, and an increased IgG index.

*transverse myelitis* When an acute myelopathy is suspected in a patient, the next diagnostic step after the history and physical is to evaluate for a compressive or structural etiology with an MRI scan. Transverse myelitis is a neurological disorder caused by inflammation of the spinal cord. Spinal cord dysfunction can occur over hours to weeks with this condition. Although there can be a wide spectrum of findings depending on the affected cord levels, there are four classic features: (1) weakness of the legs and arms, (2) pain (usually in the back), (3) sensory alterations, and (4) bowel and bladder dysfunction. Transverse myelitis - Motor symptoms - typically a rapidly progressive paresis - Sensory level - e.g., pain, dysesthesia, and paresthesia - Autonomic manifestations - e.g., bowel and bladder problems and sexual dysfunction

Radiography for genu valgum is indicated for children:

- Older than age 7 years with intermalleolar distances greater than 8 cm - With intermalleolar measurements outside 2 standard deviations from the mean for age - With other indicators described earlier that increase the risk of associated pathology

Severe persistent asthma

- Symptoms throughout the day - Nighttimes awakenings every night - Use a SABA multiple times per day - Have extreme interference with normal activity - Have FEV1 <60%

The FDA has given emergency use authorization for monoclonal antibody therapy to treat mild to moderate COVID symptoms in some adults and adolescents. Monoclonal antibodies, given through an IV, may help block the SARS-CoV-2 virus from entering cells and make it easier for the body to destroy. To be considered for monoclonal antibody therapy, adolescents must be

- age 12 or older and weigh at least 88 pounds - have had symptoms of SARS-CoV-2 infection and a positive COVID test for less than 10 days - be at high risk of getting very sick from COVID-19, but not yet hospitalized

An 18-month-old boy is brought to the emergency department by ambulance after being rescued from a house fire. He is brought to the resuscitation room and attached to a cardiorespiratory monitor. Intravenous access is obtained. His airway is secured via endotracheal intubation, and a normal saline bolus is started. His entire chest, entire abdomen, entire right arm, and anterior aspects of both legs sustained deep partial-thickness burns. His electronic medical records show that his weight was 10 kg at a health maintenance visit 1 week ago. Of the following, the amount of fluid this child requires over the first 24 hours is

2600 ml The severity of the burns necessitates intravenous fluid replacement. To calculate the amount of fluid needed in the first 24 hours, one must consider both the maintenance fluid requirements and the fluid deficit. The entire fluid deficit is typically replaced in the first 24 hours after the burn. One of the more commonly used calculations to determine the fluid deficit is the Parkland formula: Fluid deficit = 4 mL/kg × weight (kg) × body surface area affected (%) The rule of nines divides the body into multiples of 9% as follows: Abdomen/chest and back, 18% each Anterior and posterior lower extremities, 9% each Anterior and posterior upper extremities, 4.5% each Anterior and posterior head, 4.5% each Genitals, 1% The rule of nines is modified slightly in children to account for the relatively larger head. For the child in the vignette, the percent BSA affected is approximately 40%. Thus, fluid deficit can be calculated from the Parkland formula as follows: Fluid deficit = 4 mL/kg × 10 kg × 40 (% BSA affected) = 1,600 mL The maintenance fluid required for a 10-kg child over a 24-hour period calculated by the Holliday-Segar method is 1,000 mL; thus, the total fluid requirement for the first 24 hours is 2,600 mL. Half of the fluid is administered in the first 8 hours and the remainder over the following 16 hours.

Age: kicks ball, walks up steps

24 mos

In infectious mononucleosis, no return to sport for how long?

3 to 4 weeks after symptom onset - return to contact/collision activity not advised for at least 4 weeks after symptom onset

Suture choice for trunk

3-0, 4-0 - remove after 10-14 days

A 10-year-old girl has had a chronic wet cough, present day and night, for 6 months. She has no identified triggers. It is not exacerbated by exercise and does not limit her in any way. There is no seasonal pattern and no tobacco smoke or other irritant exposure. There is a pet dog in the home. Review of systems finds nasal congestion without rhinorrhea. The family history is negative for asthma, allergies, and immunodeficiency. Physical examination shows mildly edematous nasal mucosa without discharge. The oropharynx and chest wall anatomy are normal. Breath sounds are somewhat coarse with a few scattered rhonchi and no fine rales or wheezes. Spirometry findings are normal. Chest radiography demonstrates a mild increase in central peribronchial markings with no other abnormalities. Of the following, the BEST next management step for this patient is

3-week course of amoxicillin/clavulanic acid - Chronic cough is a cough that has been present for at least 4 continuous weeks. - Specific cough pointers (signs and/or symptoms) are findings from the history or physical examination that suggest a specific etiology for the cough and help direct management. - Nonspecific cough has no findings on history or physical examination that suggest an etiology. - Appropriate evaluation includes chest radiography and pulmonary function testing (in children >6 years old); no additional testing is needed unless an abnormality is suggested by the results of these tests.

In suspected septic shock, immediately following establishment of intravenous (IV) or intraosseous (IO) access, aggressive, early goal-directed therapy (EGDT) should be initiated unless there are significant concerns for cardiogenic shock as an underlying pathophysiology. Rapid IV administration of 20 mL/kg isotonic saline should be initiated in an attempt to reverse the shock state. This bolus should be repeated quickly up to ...

60-80 mL/kg; - not unusual for severely affected patients to require this volume within the first hour - fluid resuscitation in the first hour is unquestionably essential to survival in septic shock, regardless of the fluid type administered.

Obese BMI for kid

95-99% - morbid (severe) obesity BMI = >99%

A 15-year-old girl presents to the office with complaints of abdominal cramping, diarrhea, and nausea after food intake for the last four months. She has a 10-pound unintentional weight loss over the last six months. Blood work is done, and labs are consistent with iron deficiency anemia and immunoglobulin A antibody against tissue transglutaminase. An upper gastrointestinal endoscopy is performed, and a biopsy is taken from the small intestine. Histology shows loss of villi, mucosal atrophy, and crypt hyperplasia. What human leukocyte antigen (HLA) type is commonly associated with her diagnosis?

HLA-DR4-DQ8 Celiac disease - majority of patients with celiac disease have a positive human leukocyte antigen (HLA) HLA-DR3-DQ2 or HLA-DR4-DQ8. This HLA association is seen in multiple family members with celiac disease. Additionally, these particular HLA alleles are associated with type I diabetes mellitus.

Characterized by palpable purpura, abdominal pain, and features of microscopic hematuria and minimal proteinuria suggesting glomerulonephritis

Henoch-Schönlein purpura (HSP) - mild --> tx = supportive care - In patients with Henoch-Schönlein purpura, isolated microscopic hematuria or non-nephrotic-range proteinuria is best managed with supportive care and weekly urinalysis - Indications to refer patients with Henoch-Schönlein purpura to a pediatric nephrologist include nephrotic syndrome, nephrotic-range proteinuria, hypertension, and acute renal insufficiency

A 12 yr old girl experiences acute monocular blindness of 2 days' duration. Past medical history reveals that she has had headaches for the past 3 yr that she cannot characterize, 1 brief episode of diplopia, and 1 episode of paresthesias of the feet. These episodes were not related in time, did not occur in immediate proximity to the headache, and resolved spontaneously. Findings on physical examination, including the funduscopic examination, are unremarkable other than reduced visual acuity. The most important diagnostic step is to perform:

MRI - Demyelinating disorders of the central nervous system (CNS) cause acute or relapsing-remitting encephalopathy and other multifocal signs of brain, brainstem, and spinal cord dysfunction. - Cranial MRI exhibits discrete T2 lesions in cerebral white matter, particularly periventricular regions as well as brainstem, cerebellum, and juxtacortical and deep gray matter. Alternatively, tumefactive T2 lesions are also seen. Spine MRI typically shows partial-width cord lesions restricted to 1-2 spine segments.

Greatest risk factor for RDS

low GA

Development of male external genitalia is typically complete by which of the following gestational ages?

*12 weeks* - Fetuses in the first weeks of development have sexually indifferent reproductive organs. - Around six weeks gestation, sexual differentiation begins and genetically male fetuses begin to have testicular development. Male external genitalia are distinct around the ninth week of gestation. This process in males is dependent on sufficient testosterone production by the cells of Leydig. - By 12 weeks, external differentiation of male genitalia is complete. - Female external genitalia develop as the result of the absence of testosterone beginning around eight weeks of gestation. - Full development of the female sexual phenotype is typically completed after 12 weeks of gestation. This process is not androgen dependent. In fact, the presence of increased androgens in genotypic females can lead to virilization. Similarly, insensitivity to androgens in genotypic males may result in development of female genitalia.

Age? - sits momentarily propped on hands - transfers objects hand to hand - reduplicative babble with consonants

*6 mos* Also... - rakes pellet - feeds self crackers - places hands on bottle - touches reflection and vocalizes - stranger anxiety - stops momentarily to "no" - gestures for "up" - listens, vocalizes, when adults stops

APGAR: Pulse of 130, acrocyanotic, grimaces to stimulation, moving all extremities and crying. Score?

*8* 2 pts for pulse 1 for color 1 for irritability 2 for tone 2 for respiration General info about how the newborn tolerated labor (1min) and the newborn's response to resuscitation (5min)

Age? - pulls to stand - pincer grasp - separation anxiety - non-specific mama

*9 mos* - begins creeping - bangs two cubes together - bites, chews cookie - inspects bell - rings bell - orients to name - enjoys gesture game - non-reduplicative babble - imitates sounds

Which of the following geographic areas in the United States is endemic for Histoplasma capsulatum?

*Central United States* - Histoplasma capsulatum is endemic to the central states. - Blastomyces dermatitidis is endemic to the - Mississippi and Ohio River Valleys. - Coccidioides sp is a fungus found in the soil of southwestern states.

12 yo M + right leg pain immediately after he tripped during soccer game appx 1 hour ago. Ice applied on field with no relief, and pt has been unable to walk. Since early childhood, he has experienced "growing pains" in his legs that have frequently awakened him at night. Current discomfort is sign more severe and localized. Pt has been healthy and takes no meds. He is active and enjoys playing sports. Spleen palpable 5 cm below left costal margin. Hgb 10.5 and platelet 95,000. Point tenderness over right tibia proximally. Unable to bear weight on affected leg. X-ray = transverse proximal right tibial diaphyseal fracture. Metabolic disturbance?

*Glucocerebroside* - AR - lysosomal storage disorder - glucocerebrosidase def - glycolipids/glucocerbrosides accumulate within lysosomes of macrophages Type 1 - most common - anemia, thrombocytopenia, splenomegaly, bony pain, pathologic fractures

38 wks LGA infant born by C/S to an A2GDM has dyspnea/grunting ....

*TTN* pathophys = lung fluid not squeezed out / retained prognosis = usually minimal O2 needed. self-resolves in hours to days

Myotonic dystrophy worsens with each generation due to which of the following?

*anticipation* - Myotonic dystrophy is one disease that is inherited in a non-Mendelian pattern as the result of anticipation. In this situation, each generation exhibits earlier-onset or more severe disease. Diagnosis is often delayed until affected offspring exhibit significant symptoms. Then affected parents and grandparents, who have milder disease, may be identified. - In myotonic dystrophy, the CTG trinucleotide repeats grow in number with each subsequent generation, and each longer repeat is associated with a more severe phenotype. - Another mechanism for anticipation is seen in short telomere syndromes such as dyskeratosis congenita. In this group of diseases, the telomeres shorten with each generation, resulting in a progressively younger age of onset for each subsequent generation.

A patient successfully treated for acne vulgaris with systemic antibiotics now breaks out with facial papulopustular lesions that resist higher doses of antibiotic. What is the diagnosis?

*gram negative folliculitis* - Gram-negative folliculitis is generally seen following prolonged antibiotic treatment. - Isotretinoin is the recommended treatment. - Treatment with trimethoprim-sulfamethoxazole is an acceptable alternative. - The offending antibiotic should be discontinued. - Some experts also recommend benzyl peroxide washes.

A 36-month-old boy presents to clinic for routine well-child care. On review of history, his mother had verbalized concern at the last visit for his lack of speech expression. Screening for autism spectrum disorder was positive, and he was referred to a developmental specialist for comprehensive evaluation, which resulted in a diagnosis of autism spectrum disorder. Which treatment approach for autism has been shown to have the greatest long-term benefit?

*intensive behavior therapy* - Although many symptoms manifest by age 18 months, formal diagnosis is often delayed until after 3 years of age. - Once identified, intensive behavioral therapy is an essential treatment to implement for the long-term benefit of the child. The goal of behavioral therapy is to maximize functioning, move the patient toward independence, and improve the quality of life for the child. - Greater intensity and duration of therapy are associated with improved outcomes, and experts recommend at least 25 hours per week of intensive intervention.

Refer if vision worse than...

- 20/50 in 3 yo - 20/40 in 4 yo - 20/30 in >/=5 yo >/= 2 line difference in acuity between eyes

strep throat treatment

- Amoxicillin (best tasting): 50 mg/kg dose (max 1 g) daily x 10 days - Penicillin G benzathine IM x 1 - Consider first dose in ED + Rx note

failure to thrive defined as ...

- BMI for age < 5th percentile - length for age less than 5th percentile - weight deceleration crossing two major percentile lines - weight for age less than 5th percentile - weight less than 75 percent of median weight for age - weight less than 75 percent of median weight for length - weight velocity less than 5th percentile

If bedsharing is occurring with new infant, can provider info on safer ways to do so, including:

- NEVER with person who uses drugs/alcohol/tobacco - NEVER on water mattresses or couch - NEVER prone

Intermittent asthma

- Symptoms <3 days/week - Nighttimes awakenings less than 3 times per month - Uses a SABA <3 days/week - Has no interference with normal activity - Normal FEV1 function between exacerbations - Treat with SABA as needed

Mild persistent asthma

- Symptoms >2 days/week (but not daily) - Nighttime awakenings 3-4 times/month - Use a SABA >2 days/week (but not daily) - Has minor interference with normal activity - Has FEV1 >80% - Add a low-dose inhaled corticosteroid

Wide pulse pressure differential

- aortic insufficiency - arteriovenous fistula - patent ductus arteriosus - thyrotoxicosis - warm shock wide pulse pressure = >40 mmHg pulse pressure = systolic pressure - diastolic pressure

perinatal depression defined as...

- apgar <5 at 10 minutes or - need for resuscitation at 10 minutes (ETT or BM ventilation, or chest compressions), or - pH <7 in cord, arterial, or venous blood <60 min of age, or - base deficit >/= 15 in cord, arterial, or venous blood <60 minutes of age

Indications for endotracheal intubation at birth include any of the following for infants </= 27 wks gestation...

- apnea - need for FiO2 >/= 0.4 - arterial pH </= 7.25 - need to maintain airway - PaCO2 >/= 60 mmHg

The adolescent in the vignette has postconcussive syndrome, a neurocognitive disorder affecting approximately 10% to 15% of those with concussion. Symptom onset is within 4 weeks after a concussion and lasts a minimum of 3 months. Risk factors for the development of postconcussive syndrome include:

- pre-existing psychiatric disorders such as anxiety, depression, or poor coping skills - multiple prior concussions - history of migraine headaches - learning disorder - factors related to the concussion itself (severe initial - symptom burden or delayed symptom onset, loss of consciousness for >1 minute, vestibular symptoms)

Meconium plugs are associated with

- small left colon syndrome in infants of diabetic mothers and with cystic fibrosis - rectal aganglionosis - maternal opiate use - magnesium sulfate therapy for preeclampsia

Baby Harris has a central line and is receiving D17 at 115 ml/kg/day. What is his GIR?

0.17 (dextrose receiving) * 115 (volume) * 0.69 (constant) = 13.48 = 13.5 mg/kg/min

If you take 60 units daily, your I:C ratio would be

1 unit per 8 grams of carb (500 ÷ 60 ≅ 8)

5 causes of hypoxemia ...

1. V/Q mismatch 2. Hypoventilation 3. Right-to-Left Shunt 4. Thickened diffusion barrier 5. Low inspired ppO2 http://yanyu.ca/mednotes/Course%203/5MechanismsOfHypoxemia.pdf

A 5 yr old boy presents with pubic hair development. He is tall and has increased pigmentation of his genitalia and phallic enlargement. Blood pressure is 130/90 mm Hg. Measurement of which of the following hormones would be most likely to be diagnostic?

11-deoxycortisol 11β-Hydroxylase deficiency a - 5% of cases of adrenal hyperplasia - although cortisol is not synthesized efficiently, aldosterone synthetic capacity is normal, and some corticosterone is synthesized from progesterone by the intact aldosterone synthase enzyme - plasma levels of 11-deoxycortisol and deoxycorticosterone are elevated

At what age does laryngomalacia typically resolve?

12 to 18 months

holliday-segar method ex. 25 kg

1600 cc/d (1000 + 500 + 100) = 65 cc/h

Clinicians should screen for autism at ...

18 and 24 mos - or at any point when a caregiver or clinician has concern

Age? - 10-15 words - Can point to 3 body parts

18-months

In CA, kids should be rear-facing in car seat until they are...

2 year old or 40 lbs and 40 inches - as long as fit in car seat - can leave them rear-facing longer time if they won't

Car safety seats should stay rear facing until

2 years

EE (ethinyl estradiol) dose for PCOS-related acne

20-30 ug

While over forty syndromes with Robin sequence have been described, what are the two most commonly associated syndromes?

22q11.2 deletion syndrome Stickler syndrome

Refer infants who do not track well after...

3 months of age

Hip instability may be detected by the Barlow(back) and Ortolani(out) maneuvers until approximately

3 mos old - After 3 months of age, limited hip abduction in the supine position (less than 75 degrees) is the most reliable sign

When to give varicella post-exposure prophylaxis from exposure

3-5 days

Age: jumps up

30 mos

Age: points to 6 body parts

30 mos

Nasal CPAP in infants is administered between...

4 and 6 cmH2O

Suture choice for arms

4-0, 5-0 - remove after 7-10 days

Suture choice for scalp

4-0, 5-0 - remove after 7-10 days

Average length at birth is ...

50 cm - birth length goes up by 50% at age 1, doubles by age 4, triples by age 13

Intussusception most commonly occurs in what age range?

6-36 mos - most commonly seen in ileocolic region - 75% of cases are idiopathic

The risk of an autosomal recessive disease in the offspring of a consanguineous mating between 1st cousins is:

6-8% Consanguinity - existence of a relationship by a common ancestor and increases the chance that both parents carry a gene affected by an identical mutation that they inherited - risk of a genetic disorder for the offspring of a 1st-cousin marriage (6-8%) is about double the risk in the general population (3-4%)

ELBW infants:

<1000 g

Arterial oxygen content

= (hgb x 1.34 x SaO2) + (0.0031 x PaO2)

A previously healthy infant with a history of vomiting and diarrhea is brought to the ER by her parents. During your assessment, you find that the infant responds only to painful stimulation. The infant's RR is 40/min, and central pulses are rapid and weak. The infant has good bilateral breath sounds, cool extremities, and a capillary refill time of ore than 5 seconds. The infant's blood pressure is 85/65 mmHg, and glucose is 30 mg/dL (1.56 mmol/L). You administer 100% oxygen via facemsk and start an IV. Which treatment is the most appropriate for this infant?

Administer a bolus of isotonic crystalloid 20 ml/kg over 5-20 minutes, and also give D25W to 4 ml/kg IV

A 3 year old unresponsive, apneic child is brought to the ED. EMS personnel report that the child became unresponsive as they arrived at the hospital. The child is receiving CPR with bag-mask ventilation. The rhythm shown here is VFIB. A biphasic manual defibrilator is present. You quickly use the length-based resuscitation tape to estimate the approximate weight as 15 kg. Which therapy is most appropriate for this child at this time?

Attempt defibrillation at 30 J and then resume CPR, beginning with compressions

You are giving chest compressions for a child in a cardiac arrest. What is the proper depth of compressions for a child

Compress at least 1/3 the depth of the chest , about 2 inches (5cm)

A one-month-old boy is brought to clinic by his mother for a well child examination. He was born at 39 weeks to a 23-year-old G1, P1 mother via normal spontaneous vaginal delivery. There were no complications at delivery. His birth weight is 2600 grams. He has been exclusively breastfeeding, which has been going well. He has been gaining about 35 grams per day. Physical examination is normal. The mother notes that he has a bowel movement only once a week and is worried about constipation. Which of the following is an alarm sign that suggests an organic cause of constipation?

Constipation present from birth or early infancy - Organic causes are responsible for fewer than 5 percent of children with constipation but are more common among young infants and among infants and children presenting with atypical features or "alarm signs." - Alarm signs from the history that suggest the possibility of organic causes include acute signs such as delayed passage of meconium (first meconium passed after 48 hours of life), fever, vomiting, or diarrhea, rectal bleeding (unless attributable to an anal fissure), and severe abdominal distension. - Chronic alarm signs include constipation that present from birth or early infancy, "ribbon" stools (very narrow in diameter), urinary incontinence or bladder disease, weight loss or poor weight gain, delayed growth, extraintestinal symptoms (especially neurologic deficits), congenital anomalies or syndromes associated with Hirschsprung disease (like Down syndrome) and family history of Hirschsprung disease.

A 7-year-old boy is being cared for in the intensive care unit after suffering a devastating neurological injury secondary to a motor vehicle collision. He is intubated and mechanically ventilated, however, he does breathe over the vent. He has no purposeful movements and does not respond to painful stimuli. He does not have a pupillary, gag, or corneal reflex. He has developed polyuria secondary to his neurologic injury and requires intravenous vasopressin to maintain normal sodium levels. Circulatory support is sustained with vasopressor support. Neurology, neurosurgery, nephrology, and the palliative care team are in agreement that life-sustaining measures at this time are futile and multiple multidisciplinary care meetings have been held regarding the possibility of withdrawing the life-sustaining support. However, the parents have refused each time and continue to request that everything that can be done continues to be done for him. What is the best next option in this situation?

Consult hospital ethics committee - Open communication with the child's family in a structured setting is of utmost importance to discuss goals of care and to manage expectations of outcomes. - Unfortunately, despite multiple family meetings, there remains a discrepancy between what the medical teams deem is appropriate medical therapy and what the parents' expectations are. In this situation, a reasonable next step would be to consult the hospital ethics committee, as it may be able to provide additional expert consultation on the situation.

A newborn infant fails to pass meconium for the first 48 hr. Abdominal distention and emesis have occurred overnight. The next diagnostic steps would include:

Contrast imaging of the lower gastrointestinal tract - When meconium ileus is suspected, a nasogastric tube is placed for suction and the newborn is hydrated. - In many cases, diatrizoate (Gastrografin) enemas with reflux of contrast material into the ileum not only confirm the diagnosis but have also resulted in the passage of a meconium plug and clearing of the obstruction. - Children who are successfully treated generally have a prognosis similar to that of other patients with severe CF mutations. - Infants with meconium ileus should be treated as if they have CF until adequate diagnostic testing can be carried out.

A previously healthy 6-year-old girl presents for evaluation of focal swelling on the left anterior neck that developed one week prior to presentation. The child developed fever four days prior to presentation, and in that time, the swollen area has become red, tender, and progressively larger. On further questioning, there is a history of an upper respiratory illness that preceded the onset of the swelling, but these symptoms seem to have resolved. Examination is remarkable for a nontoxic-appearing child with a fever of 39°C and a tender area of localized swelling deep to the left sternocleidomastoid. There is overlying erythema, and the area has peripheral induration and central fluctuance. There is limitation in active side-bending to the left due to associated pain. The remainder of the exam is within expected limits. The patient is started on a trial of ampicillin-sulbactam, but there is minimal improvement after 48 hours of therapy. Which of the following is the most appropriate next step in evaluation?

Contrast-enhanced CT of the neck suppurative cervical lymphadenitis - bacterial superinfection of a reactive lymph node, and a trial of empiric antibiotic management is reasonable. - Most cases are attributed to Staphylococcus aureus or Streptococcus pyogenes, and treatment should be directed to coverage of these organisms, typically with either clindamycin or ampicillin-sulbactam. R - mass's proximity to the sternocleidomastoid should prompt consideration of a contrast-enhanced CT scan of the neck for more detailed anatomic evaluation.

high blood sugar correction dose

Difference between actual blood sugar and target blood sugar÷ correction factor - Actual blood sugar minus target blood sugar

Pediarix

Diphtheria and tetanus toxoids and acellular pertussis (DTaP), HepB (Engerix-B 10 mcg/mL), and inactivated poliovirus vaccine (IPV) - typically administered at two, four, and six months of age - DTaP-HepB-IPV combination vaccine should not be administered before six weeks or after seven years of age.

A 4 yr old boy presents with sore throat and fever of sudden onset. He has difficulty swallowing and his breathing is labored. He is drooling and sitting upright and leaning forward in a tripod position. What is the appropriate next step in patient management?

Direct laryngoscopy in the operating room Epiglottitis - acute rapidly progressive and potentially fulminating course of high fever, sore throat, dyspnea, and rapidly progressing respiratory obstruction - respiratory distress can also be the 1st manifestation - the otherwise healthy child suddenly develops a sore throat and fever - within a matter of hours, the patient appears toxic, swallowing is difficult, and breathing is labored - drooling is usually present and the neck is hyperextended in an attempt to maintain the airway - child may assume the tripod position, sitting upright and leaning forward with the chin up and mouth open while bracing on the arms - diagnosis requires visualization of a large, cherry-red, swollen epiglottis by laryngoscopy - occasionally, the other supraglottic structures, especially the aryepiglottic folds, are more involved than the epiglottis itself - in a patient in whom the diagnosis is certain or probable based on clinical grounds, laryngoscopy should be performed expeditiously in a controlled environment such as an operating room or intensive care unit. - if epiglottitis is thought to be possible but not certain in a patient with acute upper airway obstruction, the patient can undergo lateral radiographs of the upper airway first

The X-linked lymphoproliferative (XLP) syndrome is classically associated with overwhelming infection by which of the following agents?

EBV Xlinked lymphoproliferative (XLP) disease - Xlinked recessive trait characterized by an inadequate immune response to infection with EpsteinBarr virus (EBV) - Affected males are usually healthy until they acquire EBV infection - The mean age of presentation is < 5 yr - There are 3 major clinical phenotypes: (1) fulminant, often fatal, infectious mononucleosis (50% of cases); (2) lymphomas, predominantly involving Blineage cells (25%); or (3) acquired hypogammaglobulinemia (25%) - There is a marked impairment in production of antibodies to the EBV nuclear antigen (EBNA), whereas titers of antibodies to the viral capsid antigen (VCA) have ranged from absent to markedly elevated - XLP has an unfavorable prognosis; 70% of affected boys die by age 10 yr

Your patient has an albumin of 2.0. How would you expect this to affect your calculation of her anion gap?

Falsely decreases the anion gap - Hypoalbuminemia leads to a false decrease in the anion gap. This is because albumin is negatively charged and when it is decreased, other negative anions such as Cl and HCO3 have to increase to maintain electroneutrality. - As both Cl and HCO3 are included in the AG calculation, this would lead to a decrease in your anion gap, even if a significant anion gap acidosis (ie lactic acidosis or DKA) is occurring. - Hence, the AG should be corrected in the setting of significant hypoalbuminemia. AGcorr= AG + 2.5(4-pt's albumin g/dl)

Examples of hypotonic fluids

Fluids with a lower osmotic pressure than blood: dextrose 5% in 0.45% sodium chloride [*D5 1/2NS*] dextrose 5% in 0.225% sodium chloride [*D5 ¼ NS*]

What is flonase?

Fluticasone furoate - synthetic corticosteroid derived from fluticasone - marketed by GlaxoSmithKline as Veramyst and Flonase Sensimist - approved for use in the United States by the Food and Drug Administration for long-term maintenance treatment of airflow obstruction in patients with COPD, including chronic bronchitis and emphysema - as of 2015, it is also approved for the treatment of *asthma* - point up and out!! towards turbinates

Modified Centor (McIsaac) Criteria and Decision to Test

For GAS - Fever (T > 38) = 1 - Absence of cough = 1 - Swollen, tender anterior cervical nodes = 1 - Tonsillar swelling or exudate = 1 - Age 3-14 yo = 1 - Age >/= 15 = 0

In which situations are tuberculosis blood tests preferred methods of testing for tuberculosis?

For individuals age 2 and older who have received the bacille Calmette-Guerin vaccine Those who will have difficulty returning to get the tuberculin skin test read after its placement

What causative organism should be suspected in lymphadenitis in a neonate?

GBS

In our current TPN ordering in the NICU, you order in g/kd/day, not mg/kg/min. You can calculate this one of two ways:

GIR x 1.44 or (dextrose as decimal) x (ml/kg/day) - Be very careful. - We are all taught to think in mg/kg/min, but the TPN is ordered in g/kg/day and that has the potential for error.

Can distinguish medications that may cause false-positive tox screen

Gas chromatography or gas mass spectroscopy

A full-term newborn infant with birth weight of 4.5 kg has a normal Apgar score at birth. On the second day of life, she has a seizure and her blood glucose level is 18 mg/dL. The most appropriate next step in management is to:

Give a mini-bolus of D10W plus continuous intravenous glucose infusions - Treatment of acute symptomatic neonatal or infant hypoglycemia includes intravenous administration of 2 mL/kg of D10W, followed by a continuous infusion of glucose at 6-8 mg/kg/min, adjusting the rate to maintain blood glucose levels in the normal range. - If hypoglycemic seizures are present, some recommend a 4-mL/kg bolus of D10W.

What is the definition of abnormally high height velocity in a 3-year-old?

Greater than 3.5 inches per year

To be diagnosed with mild persistent asthma or intermittent asthma, a patient requires what peak expiratory flow or forced expiratory volume in one second when not having an exacerbation?

Greater than 80% of predicted values.

HEADSSS Assement

HOME Education/Employment/Eating Activities Drugs Sexuality Suicide/Depression Safety

Imaging for kid with.... chronic interstitial process

HRCT

EKG right axis deviation

Hand's shake = right axis deviation look at leads I & 3

Which of the following organisms is the most likely cause of fungal pneumonia in immunocompetent persons living in the Ohio and Mississippi River valleys?

Histoplasma capsulatum - saprophytic form of Histoplasma capsulatum is found in soil throughout the midwestern USA, primarily along the Ohio and Mississippi rivers - acute pulmonary histoplasmosis follows initial or recurrent respiratory exposure to microconidia - median incubation time is 14 days - prodrome is not specific and usually consists of flulike symptoms including headache, fever, chest pain, cough, and myalgias

Topical cocaine is used to confirm a diagnosis of what ophthalmologic disease?

Horner syndrome - can be confirmed with the use of topical cocaine or apraclonidine drops - normal pupil dilates within 20-45 min after instillation of 1 or 2 drops of 4% cocaine, whereas the miotic pupil of an oculosympathetic paresis dilates poorly, if at all, with cocaine - should be used in caution in young children because it can cause excessive sedation owing to its CNS side effects

The cell receptor for most rhinoviruses is:

ICAM-1 - The majority of human rhinoviruses infect respiratory epithelial cells via intercellular adhesion molecule-1 (ICAM-1), but some rhinovirus strains utilize the LDL (low-density lipoprotein) receptor. - Infection begins in the nasopharynx and spreads to the nasal mucosa and in some cases to bronchial epithelial cells in the lower airway.

You are preparing to use a manual defibrillator in the pediatric setting. Which best describes when it is appropriate to use the smaller, pediatric-sized paddles?

If the child weighs less than 10 kg or is less than 1 year old

A newborn infant spends about 40 min with the mother but then falls asleep and does not respond to the mother's voice. Which of the following statements regarding this lack of activity is true?

It is normal - The initial period of social interaction, usually lasting about 40 min, is followed by a period of somnolence. - After that, briefer periods of alertness or excitation alternate with sleep. - If a mother misses her baby's first alert-awake period, she may not experience as long a period of social interaction for several days.

Most common anterior mediastinal mass in children

Lymphoma

Imaging for kid with.... posterior mediastinal mass

MRI

Imaging for kid with.... visualized vascular rings

MRI

A 15 yr old boy is struck by a car while walking. On arrival in the emergency department, he is alert and in respiratory distress with no signs of upper airway obstruction. Pulse is 140/min; respiratory rate, 40 breaths/min; and blood pressure, 70/50 mm Hg. Heart sounds are distinct, but breath sounds are decreased in the left hemithorax. What is the most appropriate next step in patient management?

Needle thoracentesis of the left hemithorax - Tension pneumothorax occurs when air accumulates under pressure in the pleural space. The adjacent lung is compacted, the mediastinum is pushed toward the opposite hemithorax, and the heart, great vessels, and contralateral lung are compressed or kinked. Both ventilation and cardiac output are impaired. - Characteristic findings include cyanosis, tachypnea, retractions, asymmetrical chest rise, contralateral tracheal deviation, diminished breath sounds on the ipsilateral (more than contralateral) side, and signs of shock. - Needle thoracentesis, followed by thoracostomy tube insertion, is diagnostic and lifesaving.

The most common cause of bacterial meningitis in children 1 mo-12 yr of age in the USA is

Neisseria meningitidis

Formula for infants <37 wks

Neo 22

A previously healthy 6 mo old child who just completed antibiotic treatment for acute otitis media and now is healthy and asymptomatic undergoes a stool culture to check for antibiotic-associated diarrhea. The stool culture grows C. difficile. The preferred antibiotic treatment is:

None of the above; antibiotic treatment is not indicated - Initial treatment of CDI involves discontinuation of any nonvital antibiotic therapy and administration of fluid/electrolyte replacement. - Infants are commonly colonized with C. difficile but do not develop colitis and do not require treatment.

For herpetic gingivostomatitis, what is the pediatric treatment/dosing?

Oral acyclovir = 15 mg/kg/dose 5 times a day PO for 7 days, maximum 1 g/day = Started within 72 hr of onset reduces the severity and duration of the illness - Pain associated with swallowing may limit oral intake of infants and children, putting them at risk for dehydration. - Intake should be encouraged through the use of cold beverages, ice cream, and yogurt.

A 10 yr old boy sustained 30% BSA burns and had been requiring dressing changes for physical therapy. Which of the following regimens will provide the best pain management?

Oral morphine and oral lorazepam - From the onset of treatment, preemptive pain control during dressing changes is of paramount importance. - Opiate analgesia, prescribed in an adequate dose and timed to cover dressing changes, is essential to comfort management. - Anxiolytic medication added to the analgesic is usually helpful and has more than a synergistic effect.

Most children with Clostridium difficile disease have mild to moderate illness characterized by watery diarrhea, low-grade fever, and mild abdominal discomfort. Severe and complicated C difficile disease includes cases with hypotension or shock, pseudomembranous colitis on endoscopy, ileus, or toxic megacolon. What is the recommended treatment for an initial episode of severe C difficile colitis?

Oral vancomycin (40 mg/kg per day) every 6 hours for 10 days

What is synagis?

Palivizumab - brand name Synagis - monoclonal antibody produced by recombinant DNA technology - used in the prevention of respiratory syncytial virus (RSV) infections - recommended for infants that are high-risk because of prematurity or other medical problems such as congenital heart disease - humanized monoclonal antibody (IgG) directed against an epitope in the A antigenic site of the F protein of RSV - dosed once a month via intramuscular (IM) injection, to be administered throughout the duration of the RSV season - targets the fusion protein of RSV, inhibiting its entry into the cell and thereby preventing infection - was approved for medical use in 1998

A 10 yr old boy has been diagnosed with sarcoidosis. Which of the following studies should be performed to monitor for irreversible damage from sarcoidosis?

Slit lamp examination of the eyes now and on a yearly basis - Serial pulmonary function tests and chest radiographs are useful in following the course of lung involvement. - Monitoring for other organ involvement should also include electrocardiogram with consideration of an echocardiogram, urinalysis, renal function tests, and measurements of hepatic enzymes and serum calcium. - Other potential indicators of disease activity include inflammatory markers and serum ACE, although changes in ACE level do not always correlate with other indicators of disease status. - Given the frequency of asymptomatic eye disease and the ocular morbidity (loss of vision) associated with pediatric sarcoidosis, all patients should have an ophthalmologic examination at presentation with monitoring at regular intervals, perhaps every 3-6 mo as recommended in children with juvenile idiopathic arthritis.

Intraoperative steroid dosing...

Solumedrol IV 20 mg/kg Immediately thereafter = taper starting with 1 mg/kg IV BID x2 days - then continued taper every 2 days (then 0.8 mg/kg/dose then decrease by 0.1 mg/kg/dose q2days)

Murmur - systolic - LLSB - 3-6 yo - grade 2-3/6 - low-frequency vibratory

Still's murmur

The radiologist calls to report that a plain radiograph that you ordered to evaluate a hip click shows periostitis. This finding suggests congenital infection by which of the following agents?

Syphilis/Treponema pallidum - In congenital syphilis, bone involvement is common. - Roentgenographic abnormalities include Wimberger lines (metaphyseal demineralization of the medial aspect of the proximal tibia), multiple sites of osteochondritis at the wrists, elbows, ankles, and knees, and periostitis of the long bones and rarely the skull. - The osteochondritis is painful, often resulting in irritability and refusal to move the involved extremity (pseudoparalysis of Parrot).

Units of basal insulin per day =

TDD/2 - TDD (total daily dose) of insulin starting dose: prepubertal child = start at 0.5 units/kg/day; pubertal child = start at 0.7 units/kg/day - ICR (carb factor) = 500/TDD = g of CHO per 1 unit insulin

For, AHI/OSA in the mild range​, anti-inflammatory treatment can reduce adenotonsillar size and OSA symptoms:

Topical intranasal corticosteroids ​ - Flonase ​ ​ Leukotriene receptor antagonist ​ - Montelukast (Singulair) ​ - Elevated leukotriene receptors were found in tonsils of children with OSA​ - Leukotrienes are inflammatory mediators in the respiratory system ​ - Montelukast decreases the activation of the leukotriene pathway ​ - Black box warning for reports of mental health side effects; Depression, Suicide, tremors, and anxiety​​

Treatment of choice for tympanostomy tube otorrhea.

Topical ofloxacin

Lines UAC length (cm) = UVC length (cm) =

UAC length (cm) = [3 x wt (kg)] + 9 (Goal T7-T9) UVC length (cm) = ½ UAC length + 1 (Goal above diaphragm) **Always prescribe Nystatin 0.5 ml to each cheek q6 when your pt has any central line (UAC, UVC, PICC, Broviac) for fungal prophylaxis

Most common CHD lesion

VSDs - perimembranous VSDs = most common

Which intelligence and achievement tests (standardized on the same population) can be used together to reduce measurement error when evaluating for learning disability in a school-aged child?

Wechsler intelligence scale for children (WISC) and the Wechsler individual achievement test (WIAT).

Adolescent idiopathic scoliosis is characterized by lateral curvature of spine in otherwise healthy adolescent. Thoracic or lumbar prominence on forward bend test is indicative of scoliosis, and a scoliometer angle of rotation >/= 7 degree requires...

additionally eval with *x-ray of spine*

Infant has evidence of heart failure with tachypnea, poor feeding, sweating with feedings, tachycardia, irritability, a gallop, cool distal extremities, and hepatomegaly. This could present in heart failure, whether systolic dysfunction resulting from cardiomyopathy or pulmonary overcirculation resulting from an aortopulmonary window. The presence of the deep Q waves in the inferior leads on the electrocardiogram makes this presentation most consistent with

anomalous left coronary artery from the pulmonary artery (ALCAPA) - left coronary artery's arising from the pulmonary artery instead of from the aorta - pulmonary vascular resistance drops early in infancy, and in children with ALCAPA, this makes it easier for blood to bypass the aberrant coronary artery and go out to the pulmonary vasculature, resulting in ischemic injury to the myocardium and, ultimately, heart failure - ischemia results in the Q waves seen on electrocardiogram, which distinguishes ALCAPA, rather than the other potential causes of heart failure listed in the response choices, as the diagnosis in this case

Car seat rear facing until your baby is how old?

at least 2 years old (or highest weight/height allowable by the rear facing convertible car seat's manufacturer)

Given the benefits to most newborns and concordant with other professional organizations, the American College of Obstetricians and Gynecologists now recommends a delay in umbilical cord clamping in vigorous term and preterm infants for

at least 30-60 seconds after birth

Your patient is POD#0 s/p repair of Tetralogy of Fallot and now has developed a narrow complex tachycardia to 200 bpm with no clear p waves preceding the QRS on ECG monitoring. What might you see on the CVP waveform to support your suspected diagnosis?

cannon a waves - The patient described most likely has junctional ectopic tachycaria (JET), which commonly occurs after congenital heart repairs. - It presents as a narrow complex tachycardia with evidence of AV dissociation. Hence, the atria may contract against a closed tricuspid valve, leading to a prominent "a" wave known as a cannon "a" wave. - Treatment for JET includes cooling, reducing inotropes as able, magnesium, and sometimes arrhythmic such as amiodarone.

Used as initial eval of esophageal atresia

chest radiograph after orogastric tube placement

A 24-month-old boy in foster care is seen for a medical clearance examination. He is placed in foster care temporarily while his biological mother finds more stable housing, with the goal of reunification. His medical record is complete and shows that he has been healthy. The foster parents have no experience caring for a child with an uncircumcised penis. The parents have noted that the foreskin of their foster child cannot be completely retracted and would like to know how to care for him. Physical examination findings for the foster child are normal. His foreskin appears normal and can be retracted to the point of barely visualizing the urethral meatus. Of the following, the BEST advice for the foster parents is to

cleanse it without retraction -Phimosis is the inability to fully retract the uncircumcised penile foreskin beyond the head of the penis and can be physiologic or pathologic. - Physiologic phimosis is present in most males at birth and resolves gradually over time through desquamation. The debris formed during this process is referred to as smegma and may be mistaken for infection but requires no special treatment aside from routine cleansing. - Eighty to ninety percent of uncircumcised boys will have fully retractile foreskins by 3 years of age, and 99% will have fully retractile foreskins by adolescence. - Pathologic phimosis, however, may require treatment. It may be caused by recurrent inflammation, such as from forceful retraction, or infection, such as from balanitis. Medical treatment usually involves applying a topical steroid cream such as 0.05% betamethasone or 0.1% triamcinolone acetonide 2 or 3 times daily for 1 to 2 months along with gentle retraction. If medical therapy is unsuccessful, a urologist may be consulted. Surgery may be considered in severe cases. - Paraphimosis may be described as the opposite of phimosis in which the retracted foreskin cannot be reduced and becomes edematous and painful. This is an emergency that necessitates immediate urology referral to prevent ischemia.

The recommended treatment for cervical lymphadenitis caused by atypical mycobacteria is:

complete surgical excision alone - nodes should be removed while still firm and encapsulated - if there is concern for possible M. tuberculosis infection, therapy with isoniazid, rifampin, ethambutol, and pyrazinamide should be administered until cultures confirm the cause to be NTM

Maneuvers that can elicit costochondritis

crowing rooster horizontal arm traction

oxygen consumption (VO2)

delivery - excraction - via pulmonary artery catheter - true mixed venous sat via pulm artery cath - can get via neck line in right atrium - atrial septal defect = falsely high = red blood mixed = falsely elevated - most accurate = PA but don't do that bc don't float PA caths out there

The diagnosis of recent WNV infection is generally made by

detection of WNV IgM antibody in the CSF or blood - West Nile virus IgM antibody may be detected in the serum for more than 1 year after infection; however, detection of WNV IgM in the CSF is indicative of a recent central nervous system infection - West Nile virus IgM antibodies are detectable in the CSF by 3 to 5 days of onset of illness

The Barlow test is used to diagnose:

developmental dysplasia of hip

Acetaminophen is toxic at what doses?

doses of 150 mg/kg in a child 7.5 g for an adult - The ideal time to obtain a serum acetaminophen level to predict the risk of toxicity and guide management is 4 hours after an acute ingestion - N-acetyl cysteine is the antidote for acetaminophen toxicity; it is equally effective when given orally or intravenously

A 16-year-old adolescent boy is seen for a pre-travel evaluation. In June, he will travel to Nigeria for a mission trip that will last 4 weeks. He has a history of well-controlled asthma and anxiety. At today's visit, he would like to receive vaccinations pertinent for travel and has been advised by the agency organizing the trip to receive malaria prophylaxis. Of the following, the prophylaxis BEST suited for this adolescent is

doxycyline - In Africa and in Nigeria specifically, Plasmodium falciparum is the malaria species of concern. Because Nigeria has widespread chloroquine resistance and because mefloquine is contraindicated in patients with anxiety disorders, doxycycline is the most appropriate choice. - Common adverse effects of doxycycline include gastrointestinal upset and photosensitivity. - Doxycycline should not be used in children younger than 8 years or pregnant women.

Presentation (dysphagia with solids) in an older child, particularly with a history of atopy, is common for

eosinophilic esophagitis

You are called to help treat an infant with severe symptomatic bradycardia (HR 66/min) associated with respiratory distress. The bradycardia persists despite establishment of an effective airway, oxygenation, and ventilation. There is no heart block present. Which is the first drug you should administer?

epinephrine

A 17 yr old boy presents to the emergency department with difficulty breathing. He has had a sore throat and fever all week. Chest radiograph shows multiple nodules. Blood culture is most likely to grow:

fusobacterium necrophorum - uncommon but characteristic infection of the parapharyngeal space is Lemierre disease, in which infection from the oropharynx extends to cause septic thrombophlebitis of the internal jugular vein and embolic abscesses in the lungs - typical presentation is that of a previously healthy adolescent or young adult with a history of recent pharyngitis who becomes acutely ill with fever, hypoxia, tachypnea, and respiratory distress - chest radiography demonstrates multiple cavitary nodules, often bilateral and often accompanied by pleural effusion - blood culture may be positive - treatment involves prolonged intravenous antibiotic therapy with penicillin or cefoxitin; surgical drainage of extrapulmonary metastatic abscesses may be necessary

SIRS in kids

heart rate >2 SD from normal - infants = low HR temp <36 or >38 WBC abnormal tachypneic / RR - 2 SD

Spinal muscular atrophy presents with

hypotonia, weakness, areflexia, and tongue fasciculations in infants and children

For stridor in infant (usually due to laryngeal edema), give...

inhalations of the vasoconstrictor, racemic epinephrine 2.25% (0.2 - 0.25 mL)

Medication of choice for all individuals suffering persistent asthma

inhaled corticosteroids - most effective anti-inflammatory medication for asthma

Most common types of CHD that cause shock are...

left-sided obstructive lesions

The disease associated most frequently with atypical mycobacteria in children is:

lymphadenitis - of the superior anterior cervical or submandibular lymph nodes is the most common manifestation of nontuberculous mycobacteria (NTM) infection in children - most common in children 1-5 yr of age and has been related to their tendency to put objects contaminated with soil, dust, or standing water into their mouths

Previously healthy boy has a sudden onset of lethargy, pinpoint pupils, and hypopnea. Evaluation shows respiratory acidosis and hypoxia. These findings suggest

opioid toxidrome - most appropriate next step in management is intravenous naloxone

Fractional excretion of sodium <1% is consistent with...

prerenal acute kidney injury

A mother brings her 6-year-old daughter for an evaluation for concerns about "knock knees." Her parents noticed that her knees seem to bump together when she runs. The girl denies any pain. She is otherwise healthy with normal growth and development. Physical examination reveals full range of motion and normal strength throughout both lower extremities. Genu valgum is apparent on stance and gait evaluation. Supine intermalleolar measurement is 7 cm. Of the following, the BEST next management step for this girl is

reevaluation in 6 months - Physiologic genu valgus ("knock knee") peaks in early childhood and then regresses to adult values over the next several years. - Radiography should be performed in children older than 7 years with intermalleolar distances greater than 8 cm.

What is the primary lab you are tracking to assess response to treatment in DKA?

renal panel - The anion gap reflects the degree of ketosis and is the primary lab being followed. - While pH, bicarbonate level, and to a lesser extent, urine ketones can be helpful, they may not reflect the actual ketoacidotic state of the patient - Similarly, the urine ketones may lag behind the actual serum ketones.

Inhaled steroids are a risk factor for oral candidiasis, and this risk can be mitigated by

rinsing with water after steroid inhalation

A 9 mo old girl has repeated night awakenings after going to sleep and has been unable to return to sleep without breastfeeding. This behavior has been present for the past month, prior to which she slept the entire night without waking or requiring middle-of-the-night feedings. Her growth and development are normal. She breastfeeds 6-7 times/day and eats a variety of solid foods. Of the following, which is the most likely explanation for this behavior?

separation anxiety - The advent of object permanence corresponds with qualitative changes in social and communicative development. - Infants look back and forth between an approaching stranger and a parent and may cling or cry anxiously, demonstrating stranger anxiety. - Infants who have been sleeping through the night for months begin to awaken regularly and cry, as though remembering that the parents are in the next room.

Infant has nystagmus, torticollis, and head titubation, a triad of clinical findings consistent with

spasmus nutans - typically presents in the first year, usually after 6 months of age, and resolves during childhood. - head titubation and torticollis can occur intermittently and may be a compensatory movement for the nystagmus, resulting in the appearance of "spasm" - nystagmus is divided into 2 types: jerk nystagmus, which is characterized by a slow movement to one side with a fast corrective jerk to the opposite side, and pendular nystagmus, which is a slow sinusoidal oscillation lacking a fast component

Without newborn screening the diagnosis of homocystinuria is usually made after 3 yr of age, when

subluxation of the ocular lens (ectopia lentis) occurs - This causes severe myopia and iridodonesis. - Progressive intellectual disability is common. - skeletal abnormalities resembling those of Marfan syndrome; they are usually tall and thin, with elongated limbs and arachnodactyly. - Scoliosis, pectus excavatum or carinatum, genu valgum, pes cavus, high arched palate, and crowding of the teeth are common.

A 16 yr old boy who has recently traveled to sub-Saharan Africa is diagnosed with Plasmodium vivax malaria. The fever pattern is one of febrile periods every other day. The term that best characterizes this fever pattern is:

tertian fever - occurs on the 1st and 3rd days (malaria caused by Plasmodium vivax)

Thrush is usually treated with

topical antifungals including nystatin, clotrimazole, or miconazole - Oral fluconazole can be considered if first-line treatments for mucocutaneous infections fail

Foreign body ingestion peaks in young children of what age

under 2 yo

Laboratory monitoring varies with AED choice. Hepatic function panels are recommended for AEDs metabolized through the liver, such as

valproic acid oxcarbazepine phenytoin

What cephalosporins are commonly used in the outpatient management of sinopulmonary infections and otitis media?

variety of orally administered 2nd generation agents (cefaclor, cefprozil, loracarbef, cefpodoxime)

Immunotherapy against Hymenoptera is indicated in those ≥ 17 yr of age if ...

venom skin test results are positive and there is a history of generalized urticaria or a systemic reaction - because their risk for future systemic reactions is 60-70%

Imaging for kid with.... anomalies of tracheobronchial tree, evaluate lumen and pulmonary infiltrates that fail to resolve in 10 to 14 days

virtual bronchoscopy

Girl is brought to your office for health supervision visit. When you enter the room, pt is walking around, stoops to pick up object on floor, and hands it to her father. She has 2 to 3 words and points to objects that she wants her father to notice. Her dad reports that she is unable to climb steps and cannot point to any body parts when asked. Most likely age of child?

*15 mos* - can walk well and stoop to pick up objects from floor - 3-5 words - can imitate environmental sounds

A 4-year-old boy presents as the unrestrained passenger from a motor vehicle collision. The boy was asleep on the backseat when the mother hit a parked car at around 20 miles per hour. The patient appears distressed but is consolable. Neurologic exam is nonfocal and Glasgow Coma Scale is 15. The patient has bruising on his back but has no midline tenderness over the spine. He has a small laceration on his bottom lip as well as multiple dental caries. He is at 55th percentile for height and sixth percentile for weight. He complains of a mild pressure-like headache and that he is hungry. His mother feels that her son is fine and requests that he be discharged. What is the most appropriate next step in management?

*Admit the patient* - Child neglect can include failure to provide safe environments (e.g., not using a car seat and leaving the child unrestrained, requesting discharge before medically appropriate), failure to provide medical care (e.g., poor oral care), and failure to provide adequate nutrition (e.g., large discrepancy in percentiles of weight versus height, hunger). - If suspicion for neglect is high, a patient should be admitted to provide a safe environment until the diagnosis can be established. Admitting children at risk for neglect also provides an opportunity for parents to access resources through the hospital (e.g., dentistry, food pantries).

A 5 year old male presents to your office with a chronic cough - Cough is productive, increased at night, recurrent - Worse with exercise and with upper respiratory infections - Growth has been normal - Chest xray findings are normal except for mild hyperinflation

*Asthma* - typically develops in childhood - 50% before 3 yo; majority before 8 yo - bronchial obstruction

An 8-year-old presents to the pediatrician's office with her mother repeatedly for enuresis. She has been seen in the office once a week over the past several months. She insists that her daughter wets the bed every night and demands that "something needs to be done about this." She claims that the family has tried enuresis alarms and behavioral modifications and she is now giving nightly desmopressin and her daughter continues to wet the bed. The child does not have constipation, and previous laboratory evaluations have shown normal electrolytes and blood sugar. When the pediatrician questions the child, she denies wetting the bed. The mother states "she tells me that every morning and every morning I have to change the sheets." She demands more testing to determine what the problem is, stating "put her in the hospital if you have to, I don't care." The pediatrician suspects that the mother may be fabricating the history of illness and calls the child's father who confirms that he has no concerns about bedwetting but states that he was unaware of the multiple doctors visits or the nightly desmopressin. A call to the pharmacy confirms that the mother has been filling the desmopressin prescription monthly. What is the most appropriate next step in management?

*Contact child protective services and a child abuse specialist*

Newborn male with no palpable testes = cryptorchidism --> associated with ... ?

*Prune belly syndrome* - next best test = US if not palpable

An almost 6-year-old boy presents to clinic for a well-child check. He will be starting kindergarten in a few weeks. His mother expresses concern that he does not hold a pencil correctly and is not yet able to write his first name. His preschool teacher had concerns that he was not able to use scissors. Which of the following fine motor skills would also be expected of this boy?

*buttons a shirt* = 4-5 yo --> write his first name and use scissors well around age 5 + tying single knot, using fork, copy square, draw 6-8 part person, dress himself

According to the US Centers for Disease Control and Prevention Sexually Transmitted Diseases Treatment Guidelines, the recommended management for uncomplicated gonococcal cervicitis is

*ceftriaxone 250 mg intramuscularly, and either azithromycin 1 g orally in a single dose or doxycycline 100 mg twice a day orally for 7 days* - azithromycin or doxycycline is included in the regimen because of the risk of co-infection with Chlamydia trachomatis as well as the growing concern for cephalosporin-resistant Neisseria gonorrhoeae - Azithromycin is preferred over doxycycline when possible because it is administered as a single dose. - Nucleic acid amplification tests have better overall sensitivity and specificity compared to other diagnostic tests for gonococcal infections.

A 19-year-old woman presents to your office for primary amenorrhea. You perform a pelvic exam and note the presence of a blind vaginal pouch. Further workup confirms the diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome. Magnetic resonance imaging reveals the presence of a horseshoe kidney. Which of the following is the embryologic origin of the kidney?

*metanephros* - Agenesis of the vagina defines Mayer-Rokitansky-Küster-Hauser syndrome. - While the female genital tract originates from the Müllerian ducts, urogenital sinus, and vaginal plate, the metanephros differentiates to form the kidneys around five weeks gestation. - When present, it is ipsilateral to the side of the uterine defect.

+ TB test + positive exam or CXR. Next steps...

- <12 mo --> obtain LP looking for CNS infection; consider LP if 13-24 mo - 3 sputum samples or gastric aspirates for AFB and smear - Report to SF TB Control (415-206-8524) - Start tx with 4 drug therapy); do not wait for the results - Home or in-hospital isolation

A family is switching from ready to feed formula to powdered formula and asks how to prepare 3 ounces their baby usually drinks. They use Enfamil infant. Correct options:

- Add 4 ounces of water to bottle and then add 2 scoops of formula powder. Save extra ounce for later in refrigerator. - Add 6 ounces of water to bottle and then add 3 scoops of formula powder. Split into 2 bottles and save extra 3 ounces for later.

How to prepare for CDH resuscitation

- FiO2 at 1 - 2 umbilical catheters prepared/wet down - pre- and post-ductal SpO2 monitors - surfactant if </= 34 wks GA (1.5-2 ml/kg d/t low lung volume) = repogle and suction available - morphine 0.1 mg/kg - vecuronium 0.1-0.2 mg/kg - intubate immediately Vent management - low PEEP (2-3 cmH2O) - limit PIP (</=30 cm H2O) - high rate (>200) if chest excursion poor - rapid surfactant administration if </= 34 wks' gestation

Indication for Tdap in the U.S.

- Immunization for children ≥7 yrs. of age - Routine immunization of children aged 11-12 yrs. of age - Adolescent age ≥13 years and unimmunized with Tdap - Pregnancy (single dose during each pregnancy) - Unvaccinated or incompletely vaccinated against pertussis - Wound management (Td or Tdap) - Tetanus or diphtheria disease - Unimmunized or incompletely immunized in close contact with diphtheria or pertussis disease

Recognize hypospadias

- In patients with hypospadias, the urethral meatus opens on the ventral surface of the penis proximal to the normal glanular location. - Hypospadias is the most common malformation of the male genitalia, occurring in 1 in 300 births. Hypospadias is classified by the anatomic location of the meatus and the presence or absence of chordee (ventral curvature of the penis). Hypospadias can be classified as first degree (on the glans), second degree (on the penile shaft), and third degree (penoscrotal to perineal). Another classification approach is distal (glandular, coronal, subcoronal), middle (distal shaft, midshaft, posterior penile), or proximal (penoscrotal, scrotal, perineal). The most common form of hypospadias is distal (subcoronal)/first degree. - Proximal hypospadias is more frequently associated with other malformations than is distal hypospadias. Genital malformations (such as cryptorchidism and inguinal hernia) are more common (8%-15%) than urinary tract abnormalities (such as vesicoureteral reflux, ureteropelvic junction, ectopic kidneys, and renal agenesis) in patients with hypospadias. The association of distal forms of hypospadias with renal anomalies (1% in patients with distal and 5% in patients with proximal hypospadias) is no greater than that of the general population, making it unnecessary to perform renal ultrasonography in patients such as the child in this vignette. - The penile foreskin is used in hypospadias repair; therefore, circumcision is contraindicated in these patients. Patients with hypospadias should be referred to a urologist within the first few weeks after birth. This initial evaluation addresses parental concerns and provides them with information about the required surgical intervention. The optimal time for hypospadias repair is at 6 months of age. A referral to genetics should be considered in patients with third-degree or proximal hypospadias.

The Ottawa knee rules are a validated screening tool designed to minimize unnecessary ragiographs in patients presenting with acute knee injury. Radiographs should be obtained in patients with any of the following on evaluation:

- Isolated tenderness of the patella - Tenderness at the head of the fibula - Inability to flex to 90° - Inability to bear weight for 4 steps Standard radiograph views in these cases should include anteroposterior, lateral, patellar, and tunnel views.

Recognize *juvenile melanoma*

- Spitz nevi also are called juvenile melanomas. - The lesions usually are seen on the face or limbs of children and often grow rapidly initially. - The lesions do resemble a melanoma but are completely benign. - Spitz nevi are dome-shaped, red-brownish lesions which vary from 1 to 2 cms in size.

Equipment Ideally, you will have a pre-made umbilical line kit. At minimum, you will require:

- Sterile gloves (plus full sterile gown and drapes if less urgent) - Chlorhexidine - Forceps - Scalpel - Umbilical line (5 french is standard, 3.5 french in a very premature baby). A size 5 feeding tube can be used as a substitute. - Umbilical tape (or a suture for a purse string suture) - NS flush

Child has acute kidney injury (AKI) secondary to vancomycin toxicity. A high vancomycin level causes what lab abnormalities?

- acute renal tubular injury leading to increased fractional excretion of sodium - elevation in serum creatinine level

Recognize linear scleroderma

- also called morphea - involves cutaneous tissue and sometimes underlying muscle and bone - active localized scleroderma lesions have a red border and a white "waxy" center - as the lesions become inactive, they can be either hyper- or hypopigmented

When and how to start brushing children's teeth?

- begin brushing as soon as first teeth appear with fluorinated toothpaste - "brush, book, bed" - when children are 3 yo, tiny smear/rice sized of fluoride toothpaste is enough; from 3-6 yo, can increase amount to pea-sized dallop

What is robinul?

- glycopyrrolate - quaternary amine anti-muscarinic agent - doesn't cross BBB bc of quaternary structure (unlike atropine & scopolamine that cause sedation & delirium in elderly) - anti-muscarinic properties - used in anesthesia before surgery to reduce salivary, tracheobronchial, and pharyngeal secretions - decreases acid secretion in stomach --> treats stomach ulcers - used topically and orally to treat hyperhidrosis (esp gustatory hyperhidrosis) - used to treat COPD

What major changes in infant circulation occurs following birth in the lungs?

- lungs expand - PaO2 increased --> pulmonary vasodilation - drop in pulmonary vascular resistance

Reasonable initial screen for FTT

- obtaining the state's newborn screening results - complete blood cell count - urinalysis

Recognize allergic contact dermatitis

- typically appears 1 to 3 days after exposure but as early as after 8 hours in those who are highly sensitive; - may persist for 1 to 3 weeks - if the antigen involved is potent, like urushiol in poison ivy, and depending on the amount that contacts the skin, the result is an acute dermatitis with erythema, edema, vesicle or bulla formation; oozing (when vesicles rupture); and crusting (when fluid dries) - less potent antigens (eg, nickel) produce features of subacute or chronic dermatitis with lichenification and scaling - another clue is a dermatitis that persists despite topical corticosteroid treatment - in uncertain cases, patch testing to a panel of allergens may be performed - treatment of mild ACD = aimed at reducing pruritus. Options include a topical antipruritic agent (a lotion such as calamine or a product containing pramoxine), a midpotency topical corticosteroid, an oral antihistamine, or cool tap water compresses (these are soothing, promote drying of lesions through evaporation, and remove crust) - For more widespread involvement or severe involvement of the face or genitalia, systemic corticosteroid therapy is useful. Prednisone (beginning at 1 mg/kg per day or its equivalent) is tapered gradually over 2 to 3 weeks; earlier cessation may result in a rebound of the dermatitis.

EKG 1 small square =

1 mm = 0.04 sec

Ophthalmology exam for ROP commencing at what age?

1 mo for infants born <32 wks

Clavicular fracture will form callus in ...

1 wk - no tx needed - can use figure of 8 splint

Per AAP Bright Futures, for middle childhood (ages 5 - 10), when is dyslipidemia screening done?

10 yo

Conversion of cellcept to myfortic

1000mg MMF = 720mg myfortic

Delayed eruption is usually considered when there are no teeth by approximately what age?

13 mo of age - mean + 3 standard deviations - common causes include hypothyroid, hypoparathyroid, familial, and (the most common) idiopathic disorders

If you are measuring your body weight in pounds: Assume you weigh 160 lbs. In this example: TOTAL DAILY INSULIN DOSE =

160 lb ÷ 4 = 40 units of insulin/day

When is Meningococcal vaccine (MCV4) given to preteens and adolescents?

1st dose age 11-12 yo Booster at age 16 or before entering college - Adolescents with HIV should receive 3 doses - 2nd dose 2 months apart during age 11-12 plus booster at age 16 - safe in pregnancy

Normal Alveolar-arterial oxygen gradient (A-a gradient)

20-65 mmHg on 100% or 5-20 mmHg on room air

Eye disorders and vision, if corrected vision worse than what in one eye, then strongly consider eye protection of normal eye?

20/40 in one eye - eye protection is essential in certain high-risk sports

By what age can most children walk up and down stairs independently while holding a guardrail?

24 months

Dosing of MMF/mycophenolate mofetil

25 mg/kg/dose BID - starts after transplant - IV = po

How soon can 2nd MMR dose be given after 1st dose

28 days

Ideally pts should be how old to do spirometry

5 yo

Estimate the number of suicide attempts per completed suicides in the United States.

50 to 100

Recommended hours of sleep for: 13-18 years

8-10 hours

Normal ratio for FEV1 =

80%

The licensed quadrivalent meningococcal vaccines contain capsular groups:

A, C, W-135, and Y

Rare neurologic disorder presenting with acute paralysis of 1 or more limbs, preserved sensation, and normal mental status, typically within a week of onset of viral symptoms. Clinical severity is variable. MRI of the spine will show extensive longitudinal centrally located inflammation affecting the gray matter of the cord. Cerebrospinal fluid analysis typically reveals a lymphocytic pleocytosis.

Acute flaccid myelitis

A 13 yr old girl develops a sore throat and low-grade fever. A throat culture is positive for group A streptococcal infection, for which she is given oral penicillin. Seven days later, she develops a rash and fever. Urine output is normal. Her pulse is 90 beats/min; blood pressure is 110/60 mm Hg. Serum creatinine is 2.4 mg/dL. WBC count is 12,000/mm3 with 60% neutrophils, 25% lymphocytes, and 15% eosinophils. C3 level is normal. Urinalysis demonstrates specific gravity of 1.010, small amount of blood, no protein, 5-10 WBCs per high-power field, 5-10 RBCs per high-power field, and no RBC casts. The most likely diagnosis is:

Acute tubulointerstitial nephritis - classic presentation: fever, rash, and arthralgia in the setting of a rising serum creatinine value - rash can vary from maculopapular to urticarial and is often transient - patients often have nonspecific constitutional symptoms of nausea, vomiting, fatigue, and weight loss - flank pain may be present owing to stretching of the renal capsule from acute inflammatory enlargement of the kidney

Which of the following infants born to HIV-infected mothers should receive prophylaxis for Pneumocystis jiroveci?

All infants 6 wk-1 yr of age born to HIV-infected mothers, unless noninfection is proven - Prophylactic regimens are integral for the care of HIV-infected children. - All infants between 4-6 wk and 1 yr of age who are proven to be HIV-infected should receive prophylaxis to prevent Pneumocystis jiroveci infection regardless of the CD4 count or percentage. - Infants exposed to HIV-infected mothers should receive the same prophylaxis until they are proven to be noninfected; however, prophylaxis does not have to be initiated if there is strong presumptive evidence of noninfection (i.e., non-breast-fed infant with 2 negative HIV PCR tests at > 14 days and 4 wk of age, respectively). - When the HIV-infected child is > 1 yr of age, prophylaxis should be given according to the CD4 lymphocyte count. - The best prophylactic regimen is 150 mg/m2/day of trimethoprim component of TMP/SMX given as 1-2 daily doses 3 days per wk. - For severe adverse reactions to TMP/SMX, alternative therapies include dapsone, atovaquone, or aerosolized pentamidine.

Vaccine guidelines for pts undergoing cardiopulmonary bypass

All pts undergoing cardiopulmonary bypass should not receive live vaccines for 2 wks before or 6 wks after cardiopulmonary bypass - cytokine & immunologic markers return to normal by 2 mos after surgery, & vaccination schedule can be resume Children younger than 12 mos with unrepaired cyanotic CHD or those receiving diuretic therapy for CHD in acyanotic CHD should receive RSV prophylaxis with palivizumab Children who meet criteria for RSV prophylaxis after palliative sx invovling cardiopulmonary bypass, a postop dose of 15 mg/kg palivizumab should be considered

The breast-fed infant of a mother who is a strict vegan may experience deficiency of which of the following vitamins if the mother is not receiving supplements of the vitamin?

B12 - Plants are not generally a good source of B12. - Additional vitamin B12 can be obtained through dairy products and eggs, and vegans typically need fortified foods or supplements. - Breast-feeding by vegan mothers can place an infant at risk for vitamin B12 deficiency.

What is Qvar

Beclometasone dipropionate - brand name = Qvar - available as an inhaler, cream, pills, and nasal spray - inhaled form is used in the long-term management of asthma - cream may be used for dermatitis and psoriasis - pills have been used to treat ulcerative colitis - nasal spray is used to treat allergic rhinitis and nasal polyps - medicaid covered - 2 puffs morning and 2 puffs night

Guide for distance to insert an umbilical arterial catheter... Birth wt: 1,000 - 1,500 - 2,000 - 2,500 -

Birth Weight = x distance to insert umbilical arterial catheter (cm) 1,000 g = 7 cm 1,500 g = 8 cm 2,000 g = 9 cm 2,500 = 10 cm

First level of dietary change to be recommended for all children with dyslipidemias

CHILD-1 diet - specially designed for children with risk factors for coronary artery disease - focuses on limiting dietary cholesterol to 300 mg/day, limiting sugary drink consumption, using reduced-fat/skim milk, avoiding foods high in trans-type fats, limiting foods high in sodium, and encouraging consumption of foods high in fiber

Initial pediatric asthma algorithm for severe asthma exacerbation...

CR & O2 monitors, BP if Mag used, O2 to achieve O2 >90% --> Call RT, make NPO, place IV --> Immediate orders = Albuterol 2.5 mg/ipratropium 0.5mg q20 minutes x 3, until albuterol 20 mg/hr is started Methylprednisolone 1-2 mg/kg (60 mg max) IV Consider ordering: Magnesium 40-50mg/kg (2mg max) IV +/- 20 mL/kg NS bolus Reassess q15 min or more frequently as dictated by clinical need

Initial pediatric asthma algorithm for mild asthma exacerbation...

CR & O2 monitors, BP if Mag used, O2 to achieve O2 >90% --> Immediate orders = albuterol 2.5 mg neb x 1 OR albuterol MDI 4-6 puffs x 1 --> reassess after albuterol completion

Initial pediatric asthma algorithm for moderate asthma exacerbation...

CR & O2 monitors, BP if Mag used, O2 to achieve O2 >90% --> Immediate orders = albuterol 2.5 mg/ipratropium 0.5 mg neb q20 minutes x 3 (intermittent or continuous) OR albuterol 10-15 mg/hr x 1 hr (if ipratropium unavailable) and dexamethasone 0.6 mg/kg (max 16 mg) PO/IM OR prednisolone 1-2 mg/kg (max 60 mg) PO reassess after 20 minutes of albuterol/ipratropium

Most frequent cause of UTIs in ICUs

Candida - dev't of renal fungus balls or abscesses and unilateral or bilateral renal obstruction - renal insufficiency may be the first clinical manifestation of invasive candidiasis

What is robitussin?

Dextromethorphan - brand name and registered trademark for both over-the-counter (OTC) and prescription cough and cold medicines manufactured by Pfizer - used to treat acute cough, mucus buildup and nasal congestion - because this medicine contains codeine, it is usually only prescribed when a patient has a painful and persistent cough, and/or one that interferes with the patient's sleep cycle - each 5 mL of Robitussin DAC contains 10 mg of codeine, 100 mg of guaifenesin, and 30 mg of pseudoephedrine - Robitussin AC is Robitussin DAC without the pseudoephedrine - the taste mimics cherries but is said to have an unpleasant after-taste - the version of Robitussin AC with promethazine is usually flavored peach-mint or grape/menthol. These syrups are purple instead of red - Generic forms are available under the names Cheratussin DAC and Cheratussin AC

A mother reports to the nurse that her newborn is not feeding well. He was born 28 hours ago at 39 weeks' gestation via spontaneous vaginal delivery after an uncomplicated pregnancy. Her group B Streptococcus status was positive and she received 3 doses of penicillin before delivery. The neonate's Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. The neonate latched on and fed well yesterday, but has not been interested in breastfeeding today. On physical examination, his temperature is 36°C, heart rate is 145 beats/min, respiratory rate is 65 breaths/min, and blood pressure is 65/43 mm Hg. He is unresponsive. His pupils are reactive to light; anterior fontanelle is flat; heart has a regular rate and rhythm, with no murmur noted; lungs are clear with normal work of breathing. He has no hepatomegaly; +2 peripheral pulses; capillary refill 2 to 3 seconds; generalized decreased tone; limited active movement; no clonus; and decreased reflexes throughout. His glucose level on dextrose stick is 65 mg/dL (3.6 mmol/L). He is brought to the neonatal intensive care unit, where a blood culture, cerebrospinal fluid culture, arterial blood gas, and ammonia level are obtained. He is started on treatment with intravenous ampicillin, gentamicin, and acyclovir. Of the following, the BEST next test to confirm this neonate's diagnosis is

EEG - The most likely cause of this neonate's condition is seizures, which can be diagnosed with electroencephalography. - Neonatal seizures affect 1 to 3 per 1,000 neonates born at term gestation. The most common cause is hypoxia resulting from perinatal events or cardiac dysfunction. - Neonates are at increased risk for seizures compared with older children and adults for several reasons. In the first weeks after birth, neonates have decreased inhibition of cortical activity. The neonatal neuron has a relatively high chloride concentration, dampening the inhibitory neurotransmitter effect of γ-aminobutyric acid (GABA). In addition, mechanisms of inhibition within the substantia nigra are not fully developed. There is also a predominance of excitatory activity via glutamate and N-methyl D-aspartic acid (NMDA) receptors.

IVH is characterized by the extent of hemorrhage as follows:

Grade 1: Involves only the germinal matrix Grade 2: Involves the ventricle without ventricular dilation Grade 3: Involves at least 50% of the ventricle with associated dilation Grade 4: Involves periventricular brain parenchyma

A 7 mo old child presents in late October with 3 days of fever with temperatures to 103.5°F, a mildly injected pharynx, mild cervical lymphadenopathy, and diarrhea. The child has been behaving normally and eating well and has no other symptoms. On the 4th day of the illness the fever resolves, and a generalized measles-like rash appears 12 hr later. The child appears normal on physical examination. The most likely diagnosis is:

HHV-6 infection Roseola infantum - exanthem subitum or sixth disease - acute, self-limited disease of infancy and early childhood - characterized by the abrupt onset of high fever, which may be accompanied by fussiness - fever usually resolves acutely after 72 hr ("crisis") but may gradually fade over a day ("lysis") coincident with the appearance of a faint pink or rose-colored, nonpruritic, 2- to 3-mm morbilliform rash on the trunk - rash usually lasts 1-3 days but is often described as evanescent and may be visible only for hours, spreading from the trunk to the face and extremities - because the rash is variable in appearance, location, and duration, it is not distinctive - associated signs are sparse but can include mild injection of the pharynx, palpebral conjunctivae, or tympanic membranes and enlarged suboccipital nodes

The classic triad of findings in pulmonary hemosiderosis includes:

Hemoptysis, iron deficiency anemia, multiple alveolar infiltrates on chest radiograph

A boy has gram-negative bacteremia after travel abroad, which is most concerning for typhoid fever. Typhoid fever is endemic in many resource-limited countries. Most cases in the United States occur after travel to Pakistan, India, Mexico, and Bangladesh. Who are the source of enteric fever infections, including infections caused by Salmonella enterica serovars Typhi, Paratyphi A, and Paratyphi B?

Humans - Typhoidal Salmonella infections are characterized by fever, constitutional symptoms, and abdominal pain. - Patients can have diarrhea or constipation. - Physical examination findings can include hepatosplenomegaly, jaundice, and rose spots, a macular exanthem. - Laboratory abnormalities can include leukopenia, anemia, thrombocytopenia, elevated transaminase levels, and hyperbilirubinemia. - Initial treatment of invasive nontyphoidal Salmonella infections is ceftriaxone. Uncomplicated bacteremia can be treated for a total of 7 to 10 days with transition to oral therapy after negative blood cultures and exclusion of focal infection. The duration of therapy for meningitis is typically 4 weeks and for osteomyelitis is 4 to 6 weeks. - Protection against Salmonella enterica serovar Typhi is provided by 2 available vaccines and should be considered prior to international travel. - Animals are the main reservoir of nontyphoidal Salmonella; poultry, reptiles, and rodents can be colonized. Although many foods have been associated with outbreaks of Salmonella infection in the United States, chicken and eggs are most commonly implicated. Implicated foods not of animal origin have been contaminated by an animal product or human carrier.

A 4 yr old previously well girl presents with fever (temperature of 103°F), nonproductive cough, dyspnea, and left-sided chest pain. Initial examination reveals an ill-appearing but stable child; pulse rate is 125 beats/min, respiratory rate is 40 breaths/min, and room air oxygen saturation is 89%. Auscultation demonstrates decreased air movement on the left side, with crackles. Chest radiograph shows a left lower lobe consolidation. What is the appropriate initial antibiotic therapy?

IV ampicillin - For mildly ill children who do not require hospitalization, amoxicillin is recommended. In communities with a high percentage of penicillin-resistant pneumococci, high doses of amoxicillin (80-90 mg/kg/24 hr) should be prescribed. - The empirical treatment of suspected bacterial pneumonia in a hospitalized child requires an approach based on the clinical manifestations at the time of presentation. The first line of therapy is ampicillin for suspected bacterial pneumonia; parenteral cefotaxime or ceftriaxone can be used if the patient doesn't improve on ampicillin. - If clinical features suggest staphylococcal pneumonia (pneumatoceles, empyema), initial antimicrobial therapy should also include vancomycin or clindamycin.

A 6 mo old child presents with recurrent cellulitis and bacteremia due to Staphylococcus aureus. The white blood cell count is 2,500/mm3 with 5% neutrophils, 10% eosinophils, 35% monocytes, and 50% lymphocytes. The platelet count is 650,000/mm3. A brother and a female cousin died at the ages of 18 mo and 2 yr, respectively. The most likely diagnosis is:

Kostmann disease - Severe congenital neutropenia is characterized by an arrest in myeloid maturation at the promyelocyte stage in the bone marrow, resulting in consistent ANCs < 200/μL. This disorder occurs sporadically or with autosomal dominant or recessive inheritance. The dominant form is caused most often by mutations in ELA2, while the recessive form (Kostmann disease) arises from mutations in HAX1, which protects cells against apoptosis. - Patients typically show monocytosis and eosinophilia and suffer from recurrent, severe pyogenic infections, especially of the skin, mouth, and rectum. Anemia of chronic inflammation is often present. Approximately 20% of patients develop acute myelogenous leukemia or myelodysplasia associated with monosomy 7 and sometimes preceded by acquisition of mutations in the gene encoding the G-CSF receptor. - Before the advent of treatment with rhG-CSF, most of these patients died of fatal infections before reaching adolescence.

Common in chronic kidney disease and is the result of multiple, complex causes including inadequate nutrition, metabolic acidosis, fluid and electrolyte imbalance, metabolic bone disease, severe anemia, and growth hormone insensitivity.

Linear growth impairment - dialysis and renal transplant for children with CKD improves growth, but will not make up for deficits that already occurred

A 16-year-old girl presents with headaches and blurry vision. She has had these symptoms intermittently for the past few weeks, but they seem to be worsening. She has had occasional vomiting but no fever, rash, or respiratory symptoms. She takes minocycline for acne but has no other medical problems. On exam, she has some photophobia but her neurologic exam is grossly normal. You note papilledema on fundus exam. She has no nuchal rigidity. CT imaging does not show any structural lesion. What is the best approach to this patient?

Lumbar puncture with opening pressure measurement headache and blurry vision without fever or neck stiffness = idiopathic intracranial hypertension formerly known as pseudotumor cerebri - variety of medications have also been implicated, including oral contraceptives and minocycline. - Treatment is geared towards decreasing intracranial pressure, typically via a lumbar puncture with opening pressure measurement, which can be both diagnostic and therapeutic.

A 13 mo old infant is found comatose in bed after sleeping later than usual. On physical examination, the infant is afebrile and of normal size and the liver is palpable 4 cm below the costal margin. The plasma glucose level is 15 mg/dL; bicarbonate, 20 mEq/L; BUN, 35 mg/dL; ammonia, 295 μmol/L; AST, 320 units/L; and ALT, 425 units/L. Bilirubin is normal. Urinalysis is negative for glucose, ketones, protein, and reducing substances. Which of the following is the most likely diagnosis?

MCAD deficiency - most common fatty acid oxidation disorder - usually present in the 1st 3 mo-5 yr of life with episodes of acute illness triggered by prolonged fasting (longer than 12-16 hr) - signs and symptoms include vomiting and lethargy, which rapidly progress to coma or seizures and cardiorespiratory collapse - hypoglycemia is usually present - plasma and urinary ketone concentrations are inappropriately low (hypoketotic hypoglycemia) - because of the relative hypoketonemia, there is little or no metabolic acidemia - tests of liver function are abnormal, with elevations of liver enzymes (ALT, AST), elevated blood ammonia, and prolonged prothrombin (PT) and partial thromboplastin times (PTT)

A 12 yr old girl presented with nonspecific abdominal pain and hepatosplenomegaly and was found to have an ALT of 185 IU/L; total bilirubin, 2.4 mg/dL; alkaline phosphatase, 640 IU/L; and an anti-smooth muscle antibody titer, 1:80. Liver biopsy revealed a lymphoplasmacytic infiltrate of the portal tracts. She has been treated for 4 mo with prednisone without significant improvement. The next most appropriate diagnostic study would be:

MR cholangiography - The initial response to therapy in autoimmune hepatitis is generally prompt, with a > 75% rate of remission. - Transaminases and bilirubin fall to near-normal levels, often in the 1st 1-3 mo. - More extensive evaluations of the etiology of their hepatitis should be undertaken, directed particularly at reassessing for the presence of either sclerosing cholangitis or Wilson disease. - MR cholangiography may be very useful for screening for evidence of sclerosing cholangitis.

The mainstay of treatment for all forms of juvenile idiopathic arthritis with low disease activity is

NSAIDs, like ibuprofen

Recommended to be performed in all suspected foreign body ingstions, whether or not patients are symptomatic.

Plain film radiography

A 19 yr old female patient tells you she had unprotected sex yesterday with her steady partner. She does not want to become pregnant. Your emergency treatment should be to give:

Plan B 1 dose now (2 pills) or Ovral 2 pills now and in 12 hr Yuzpe method - commonly used in the USA - combination pills totaling 200 μg ethinyl estradiol and 2.0 mg norgestrel or 1.0 mg levonorgestrel - pills that can be utilized for this method include Ovral 2 pills × 2 doses 12 hours apart or Lo-Ovral 4 pills × 2 doses 12 hours apart - effective in reducing the risk of pregnancy by 75% - most common side effects are nausea (50%) and vomiting (20%), prompting some clinicians to prescribe or recommend antiemetics along with the OCs - urine pregnancy test is usually required prior to dispensing the pills to rule out an existing pregnancy - progestin-only Emergency Contraception kit (Plan B) was FDA approved in 1999 and contains 2 tablets, each with 0.75 mg levonorgestrel

Burners (also known as stingers) are common brachial plexus injuries in youth who play contact sports, particularly American football. When can players return to full activity?

Players who experience a burner may return to full activity when strength and sensation return to normal. - Recurrent burners should be evaluated for possible cervical stenosis or other underlying pathology.

Good nonvegetarian dietary sources of thiamine ...

Pork (especially lean), fish, and poultry - Main sources of thiamine for vegetarians are rice, oat, wheat, and legumes. - Most ready-to-eat breakfast cereals are enriched with thiamine.

Primary tuberculosis - most often healed with creation of calcific hilar node and parenchymal nodule- known as ...

Ranke complex

A patient has hemolytic uremic syndrome (HUS) that developed 2 weeks after a diarrheal illness characterized by severe abdominal cramping and bloody diarrhea. Evaluation reveals microangiopathic hemolytic anemia (as evidenced by schistocytes on the peripheral smear) and thrombocytopenia. What diagnostic study would reveal abnormalities?

Renal function panel --> then the diagnosis of HUS can be confirmed by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and kidney injury.

X-linked disorder, predominantly affecting girls, characterized by a period of normal development followed by motor, language, and cognitive regression, loss of purposeful hand movements replaced with hand wringing, and an autistic-like phenotype. Serial head circumference measurements can demonstrate decelerating head growth during the time of normal development and be an early clue in patients with this syndrome.

Rett syndrome

A 12 yr old white girl presents with arthralgias of the knees and elbow and swollen hands of 6 months' duration. She has had intermittent fever and has lost 15 lb. Other than swollen joints, findings on physical examination are normal. Three years earlier, she was found to have thrombocytopenia and was diagnosed with idiopathic thrombocytopenic purpura (ITP). In addition, one summer she had severe sunburn and 2 yr ago she had mouth sores. Today she has a hematocrit of 25% and a positive result on a Coombs test, and the urinalysis shows multiple red blood cells. The most likely diagnosis is:

SLE

Current teaching is that central venous line tips should be at...

SVC-RA or IVC-RA junction - SVC-RA junction appx at T6 level and below right main bronchus - catheters left too short will migrate into upper system veins - catheters left too long may migrate through tricuspid valve or IVC

Most likely Salter-Harris type fracture to be associated with growth arrest is...

Salter-Harris type V fracture - crush injury to physis - most likely to result in focal bone growth arrest

A 15-year old non-sexually active girl complains of painful menstrual cramps causing her to miss school once per month. The most appropriate treatment option is:

Scheduled naproxen sodium just before menses begins for 24 hr - Because adolescents suffering from dysmenorrhea have high levels of prostaglandins, they experience symptomatic relief when prostaglandin synthetase inhibitors are administered. - If given before a menstrual period (or shortly after it begins), administration of a rapidly absorbed prostaglandin synthetase inhibitor, such as naproxen sodium, is effective in reducing prostaglandin production before pain occurs (2 tablets of 275 mg each taken with the onset of menses and 1 tablet taken every 6-8 hr after that for the 1st 24 hr). - Medication is rarely needed beyond the 1st day. - For the teenager with dysmenorrhea who requires contraception, combined hormonal therapy in the form of oral contraceptives, the contraceptive patch, or vaginal ring may be indicated.

What is an uncommon yet serious complication of repeated closed head injury that can result in rapid mental deterioration, mental status changes, and uncal herniation?

Second impact syndrome - can result from a second episode of closed head injury while the patient is still symptomatic from an initial concussion event, and has a mortality rate of 70% to 80%.

Most common cyanotic CHD

Tetralogy of Fallot - 5% of all CHD - develops from anterior malalignment of interventricular septum --> leads to VSD, as well as overriding of VSD by aorta - there is narrowing of pulmonary outflow tract due to septal deviation --> causes RV outflow (infundibular) obstruction and consequent RV hypertrophy

The parents of a 6 yr old girl relate a history of urticarial reaction and vomiting after administration of amoxicillin in the past. Skin testing to major and minor determinants of penicillin is positive. Which of the following statements regarding administration of a cephalosporin constitutes appropriate advice for the parents?

There is a 2% risk of anaphylaxis to a cephalosporin - Varying degrees of in vitro cross reactivity have been documented between cephalosporins and penicillins. - Although the risk of allergic reactions to cephalosporins in patients with positive skin test responses to penicillin appears to be low (<2%), anaphylactic reactions have occurred after administration of cephalosporins in patients with a history of penicillin anaphylaxis. - If a patient has a history of penicillin allergy and requires a cephalosporin, skin testing for major and minor determinants of penicillin should preferably be performed to determine whether the patient has penicillin-specific IgE antibodies. If skin test results are negative, the patient can receive a cephalosporin with no greater risk than found in the general population. If skin test results are positive for penicillin, recommendations may include: administration of an alternative antibiotic; cautious graded challenge with appropriate monitoring, with the recognition that there is a 2% chance of inducing an anaphylactic reaction; and desensitization to the required cephalosporin.

A formerly healthy 18-year-old college student presents with his mother for weight loss. His mother is concerned because he has lost approximately 15 pounds over the past year. She has noticed that when he is at home during breaks his appetite seems normal. He denies intentionally trying to lose weight. He has had no fever, night sweats, vomiting, or diarrhea. Upon direct questioning with his mother absent, he admits to using amphetamine/dextroamphetamine mixed salts at school that he has obtained from a friend. Which of the following represents the most likely reason for this patient's improper medication use?

To improve academic performance - Abuse of stimulant medications is associated with the use of alcohol, marijuana, and other substances. - Students report that the most common reason for using nonprescribed stimulants is to improve their academic performance.

Prefixes are used to describe therelative direction of the misaligned eye's deviation,such as "eso" for

adducting (inward or nasal) tendency

absence of dermatoglyphs and is caused by a SMARCAD1 gene mutation

adermatoglyphia

If unexplained vomiting, make sure you get this lab

ammonia - assess for urea cycle defect (OTC deficiency most common and can present with liver failure) - can be ppt'ed by food - classic = 1-2 yo + switch from formula to whole milk

A family returns to your travel clinic after a 3-week vacation to the Peruvian Andes. Their 12 yr old boy has become ill with fever, malaise, and a yellowish, pale color. On physical examination, he is pale, is tachycardic with mild icterus, and has generalized lymphadenopathy. The most likely etiologic agent is:

bartonella bacilliformis - Bartonellosis is a zoonosis found only in mountain valleys of the Andes Mountains in Peru, Ecuador, Colombia, Chile, and Bolivia at altitudes and environmental conditions favorable for the vector, which is the sandfly Lutzomyia verrucarum. - The incubation period is 2-14 wk. - Patients may be totally asymptomatic or may have nonspecific symptoms such as headache and malaise without anemia. - Oroya fever is characterized by fever with rapid development of anemia. - Clouding of the sensorium and delirium are common symptoms and may progress to overt psychosis. - Physical examination demonstrates signs of severe hemolytic anemia, including icterus and pallor, sometimes in association with generalized lymphadenopathy.

A 15-year-old adolescent girl is evaluated in the office for a 2-month history of chronic nonproductive cough associated with fatigue, 5- to 10-lb weight loss, low-grade fever, and mildly painful nodules on her arms and legs. She has not traveled out of the state or had any known sick exposures. On pulse oximetry, her oxygen saturation is 98% on room air. On physical examination, the girl has no respiratory distress, and no crackles or wheezes are heard. She has several 1- to 3-cm diameter, tender, nonerythematous, nonexcoriated nodules on the extensor surfaces of both arms and legs. Chest radiography suggests hilar adenopathy. Computed tomography additionally demonstrates interstitial changes in the pulmonary parenchyma. Pulmonary function testing shows a mild restrictive pattern with a decrease in forced vital capacity and total lung capacity. Carbon monoxide diffusion is slightly decreased for age and hemoglobin concentration. A tuberculin skin test has a negative result 60 hours after placement. Of the following, the BEST next step in the evaluation of this adolescent is

bronchoscopy and bronchoalveolar lavage - Bronchoscopy will not produce a diagnosis, though the bronchoalveolar lavage findings may show lymphocytosis with a reduced number of CD8 lymphocytes. However, this is not specific for sarcoidosis and not necessary for the diagnosis. - Sarcoidosis is a multisystem disease that usually presents between 20 and 60 years of age. Sarcoidosis is 4 times more common in black than white populations, and more common in women than men. - Black women are most commonly affected; they may develop disease up to 10 years earlier than white patients. - The most specific diagnostic test for sarcoidosis is biopsy of an affected organ; for the adolescent in the vignette, biopsy of a skin nodule is the least invasive approach to obtaining a tissue diagnosis. Biopsy of a hilar lymph node would be a more invasive approach than necessary. Sarcoidosis can affect all organ systems; the lungs are involved in most cases. Extrapulmonary sites may be affected exclusively or may be involved before pulmonary findings. After the lungs, the skin, eyes, and lymph nodes are the most commonly involved organs. The liver, spleen, parotid glands, and bone marrow may also be sites of noncaseating granulomas characteristic of sarcoidosis.

A 5 year old with trisomy 21 presents to the office for a check up. Mom notes that she has very heavy breathing when she is sleeping and frequent night awakenings. On physical exam, mild midface hypoplasia, large tongue and 2+ tonsils. Her lungs are clear. BMI is in the 25itle. What is the AAP guideline for referral for polysomnogram for patients with Trisomy 21?

by the age of 4 - Per AAP, "At least once during the first 6 months of life, discuss with parents symptoms of obstructive sleep apnea, including heavy breathing, snoring, uncommon sleep positions, frequent night awakening, daytime sleepiness, apneic pauses, and behavior problems that could be associated with poor sleep. Refer to a physician with expertise in pediatric sleep disorders for examination and further evaluation of a possible sleep disorder if any of the abovementioned symptoms occur." - If they have not received a sleep study by the age of 4, all children with DS should be referred for one. - As for treatment, adenotonsillectomy was most common, although previous studies have shown that moderate to severe OSA in children with Down syndrome is likely to persist after a tonsillectomy.

This patient has evidence of septic shock, presenting primarily with "cold" shock, which typically indicates some degree of

cardiovascular dysfunction - thought to be sepsis induced and involves downregulation of adrenergic pathways, cytokine mediated cardiomyocyte dysfuntion, and impaired intracellular Ca2+ trafficking.

5 major/Jones criteria of rheumatic fever...

carditis polyarthritis erythema marginatum subcutaneous nodules chorea

The hepatopulmonary syndrome (HPS) consists of the clinical triad of

chronic liver disease arterial deoxygen-ation widespread intrapulmonary vasodilation - pre-operative evaluation of patients suspected of having HPS should include arterial blood pO2 determination, transthoracic contrast echocardiography, arterial oxygen response to 100% oxygen administration, and quantification of intrapulmonary shunting using a macroaggregated albumin (MAA) scan

The recommended agent for treatment of pneumonia caused by Mycoplasma pneumoniae is:

clarithromycin or azithromycin - The recommended treatment is clarithromycin (15 mg/kg/day divided bid PO for 10 days) or azithromycin (10 mg/kg once PO on day 1 and 5 mg/kg once daily PO on days 2-5)

A 3 yr old child does well for the first 6 hr after surgery. Then dyspnea and tachycardia develop, with rapid shallow respirations. On physical examination the patient has decreased breath sounds and coarse rales on the right. Which of the following is the most appropriate first step in treatment?

cough, deep breathing, and percussion Postoperative atelectasis - when a large area of previously normal lung becomes atelectatic, especially when it does so suddenly, dyspnea accompanied by rapid shallow respirations, tachycardia, cough, and often cyanosis occurs - if the obstruction is removed, the symptoms disappear rapidly - physical findings include limitation of chest excursion, decreased breath sound intensity, and coarse crackles - breath sounds are decreased or absent over extensive atelectatic areas - if effusion or pneumothorax is responsible, the external compression must first be removed - often vigorous efforts at cough, deep breathing, and percussion will facilitate expansion - aspiration with sterile tracheal catheters may facilitate removal of mucus plugs - continuous positive airway pressure (CPAP) may improve atelectasis

Inside the molluscum papules is a core of white material. If the lesions are scratched, children can spread the rash (autoinoculation). If desired, the lesions can be treated with

cryotherapy cantharidin ("beetle juice") curettage - This is usually recommended for children with underlying eczema, lesions in the genital area, or compromised immune systems, including those with human immunodeficiency virus.

This patient demonstrates a recurrent pattern of fever and neutropenia about every 21 days, which is suggestive of

cyclic neutropenia - rare autosomal dominant condition due to a defect in the elastase (ELANE) gene for which a genetic test is available - typically present within the first year of life with symptoms of fever and malaise for a few days per month - depending on the severity of the neutropenia, granulocyte colony-stimulating factor (G-CSF) is often employed to treat these patients, with good results

A previously healthy 2-year-old boy is seen in the office for a worsening cough. The illness started 2 days ago with cough, rhinorrhea, and a fever of 38.3°C. His mother describes the cough as having a harsh, seal-like quality. The boy's temperature is 38°C, heart rate is 110 beats/min, respiratory rate is 25 breaths/min, oxygen saturation is 99% on room air, and blood pressure is 90/60 mm Hg. At rest, he has a frequent seal-like cough; he is not in respiratory distress and has no stridor. His lungs are clear to auscultation bilaterally with good aeration, and he has no chest wall retractions. When he becomes more active, the boy has mild stridor and mild retractions. Of the following, the BEST next step in management of the boy's symptoms is to

dexamethasone - Children with worsening mild croup and children with moderate or severe croup should be treated with oral glucocorticoids. - Nebulized epinephrine may be considered for treatment of children with moderate croup and should be administered to those with severe croup. - Bacterial tracheitis and epiglottitis must be considered in the differential diagnosis of viral croup.

Tx for croup

dexamethasone - 0.6 mg/kg/dose po (IV form orally, can also give IM) - max 16 mg - give alone for mild croup for decreased sx within 6 hrs Racemic epi - use when stridor at rest or severe upper airway obstruction - dose 0.05 - 0.1 ml/kg up to 0.5 ml max - observe up to 4hrs Mist therapy - tent at 40-100% humidified O2 for severe croup

A central venous catheter that is positioned appropriately flushes easily but fails to yield blood on withdrawal is most likely indicative of a

fibrin sheath

A 15 yr old girl with cystic fibrosis and difficult-to-manage pancreatic insufficiency is treated with pancreatic enzyme supplementation containing lipase, 10,000 IU/kg, and omeprazole, 20 mg/day, with improvement in her stools for 11 mo. She then develops severe constipation, abdominal pain, hematochezia, and vomiting. The most likely diagnosis is:

fibrosing colonopathy - Fibrosing colonopathy, consisting of colonic fibrosis and strictures, can occur 7-12 mo after high-dose pancreatic supplement therapy (6,500-58,000 IU lipase/kg/meal)

Which of the following is a risk factor for allergic rhinitis?

firstborn status - Up to 30% of individuals in the United States have allergic rhinitis, with increasing prevalence globally in industrialized countries. - Risk factors for allergic rhinitis include firstborn status, family history of atopy, male sex, and birth during pollen season. - Other risk factors are early antibiotic use, maternal smoking, serum immunoglobulin E > 100 international units/mL prior to age 6 years, and presence of allergen-specific immunoglobulin E.

As RBCs are lysed, they release hemoglobin. Heme molecules (from hemoglobin) are converted to bilirubin. Bilirubin (unconjugated or indirect) is bound to serum albumin and transferred to liver where it is conjugated to ...

glucuronate - by glucuronyl transferase - conjugated (direct) bilirubin excreted into bile

A 3 yr old boy presents with a 7-day history of fever, cervical lymphadenopathy, foul breath, and painful oral lesions on his tongue, gums, and lips. For the past 3 days he has had a red, painful swollen area about the nail of his right thumb with an area of fluid by the nail bed, unresponsive to warm soaks and a 1st-generation cephalosporin. The most likely etiologic agent is:

herpes simplex virus Herpes whitlow - HSV infection of fingers or toes, although strictly speaking it refers to HSV infection of the paronychia - Among children, this condition is most commonly seen in infants and toddlers who suck the thumb or fingers and who are experiencing either a symptomatic or a subclinical oral HSV-1 infection

What treatment can be used to decrease turbulent flow in children with severe croup?

inhaled oxygen and helium mixture

What can accelerate the onset of Tet spells?

iron deficiency anemia

A healthy, 15-year-old adolescent by is brought to the emergency department with concerns of acute intoxication. His parents found him several hours ago sitting alone in a bathroom filled with a pungent odor surrounded by drug paraphernalia. He was unable to respond coherently. On physical examination, his temperature is 37.5°F, heart rate is 110 beats/min, respiratory rate is 22 breaths/min, blood pressure is 130/85 mm Hg, and his oxygen saturation on pulse oximetry is 98% in room air. He is moving slowly and is slightly unkempt. He answers some questions appropriately but others nonsensically. He reports having auditory hallucinations. His physical examination findings are otherwise normal. Of the following, the MOST likely cause of this adolescent's symptoms is

marijuana - Acute marijuana intoxication may lead to hypertension, tachycardia, sedation, and psychosis. - Although treatment of toxicity from plant-derived substances is mostly supportive, antidotes to select toxins can be life-saving.

A 2 mo old infant presents to the emergency department with nasal flaring, wheezing, and respiratory rate (RR) of 70. RSV testing is positive. He is admitted to the floor and given IVF and nasal suctioning. This morning, he sleeps through your examination. RR is 45. He has not awakened to feed in 5 hr. The next step in management is:

obtain stat cap blood gas measurement - Nasal flaring is an extremely important sign of distress, especially in infants. - The state of responsiveness is another crucial sign. Lethargy, disinterest in surroundings, and poor cry are suggestive of exhaustion, hypercarbia, and impending respiratory failure.

The rash of neonatal lupus classically appears where?

on the scalp and periorbital areas - characterized by annular plaques that may undergo central atrophy - margins of the plaques may be raised - periorbital lesions may lead to a raccoon eye appearance - lesions typically regress between 6 and 8 months of age as maternal antibodies wane

A 4 yr old child has an acute illness with coryza, barky cough, hoarseness, and anorexia. There is no fever, and lower respiratory tract findings are normal. The most likely etiologic agent is:

parainfluenza virus - The most common types of illness due to parainfluenza infection consist of some combination of low-grade fever, rhinorrhea, cough, pharyngitis, and hoarseness and may be associated with vomiting or diarrhea

The triage history of a 9 mo old child presenting with a temperature of 39.2°C shows the immunization history as "up-to-date." Further review of the medical record shows that the patient has had 3 doses of Hib conjugate vaccine. Of the following immunizations, which is the most important to document before determining any management decisions?

pneumococcal conjugate vaccine - Guidelines for managing febrile children 3-36 mo of age who have received both Hib and S. pneumoniae conjugate vaccines have not been established, but careful observation without empirical administration of antibiotic therapy is generally prudent. - Empirical antibiotic therapy for well-appearing children < 36 mo of age who have not received Hib and S. pneumoniae conjugate vaccines and who have a rectal temperature of > 39°C and a WBC count of > 15,000/μL is strongly recommended.

Bolus feeds (gravity, pump) are contraindicated for...

post-pyloric feedings - large hyperosmotic volumes to jejunum can cause hyperperistalsis, cramping, diarrhea

A 6-week-old male infant is brought to the clinic for evaluation of fussiness. His mother reports that for the past week, he has been crying excessively. He usually cries in the evening for 3 hours and is difficult to console. She has tried swaddling and comforting her infant without success. He has been afebrile, breastfeeding well, and voiding and stooling normally. His physical examination yields normal findings. The mother asks which treatment may help her infant. Of the following, the BEST recommendation is

probiotics - Colic is common, is self-limited, and can be diagnosed with a careful history and physical examination. - First-line management of colic consists of parental support and infant soothing measures. - For breastfed infants who do not respond to standard measures and whose parents prefer to use probiotics, Lactobacillus reuteri may be considered after discussion of the uncertainties, risks, and benefits of its use.

A previously healthy 2-year-old girl is seen in the office for evaluation of bloody discharge from her left ear. She was seen earlier in the day at an urgent care center for ear pain. The urgent care provider could not visualize the left tympanic membrane because of the impacted cerumen. She used an ear curette to remove the cerumen and subsequently visualized a normal left tympanic membrane. After the visit, her mother noted blood coming from the left ear. The girl's vital signs are normal. Her physical examination findings are normal except for crusted blood in her left ear canal obscuring the tympanic membrane. Of the following, the BEST next step in management is to

provide supportive care - Blood coming from the ear can be alarming to parents. Fortunately, the most common causes are relatively benign and readily treatable. - Superficial ear canal irritation such as from an ear curette or scratching from seborrheic dermatitis is usually self-limiting, and parents can be reassured that hearing will not be impaired.

A 5 mo old previously well infant is found to have a loud holosystolic murmur (4/6) at the left sternal border. The 1st and 2nd heart sounds are normal; there is no tachycardia, rumble, or gallop; and hepatomegaly is not noted. The child feeds well and has grown adequately. You suspect:

restrictive VSD - clinical findings of patients with a VSD vary according to the size of the defect and pulmonary blood flow and pressure - small VSDs with trivial left-to-right shunts and normal pulmonary arterial pressure are the most common - loud, harsh, or blowing holosystolic murmur is present and heard best over the lower left sternal border, and it is frequently accompanied by a thrill

A 10-year-old child who was previously fully vaccinated undergoes chemotherapy followed by a hematopoietic stem cell transplant for high-risk acute myelogenous leukemia. He did not develop graft-vs-host disease, and he is now in remission and off all immunosuppressive and immunomodulating medications. His oncologists deem him safe for vaccination. Of the following, the MOST appropriate approach in terms of Haemophilus influenzae type b (Hib) immunization is to

revaccinate with a 3-dose regimen - Children who have received a hematopoietic stem cell transplant should receive a 3-dose series of Haemophilus influenzae type b vaccine regardless of age. - Primary routine vaccination against Haemophilus influenzae type b is given at 2, 4, and 6 months of age or at 2 and 4 months of age, depending on the manufacturer, with a booster at 12 to 15 months of age.

Tall (>2.5 mm), narrow, and spiked P waves are indicative of

right atrial enlargement - seen in congenital pulmonary stenosis, Ebstein anomaly of the tricuspid valve, tricuspid atresia, and sometimes cor pulmonale

most common type of salter-harris childhood fracture

salter-harris type II - 75% of physeal injuries - extends from physis into metaphysis - good prognosis - most common in hand and wrist

A healthy 24-month-old boy is seen for a routine health supervision visit. His mother reports that he maintains a healthy diet. He has no risk factors for nutritional deficiency. His development is normal as well as his physical examination findings. Routine lead screening reveals a level of 2.9 µg/dL (0.14 µmol/L). He is not anemic. These results are unchanged from the results of routine screening performed at 12 months of age. It is determined that the boy is not at a particularly high risk for lead exposure and that his risk will not change. The boy's mother recently gave birth to a term, healthy newborn. She wants to know what this result implies for her children. Of the following, the BEST advice to give the mother is

she should identify and eliminate common sources of lead exposure - Children with blood lead levels less than 5 µg/dL (0.24 µmol/L) who are at low risk for lead exposure and nutritional deficiency should still have sources of lead exposure identified.

A female infant born at 26 weeks' gestational age required tracheal intubation and ventilator management for 6 weeks. She then underwent extubation and was placed on nasal continuous positive airway pressure (CPAP) and eventually weaned to nasal cannula oxygen at 0.1 L/min. Since her discharge from the hospital at 4 months of age, the girl has had poor growth and repeated episodes of respiratory illness with noisy breathing, stertor, and coarse wheezing that does not respond to inhaled bronchodilators. She is currently 9 months old, and her growth trajectory has continued to decline since hospital discharge despite high-calorie infant formula and additional nutritional support. Of the following, the MOST accurate statement regarding this girl's symptoms is that

she will likely require tracheostomy and long-term ventilator management Tracheomalacia - common in premature infants - most congenital tracheomalacia will improve with time and maturity - critical tracheomalacia may require long-term intubation and mechanical ventilation

Apophysitis of the tibial tuberosity, also known as Osgood-Schlatter disease (OSD), is an overuse syndrome that occurs with repetitive traction of the patellar tendon on the open apophysis. Although OSD can occur in any child with open growth plates, it is most commonly seen in boys around the time of the adolescent growth spurt. Participation in a single sport throughout the year is a significant risk factor for OSD, thus the recommendation is...

spend at least 2 to 3 months per year away from structured sports participation - Young athletes need 1 to 2 days per week and 2 to 3 months per year of recovery time away from structured sports participation.

An 8-year-old is seen in the pediatric clinic for nausea and vomiting. While at school today, he vomited 3 times. The emesis was yellow and did not contain blood. He has had abdominal cramping and 1 watery stool. Earlier in the day he attended a breakfast at school where he consumed yogurt, granola, and pancakes. Several other classmates who attended the breakfast have similar symptoms. His vital signs are a temperature of 37.2°C, heart rate of 85 beats/min, respiratory rate of 16 breaths/min, and blood pressure of 98/60 mm Hg. Physical examination reveals increased bowel sounds and mild abdominal tenderness. Of the following, the pathogen MOST likely to be responsible for his illness is

staph aureus - The short incubation period of 2 to 4 hours for staphylococcal foodborne illness distinguishes it from many other foodborne infections. - Risk factors associated with food and waterborne infection outbreaks include consuming food in restaurants, hotels, and on cruises; consumption of unpasteurized dairy products or undercooked poultry; swimming in or drinking untreated fresh water; travel to a resource-limited country; and visiting a farm or petting zoo. - Infection with staphylococcal enterotoxins typically manifests as nausea and vomiting

suboxone vs subutex

suboxone - includes opioid (naloxone) - prescribed long-term - used during inpt & outpt subutex - no opioid blocker - used only during detox under medical supervision both - same active ingredient = buprenorphine - fights cravings - alleviates withdrawal symptoms

Acute onset of emesis (occurring over a period of 24-48 hours) should prompt consideration for

surgical causes of vomiting (small bowel obstruction, intussusception, appendicitis) foreign body or drug ingestion infections pancreatitis cholelithiasis or choledocholithiasis

In the NICU, method of calculating your TF if you do not expect any of your fluids to change at all (you are not titrating drips, no lines are coming out, etc) =

take the rate of all continuous fluids --> add them together --> multiply x 24 --> divide by weight

3 kinds of insulin pumps

tandem "T-slim" omnipod medtronic - pumps use only short acting insulin - pt's change their site (=fresh cath spot) every 2-3 days

The hallmark of puberty in boys is usually

testicular enlargement - followed by the development of pubic hair, penile growth, and more rapid growth in height.

Which is the most common cyanotic congenital heart disease?

tetralogy of fallot

Used for children requiring long term intubation

tracheostomy - tubes may be opaque plastic or metal - with or without external drum for humdification

Hepatitis C screening

universal screening for adults ages 18-79 - use anti-HCV Ab - confirm positive with HCV RNA PCR

Imaging for kid with.... chronic unexplained respiratory symptoms with low suspicion for foreign body

virtual bronchoscopy

A disease affecting all of the daughters but none of the sons of an affected father, and 50% of the sons or daughters of an affected mother, is most likely:

x-linked dominant - males are more commonly and more severely affected than females - female carriers are generally unaffected, or if affected, they are affected more mildly than males. - female carriers have a 25% risk for having an affected son, a 25% risk for a carrier daughter, and a 50% chance of having a child that does not inherit the mutated X-linked gene - affected males will have only carrier daughters. - affected males have no chance of having an affected son because they will pass their Y chromosome to their sons

What is the prevalence of lactose malabsorption in children younger than six years?

low

Most common site of origin for primary thoracic masses in pediatric population

mediastinum

Most common cause of viral croup

Parainfluenza

A Sedation Assessment Instrument for Infants and Young Children Supported on Mechanical Ventilation.

State Behavioral Scale (SBS)

Trophic feeding in NICU

'gut priming' - 10-20mL/kg - initiated in the first days of life if possible to promote GI maturation and reduce mucosal atrophy

In patients with a new diagnosis of tuberous sclerosis, which three diagnostic tests should be performed?

(1) Electroencephalogram (EEG), even if the patient is not having clinical seizures (2) magnetic resonance imaging (MRI) of the brain to look for hamartomas or other findings (3) abdominal imaging to look for renal angiomyolipomas.

When can varicella vaccine be used for post-exposure prophylaxis?

3-5 days after exposure

Ideal body weight calculation

(Height (cm)^2x1.65)/1000 Ideal body weight percent = Actual body weight (kg) / Ideal Body weight (kg) x 100

Mid-Parental Height

- size at 2 yo reflects mid-parental height Boys = [(Maternal Height + 5 in (13 in)) + Paternal Height]/2 Girls: = [Paternal Height - 5 in (13 in)) + Maternal Height]/2

Most common cause of inherited neonatal cholestasis

A1AT - leading genetic cause of liver disease in children - protein-folding disorder, toxic accumulation of insoluble protein

Age? - stoops to pick up toy - points to one part of body - uses 3 to 5 words - mature jargoning with real words

*15 mos* Also... - runs stiff-legged - creeps up stairs - walks carrying toy - builds 3 to 4 cube tower - places 10 cubes in cup - release pellet into bottle - uses spoon with some spilling - attempts to brush own ahri - turns pages of book - places circle in single shape puzzle - shows empathy - hugs adult in reciprocation - gets object from another room no demand

About one-third of all cases of narcolepsy present prior to which age?

*15 years* - Narcolepsy is categorized as type 1 (with cataplexy) and type 2 (without cataplexy). It typically presents prior to age 25, with about one-third of cases presenting before age 15. - All patients experience excessive daytime sleepiness, and some may also present with cataplexy, hypnagogic hallucinations, sleep paralysis, disturbed nighttime sleep, or neuropsychiatric symptoms such as depression or behavior problems. - Additionally, narcolepsy in children is often associated with precocious puberty and the abrupt onset of obesity. - Diagnosis in clinically suspected patients can be confirmed with nocturnal polysomnography and a multiple sleep latency test. - For children in whom cataplexy is the most debilitating symptom, sodium oxybate or antidepressants are recommended.

Recurrent otitis media (OM) is defined as

*3 episodes of acute otitis media (AOM) in the past 6 months or 4 episodes of AOM in the past 12 months, with at least 1 episode being in the past 6 months* - Prophylactic antibiotics are not recommended for children with recurrent AOM. - Conditions associated with recurrent OM include Down syndrome, Goldenhar syndrome, Treacher Collins syndrome, Turner syndrome, and diseases associated with ciliary dysfunction.

How long after injury does a wound develop its maximal tensile strength?

*3 months* - A wound develops its maximal tensile strength approximately 12 weeks after injury. - In undamaged skin, the epidermis and dermis form a protective barrier against the environment. When the barrier is broken, a cascade of events is set into motion to repair the tissue damage. These are divided into phases of blood clotting (hemostasis), inflammation, tissue growth (proliferation) and tissue remodeling (maturation). - The wound healing process is fragile and susceptible to interruption or failure, resulting in the formation of non-healing chronic wounds. Factors contributing to non-healing chronic wounds include diabetes, infection, venous or arterial disease, and metabolic deficiencies. - Many factors control the efficacy, manner, and speed of wound healing. These include local and systemic factors. A moist wound, a local factor, will heal more quickly and with less pain and scarring. Edema, ischemia, necrosis, foreign bodies, low oxygen tension, and perfusion may slow healing and result in increased pain. Systemic factors that may slow wound healing include inflammation, diabetes mellitus, deficient nutrients, metabolic disease, immunosuppression, connective tissue disorders, smoke, elderly, and illicit drug or alcohol use.

A child presents to you for a well-check, hopping into the room on one foot "like a bunny", as she describes it. While you are speaking with her mother, she draws a person with two parts. While examining her, she exclaims "Look at me!", while balancing on one foot for four seconds. She speaks in clear sentences and tells you a favorite story she reads with her parents. The mother reports she cannot yet skip. Based on these findings, what is the expected age of this child?

*4 years* - should be able to hop on one foot, balance on one foot for four seconds, throw a ball overhand, use scissors, and be adept at climbing. - should also be able to draw a person with two to four body parts, tell stories, and should be becoming more involved with make-believe play, role-playing, and storytelling. - should also be able to make a "gate" of five cubes, copy a square, and count up to four objects.

Age? - draws diamond - writes 1st and last name - understands seasons - best friend of same sex - knows days of the week

*6 years* - tandem walks - ties shoes - combs hair - looks both ways at street - remembers to bring belongings - draws 12-14 part person - simple addition/subtraction - sounds out words - plays board games - knows fantasy vs reality - can tell what words don't belong in a group - describes events in order - 10,000 word vocab

Infants normally demonstrate varus knee alignment (genu varum) caused by intrauterine positioning. Knee varus increases as children start to walk, but typically resolves by

2 years of age - at which point the knees should transition to valgus alignment (genu valgum)

When to start talking to parents about preschool plans for children...

2 years old

Polio vaccine (IPV) should be administered to healthy children at which ages?

2, 4, and 6-18 mo and 4-6 yr

Digital pain following the use of a metal cleaner may be an indication of hydrofluoric acid contact. What serum test should be ordered?

*Calcium* - Calcium becomes bound to hydrofluoric acid, and the resultant hypocalcemia may be fatal. - The symptoms of fluoride toxicity are quite rapid and chiefly gastrointestinal in nature. However, if the presentation is delayed, one may see both neurological and cardiovascular effects. - An ECG should be done stat because of hyperkalemia which may present as peaked T waves, widened QRS, bradycardia, or atrioventricular nodal blockade. In addition, hypocalcemia may present with QT prolongation. - Lavage with calcium solutions has been done but has not proven to be effective. There is not antidote to fluoride toxicity and the treatment is supportive.

1 day old, bili @ 14, direct is 0.5. Are you worried? --> Pathologic Jaundice = on 1st DOL, bili >12, d-bili >2, rate of rise >5/day ... Next best test?

*Coombs* - if positive --> means Rh or ABO incompatibility - if negative --> means twin/twin or mom/fetus transfusion, IDM, spherocytosis, G6p-DH deficiency, etc

A 4-day-old male infant presents with poor feeding, vomiting, and lethargy. On physical exam he is noticeably jaundiced with evidence of hepatomegaly. A blood culture sent on admission is growing Escherichia coli. He is found to have elevated levels of galactose-1-phosphate in his red blood cells. The infant is deficient in which of the following enzymes?

*Galactose-1-phosphate uridyl transferase* - The patient in the above clinical vignette has symptoms of classic galactosemia, which is caused by a deficiency in galactose-1-phosphate uridyl transferase. - Lactose is an ingested disaccharide that breaks down into glucose and galactose. Galactose is then converted to galactose-1-phosphate, which is then converted to glucose-1 phosphate by galactose-1-phosphate uridyl transferase. Impaired transferase activity leads to elevated levels of galactose and galactose-1-phosphate in the blood, causing the clinical signs of galactosemia. - Symptoms manifest in the first few days of life after feedings are initiated and include poor feeding, vomiting, and lethargy. - Physical examination findings demonstrate failure to gain weight, jaundice, hepatomegaly, and hypotonia. Infants with galactosemia are at higher risk for Escherichia coli sepsis. Cataracts, which are sometimes present at birth, develop due to galactitol deposition in the lens. Later complications from galactosemia include neurodevelopmental impairment, growth delay, and premature ovarian failure. - Diagnosis is made by measuring red blood cell galactose-1-phosphate uridyl transferase activity. In patients with some activity of the enzyme, symptoms may be less severe. The newborn screen can also detect galactosemia, but test results typically return after the infant becomes symptomatic. - Treatment involves immediate discontinuation of human milk and cow's milk and initiation of soy formula with the appropriate supportive care for other symptomatology, such as antibiotics, intravenous fluids, or phototherapy. In older children, milk and dairy products must be excluded from the diet. Consultation with a metabolic specialist and dietician may assist with the diagnosis and management and close neurodevelopmental follow up is necessary to assess the need for early intervention.

Primary survey

ABCDE: Airway Breathing Circulation Disability +/- dextrose - altered mental status, intentional moving all extremities, GCS (including pupils) Exposure - 2ndary survey = detailed head to toe

An immigrant from Sudan is seen with complaints of swelling in his left foot. His foot started to swell several months ago. Lately, he had noticed drainage from the left foot and associated with mild pain. He denies fever, weight loss, or any other illness. The physical reveals marked swelling of the left foot with numerous sinus tracts on the dorsal surface. The pulses are difficult to feel because of the swelling. Aspiration of the fluid from the subcutaneous tissues is sent for gram-stain. The organisms appear to exhibit a varying degree of acid fastness. The light microscopy and electron microscopy features of the aerobic gram-positive organism are shown below. What should the patient be asked that may confirm the diagnosis?

*Nocardia* - This patient has a mycetoma caused by Nocardia. Unlike Actinomycosis, Nocardia does exhibit varying degrees of acid-fastness and is aerobic. Both are filamentous and contain granules. - Mycetoma is uncommon in the United States but common in Africa and parts of the far east. It is also known as Madura foot. The disorder results in significant swelling of the subcutaneous tissues and sinus tracts. - In general, mycetoma occurs when people who walk barefoot are inoculated by fungal elements and other bacteria. The disorder is most common in farmers and sheepherders in Africa, India, and parts of the Middle East. - The disorder can cause marked disfigurement but is not fatal. The condition is often painless and regional lymphadenopathy is uncommon. It can mimic lymphedema.

Occurs with severe urinary tract obstruction or renal agenesis, which is associated with pulmonary hypoplasia (Potter's syndrome)

*Oligohydramnios*

Patients with which type of anxiety disorder typically present at the youngest age?

*Patients with selective mutism typically present at age five or younger.*

Common finding in well-appearing term neonates in the first 24 to 48 hours after birth. Does not correlate with hypoxemia. May be exacerbated by hypothermia and polycythemia.

*Peripheral cyanosis* - common in healthy newborn infants

Common side effects of cylosporine

- HT - renal dysfunction - hirsutism - gingival hyperplasia - Also: hyperkalemia, hypomagnesemia

Remember, at every birth you need to ask:

- How many babies? - What is the gestational age? - Is the fluid clear? - Are there other risk factors?

A 10-month-old boy has had three episodes of acute otitis media since birth, the last of which was treated one month ago. He presents with his mother for evaluation because he has had fever and fussiness over the past three days, and she suspects he may have another ear infection. His examination is remarkable for an opaque, erythematous, bulging right tympanic membrane. Additionally, he has diffuse xeroderma with patches of associated excoriation and erythema on his abdomen and back, and innumerable petechiae are visible on all extremities. Laboratory studies reveal a platelet count of 29,000/mm3. Which of the following is the most likely additional diagnostic finding in this child?

*Positive molecular genetic testing* - The clinical findings of eczema, thrombocytopenia, and recurrent infections in a male infant should raise suspicion for the diagnosis of Wiskott-Aldrich syndrome, an X-linked recessive disease. - The extent of eczema varies among patients, as does the severity and frequency of recurrent infections, which often develop after 6 months of age. - Classified as a primary immunodeficiency, Wiskott-Aldrich syndrome includes impaired B cell, T cell, and natural killer cell function. Recurrent infections may be bacterial or viral. - The response to polysaccharide antigens is reduced or absent, and there is particular proclivity for infections with polysaccharide-encapsulated organisms, such as Streptococcus pneumoniae. Hematologically, the thrombocytopenia is characterized by small platelets, and the diagnosis should be considered in any infant male with these findings on a complete blood count. - Definitive diagnosis can be confirmed with molecular genetic testing for the Wiskott-Aldrich syndrome protein gene.

A 1-year-old boy with fever and cough has a history of multiple bacterial infections, including pneumonia, otitis media, and a skin abscess. His parents describe multiple malodorous stools daily and a chronic diaper rash. He has a temperature of 38.5°C, heart rate of 140 beat/min, respiratory rate of 20 breaths/min, blood pressure of 90/60 mm Hg, weight of 7.5 kg (< third percentile for age), and length of 71 cm (third percentile for age). His weight-for-length percentile is 3%. He is fatigued and thin. His lung examination reveals left-sided crackles. His abdomen is distended but soft and nontender. He has an erythematous and ulcerated perianal rash. Laboratory data are shown: Laboratory Test Result White blood cell count 5,000/µL (5.0 × 109/L) Absolute neutrophil count 700/µL (0.7 × 109/L) Hemoglobin 8.8 g/dL (88 g/L) Hematocrit 27.3% Platelet count 112 × 103/µL (112 × 109/L) Pancreatic elastase < 50 µg/g stool Fecal fat (spot) 42% Ultrasonography of the abdomen demonstrates fatty replacement of the pancreas. Of the following, the MOST likely diagnosis is:

*Shwachman-Diamond syndrome* - exocrine pancreatic insufficiency (EPI) caused by Shwachman-Diamond syndrome, an autosomal recessive condition associated with skeletal abnormalities, neutropenia, and short stature. - He has findings of neutropenia and multiple infections, anemia, thrombocytopenia, short stature, and low fecal pancreatic elastase level (indicative of EPI). - Exocrine pancreatic insufficiency results when lipase secretion is less than 90% to 95% of the expected level and malabsorption occurs. Steatorrhea, excess fat in the stools, is often a presenting sign of EPI and is commonly accompanied by sequelae of fat malabsorption including weight loss, failure to thrive, and fat-soluble vitamin deficiency. - In young children, severe and persistent diaper dermatitis may be a sign of fat malabsorption. - While cystic fibrosis is the most common cause of exocrine pancreatic insufficiency in children, other causes include: chronic pancreatitis, Shwachman-Diamond syndrome, Pearson syndrome, and Johanson-Blizzard syndrome.

Oral antibiotic of choice for both prophylaxis and empiric treatment of infected human bite wounds.

Amoxicillin-clavulanate

Type of pulmonary disorder: Stridor - quiet Cough - none Voice - muffled Dysphagia/drooling - present Fever - high Toxicity - present Posture - neck extended, tripod position

*Supraglottic* disorders - epiglottitis - retropharyngeal abscess

Most important surfactant protein to have

*Surfactant protein B* - no *B* = *B*ad - Critical for absorption and spreading of surfactant

A newborn boy is born at 39 weeks gestational age via spontaneous vaginal delivery with no complications. On exam, his left foot is noted to be in a plantar-flexed position with an adducted hindfoot and forefoot, and the sole of the foot is facing inward. The abnormal positioning resists passive correction to anatomical position. Which is the correct diagnosis of this infant's condition?

*Talipes equinovarus* - commonly called "clubfoot." - incidence of about 1 in 1,000 live births - Treatment of congenital clubfoot involves casting and bracing for correction. - can be differentiated from a congenital clubfoot because the foot can be moved to a normal position with manipulation. - occurs due to the infant's position in utero. S - Serial casting may be performed using the Ponseti technique, followed by Achilles tendon lengthening.

4-week-old twin infant brought to your office for follow-up visit. Pt and his twin sister were born via vag delivery at 38 weeks of gestation after uncomplicated pregnancy. They are first children for their parents. During the visit, the baby's father expresses concern that the male twin does not make eye contact with his parents like his twin sister. He notes that although both twins will occasionally have eye crossing, his son's eyes seem to wander more often. Both twins will raise their chins off the floor when placed prone. Babies are breastfeeding well and are gaining weight appropriately. Both twins startle to loud noises, and their parents think that both will preferentially calm when they hear their mother's voice. MOST accurate assessment of twins' development is...

*The male twin shows evidence of isolated developmental delay* - atypical development in just 1 domain - typically developing 4-week-old infant lifts her chin when prone, holds hands fisted near mouth, sucks well, *makes eye contact*, follows face with eyes, recognizes mother's voice, startles to voice or loud sound, cries when in distress

What abnormality of hemoglobin can be seen in patients having a food protein-induced enterocolitis syndrome reaction?

Methemoglobinemia.

ECMO can be either...

*Veno-arterial (VA)* - blood drained from right atrium (via right internal jugular venous catheter) and is returned to thoracic aorta (via right carotids arterial catheter) - provides cardiac and pulm support *Veno-venous (VV)* - blood is drained from right atrium (via side holes of double lumen catheter) and returned to right atrium through end hole of catheter which is directed towards tricuspid valve - requires good cardiac function and avoids cannulation of carotid artery

2 forms of croup

*Viral* croup - most common cause of stridor - 3 months old - 3 years old - late fall, winter - male: female = 2:1 - begins with URI prodrome for 2-3 days, followed by stridor & cough *Spasmodic* croup - year round - preschool age children - hypersensitivity rxn

A 2-month-old male infant is brought to your office for a health supervision visit. When discussing vaccines, his mother reports that her daughter had an intussusception that was reduced by the radiologist. She would like to delay administration of the rotavirus vaccine for her son until 6 months of age.

*delaying this vaccine increases the risk of the complication about which the mother is concerned* - Rotavirus vaccine, an orally administered, live vaccine that is highly effective at preventing gastroenteritis caused by rotavirus, should be administered routinely at the 2- month health supervision visit. - There are age limits beyond which the vaccine should not be administered (14 weeks, 6 days for a first dose, 8 months, 0 days for the second or third doses); thus, delaying the vaccine could result in missed opportunities for protection. - There is a small, increased risk of intussusception with the first dose of the rotavirus vaccine, which has an acceptable risk-benefit ratio within the permitted age range.

A previously well 16-year-old adolescent girl who is a varsity athlete presents to the emergency department with new-onset exercise intolerance. Four days earlier, she competed successfully at a high school track meet. Over the next few days, her stamina during practice decreased and on the day of presentation, she became winded while climbing a flight of stairs. She has no other symptoms. Her last menstrual cycle ended 2 weeks ago and was normal in duration and intensity. She denies having seen any blood in her urine or stool. On physical examination, she is afebrile, with a heart rate of 123 beats/min and a blood pressure of 110/76 mm Hg. She appears pale and fatigued, has slightly icteric sclera, and a grade 2/6 systolic murmur. Her examination is otherwise unremarkable. Laboratory results are shown: Laboratory Test Patient Result White blood cell count - 11,000/μL (11 × 109/L) Hemoglobin - 3.9 g/dL (39 g/L) Mean corpuscular volume - 95 fL Platelet count - 455 × 103/μL (455 × 109/L) Reticulocyte count - 35% Of the following, the MOST appropriate next steps in management are to perform

*a direct antibody test, start prednisone, and transfuse the least incompatible packed red blood cell unit* Autoimmune hemolytic anemia (AIHA) is a true hematologic emergency. - Rapid diagnosis and initiation of therapy can be life-saving. - The adolescent girl in the vignette presents with signs and symptoms that strongly suggest AIHA. In particular, her tachycardia, fatigue, and exercise intolerance are likely due to hypoxemia of an acute onset. - The laboratory results show a severe, normocytic anemia with a marked reticulocytosis, suggesting a destructive process rather than a red blood cell (RBC) production failure. The reticulocyte count increases as the renal oxygen tension sensors detect a drop in oxygen-carrying capacity caused by the anemia. In response, they upregulate hypoxia-inducible factors, which in turn upregulate the production of erythropoietin, driving erythropoiesis. - The most appropriate next steps in her care would be to order a direct antibody test (DAT) and begin emergent management. The DAT will be positive if there are circulating antibody-coated RBCs. - It is important to note that in instances of brisk hemolysis from AIHA, the antibody-coated cells may be destroyed so quickly that the DAT is paradoxically negative. - Management of severe, life-threatening AIHA requires the emergent transfusion of a "least-incompatible" unit of packed RBCs, rapid initiation of immune suppression with corticosteroids, and ultimately identification of the autoantibody. The blood bank will perform compatibility testing on a number of units of packed RBC to determine which is least likely to be hemolyzed by the offending antibody.

A previously healthy 2-year-old boy presents to your office with several days of tactile fever and nasal congestion interfering with sleep, clear rhinorrhea, and cough. On physical examination, his temperature is 39°C, heart rate is 120 beats/min, and respiratory rate is 24 breaths/min. He is fussy, rubbing his eyes and ears, but is consolable. His voice is hoarse and his posterior oropharynx is erythematous without exudates. You note clear rhinorrhea and erythema of the nasal mucosa. The tympanic membranes are mildly hyperemic and retracted. There is shotty, bilateral cervical lymphadenopathy. Auscultation of the lungs reveals diffuse coarse upper airway sounds. The remainder of the physical examination is unremarkable. Of the following, the MOST appropriate therapy to recommend for this child is

*acetaminophen* The boy in the vignette has a common cold as evidenced by several days of fever, cough, congestion, and rhinorrhea with an otherwise reassuring physical examination. Because of fever and fussiness, the most appropriate therapy to recommend for this child is acetaminophen. Both the Food and Drug Administration and the American Academy of Pediatrics advise that over-the-counter (OTC) cough and cold medications including antihistamines (diphenhydramine), decongestants (pseudoephedrine), antitussives (dextromethorphan), and expectorants (guaifenesin) should not be used in children younger than 2 years; consensus opinion extends this recommendation to children younger than 6 years.

A 12-year-old boy presents to your office for evaluation after sustaining a puncture wound to his left foot from a rusty nail. Two days ago, while helping his father clean out an old barn, the boy stepped on a rusty nail, which pierced through his tennis shoe and punctured his left forefoot. Within an hour after this injury, he cleansed the wound with tap water and applied a bandage to it. His mother convinced him to come to your office for evaluation today after she noticed that the skin surrounding the wound was becoming red and warm. The patient denies any history of fever. A review of his chart reveals that he has a history of well-controlled type 1 diabetes, as well as mild intermittent asthma. He receives subcutaneous insulin via an insulin pump, and uses albuterol only occasionally for his asthma symptoms. He has had 4 prior tetanus-containing immunizations; the most recent one was administered when he was 5 years of age. In your office, the patient is afebrile and appears well. His vital signs are within normal limits for his age. His physical examination findings are significant for a 5-mm puncture wound on the sole of his left forefoot, which is surrounded by an area of erythema that is about 1 cm in diameter. His skin feels warm and is tender to palpation in this area. There is no bleeding from the wound, but the patient reports that he noticed some yellow drainage on the bandage that he changed earlier today. The remainder of his physical examination findings are unremarkable. You thoroughly irrigate the wound in your office, and debride a small amount of necrotic tissue around the puncture wound. Of the following, the MOST appropriate care regimen for this patient includes

*administration of tetanus toxoid, diphtheria toxoid, and acellular pertussis (Tdap) immunization and prescription of oral ciprofloxacin* - The boy in the vignette presents with a puncture wound to his left foot after a rusty nail penetrated through his tennis shoe while he was working in an old barn. The erythema surrounding his puncture wound and yellow drainage indicate development of an infection. - Because this child's puncture wound occurred through a tennis shoe, he is at risk for infection with Pseudomonas aeruginosa. - The most appropriate care regimen for this boy includes administration of tetanus toxoid, diphtheria toxoid, and acellular pertussis (Tdap) immunization and prescription of oral ciprofloxacin.

A 17-year-old female soccer player is brought to the emergency department (ED) following a head injury that occurred while she was playing in a soccer game. Approximately 20 minutes ago, she collided head-to-head with another player while she was running and fell to the ground. Her mother, who accompanied her daughter to the ED, tells you that she had a very brief loss of consciousness (less than 15 seconds) immediately following the injury and that she has seemed "dazed" since it occurred. She vomited once about 10 minutes ago. A review of the patient's medical history indicates that she has no significant past medical or surgical history, takes no medications, has no allergies, and has had no prior head injuries. In the ED, the patient is sleepy, but answers all questions appropriately and follows instructions. When you ask her about her current symptoms, she states: "My head still hurts, but it is starting to feel a little better now." Her vital signs are within normal limits. On physical examination, her pupils are equal in size and reactivity. She has a 3 x 4 cm area of ecchymosis near the center of her forehead, but no hematomas or step-offs on palpation of her entire forehead and scalp. There are no focal deficits on a complete neurologic examination, although she tells you that she feels tired and wants to lie down again after you ask her to walk back and forth across the room. The remainder of the physical examination is unremarkable. Of the following, the MOST appropriate next step in the management of this patient is to

*continue to observe her in the emergency department* - While computed tomography (CT) of the brain is a rapid and accurate way to identify intracranial injuries in children after head trauma, use of this diagnostic modality has downsides, including exposure to ionizing radiation, identification of minor lesions or incidental findings with unclear clinical importance, the need for sedation for younger or uncooperative pediatric patients, and significant increases in healthcare costs - The goal of pediatric providers should be to identify children with clinically important intracranial injury after head trauma to prevent deterioration and secondary brain injury, while limiting unneeded radiographic imaging in children at low risk. - For patients falling into the "intermediate risk" category for intracranial injury, observing patients clinically before obtaining computed tomography of the brain allows providers to selectively image only those whose symptoms worsen or fail to improve.

A 2-year-old African American boy presents to your clinic for evaluation of fever. His mother states that he has had fever up to 39°C that started 5 days ago and his eyes and lips are red. The boy had 1 episode of vomiting yesterday and 1 watery stool today. He is eating and drinking less than usual, but urinating normally. The boy has a sibling that is currently well. On physical examination, the boy is irritable. His temperature is 39.2°C, heart rate is 120 beats/min, and his blood pressure is 80/50 mm Hg. He has bilateral, nonpurulent conjunctivitis. His lips and tongue are red with hypertrophied papillae. He has a morbilliform rash on his chest. The dorsum of his hands and feet appear edematous. The remainder of his physical examination is unremarkable. You order laboratory studies. His erythrocyte sedimentation rate is 40 mm/h and his white blood cell count is 15,200/µL (15.2 x 109/L). His other laboratory results are normal. Of the following, the MOST concerning feature of the boy's presentation that warrants further evaluation is his

*duration of fever* The boy in the vignette has Kawasaki disease (KD). The most concerning feature that requires further evaluation of his symptoms is the duration of fever. The boy has several features of KD including fever of 5 days' duration, changes in his extremities, polymorphous rash, conjunctivitis, and mucosal findings. He has a slightly elevated erythrocyte sedimentation rate, which is nonspecific and could occur with any type of immune system activation, including a viral infection. The boy's tachycardia is likely caused by fever and not something that would necessarily warrant further workup. He had vomiting and diarrhea only once and urination is normal. The boy's white blood cell count is only slightly elevated and is not concerning in the setting of an acute illness. The differential diagnosis of a child with this presentation is broad. Early in its course, KD mimics several conditions, including several viral illnesses that can present similarly, such as adenovirus, enterovirus, influenza, roseola infantum, and Epstein-Barr virus. In very young or unimmunized patients the practitioner must consider measles. The diagnostic criteria for KD include fever for at least 5 days, with at least 4 of the following: bilateral, non-exudative, bulbar conjunctivitis; oropharyngeal changes; cervical lymphadenopathy; polymorphous rash; and peripheral extremity changes. The oropharyngeal changes can include strawberry tongue, erythema of the oropharyngeal mucosa, or erythematous or cracking lips. The extremity changes include erythema or edema of the palms and soles in the acute phase or periungal desquamation of the fingertips in the later subacute phase of KD. The diagnosis can be difficult in cases where the patient has fever for 5 or more days but does not meet criteria for diagnosis. Incomplete KD is the term used for patients with fever for 5 or more days, but with only 2 or 3 of the principal clinical features. In such cases, echocardiography is recommended. KD can be diagnosed in patients when coronary artery disease is detected with 2-dimensional echocardiography. Other laboratory study abnormalities that support a diagnosis of KD are low albumin, elevated alanine aminotransferase, significantly elevated platelets, elevated white blood cell count, or sterile pyuria. Risk factors for coronary artery aneurysms include male sex, age younger than 1 year, prolonged fever, delayed diagnosis, fever that persists after treatment, low hemoglobin, high white blood cell count, high absolute band count, very high erythrocyte sedimentation (ESR) or C-reactive protein, low platelet count, or low albumin. It should be noted that after intravenous immunoglobulin treatment, ESR is no longer helpful in determining the level of inflammation.

A 6-year-old boy presents because of diarrhea for the past year. The child was toilet trained at age 3 years, but he has been soiling his underwear for the past year. During meals, he frequently complains of abdominal pain, and his abdomen becomes distended. He has experienced no fevers, other illnesses, or weight loss. Physical examination demonstrates a well-developed child whose height is 115 cm (50th percentile) and weight is 19 kg (25th percentile). He has a protuberant abdomen and normal bowel sounds with a fullness noted in the left lower quadrant. Rectal examination is deferred because of a large amount of soft stool in the child's underwear. Of the following, the MOST likely cause of this child's symptoms is

*fecal overflow incontinence* - In the vignette, a 6-year-old boy presents with a year-long complaint of abdominal pain during meals and daytime fecal soiling. His growth and development are normal, and additional history demonstrates that he was toilet trained at 3 years of age. Although a thorough history is required as part of his complete evaluation, with particular emphasis on the time after birth of meconium passage and his subsequent stooling pattern, the information in this vignette strongly suggests a diagnosis of encopresis secondary to chronic constipation. The physical finding of a stool mass in his left lower quadrant, plus the presence of semiliquid stool in the boy's underwear, further suggest that the "diarrhea" reported by the parents represents overflow fecal incontinence. - Encopresis is defined as fecal soiling in children who are beyond the age of toilet training. Functional fecal incontinence in children may be further classified as either primary, affecting children who have never been toilet trained, or secondary, as in the vignette, presenting in the child where incontinence returns after successful toilet training. In most patients, considering each child's neurodevelopmental status, this diagnosis may be applied to soiling episodes that occur in the child older than 4 years of age. In 90% of cases, encopresis occurs as the consequence of chronic constipation in a child who demonstrates stool-withholding behavior. Although encopresis is estimated to affect 1% to 2% of children under the age of 10 years, with an 80% male predominance, a complaint of constipation and related problems may account for more than 25% of all visits to a pediatric gastroenterologist. In otherwise healthy children, incontinence is most likely to represent fecal overflow around a retained stool mass. Soiling may be reported as diarrhea by some parents; however, a pattern of stool-withholding behavior will usually be elicited, indicating a "nonorganic" diagnosis of functional constipation-related encopresis.

41 wk AGA infant was born after ROM yielded greenish-brown fluid ....

*meconium aspiration syndrome* - next best step --> intubate & suction before stimulation - complications --> pulmonary artery HT, pneumonitis

A 12-month-old boy is brought to the emergency department because of bluish discoloration of the skin. The mother first noticed the child's lips turning slightly blue 2 days ago. His lips and mucous membranes have become progressively blue and now involves the fingernails and extremities. The mother did not seek medical attention sooner because he otherwise seems like his normal self. There is no cough, runny nose, difficulty breathing, red mucous membranes, or rash. He has been fussier for the past week because he is teething. He has been normally active and eating and drinking well, and he does not fatigue easily. He is a product of a full-term delivery with no problems. He has been thriving and has never been hospitalized. His immunizations are up to date, and he has no allergies. Medications include multivitamin drops, as well as as-needed doses of acetaminophen and topical benzocaine for teething pain. Vital signs show a temperature of 37.5°C, heart rate of 140 beats/min, respiratory rate of 30 breaths/min, blood pressure of 90/50 mm Hg, and SpO2 of 85% on room air. Physical examination shows a generally well-developed, well-nourished child in no apparent distress. Oral mucous membranes are moist and not erythematous, but have a blue discoloration. He is breathing comfortably, and his lungs are clear to auscultation bilaterally. His heart has a regular rate and rhythm with no rubs, gallops, or murmurs. Extremities are warm and well perfused with no edema. There is obvious cyanosis of lips, nail beds, and skin. There is no digital clubbing. Of the following, the MOST likely cause of his cyanosis is

*methemoglobinemia* - The child in the vignette has methemoglobinemia, marked by bluish discoloration of the lips and mucous membranes, oxygen saturation of 85%, and no evidence of primary pulmonary dysfunction. In this case, the methemoglobinemia is likely because of the topical benzocaine that was given for teething. - Pulse oximetry (SpO2) is the most common technique used to monitor oxygenation. Pulse oximetry is noninvasive, therefore it is used to monitor children during surgery, transport, procedural sedation, and critical illness. Pulse oximetry estimates the saturation of hemoglobin with oxygen by measuring the differential light absorption of oxyhemoglobin and deoxyhemoglobin at 660 nm (red) and 940 nm (infrared) wavelengths. This technique requires pulsatile flow to detect oxyhemoglobin, estimating arterial saturation (Sao2). Changes in arterial pressure of oxygen (PaO2) higher than 80 mm Hg are not likely to reflect changes in Sao2 because of the shape of the hemoglobin-oxygen dissociation curve (Item C232). Also, the same PaO2 level may lead to different SpO2 levels, because the affinity of hemoglobin to oxygen is dependent on various factors. For example, acidosis, higher temperature, and elevated levels of 2,3-bisphosphoglyceric acid (2,3-BPG) lowers the affinity of hemoglobin to oxygen, favoring its unloading in tissues. In contrast, alkalosis, lower temperature, and lower 2,3-BPG increase the affinity of hemoglobin to oxygen, favoring its uptake. = Pulse oximetry should not be the only method of monitoring patients with primary ventilatory failure, such as neuromuscular conditions and central nervous system depression, because patients with satisfactory SpO2 may have dangerous levels of hypercarbia if they are receiving supplemental oxygen. Other limitations of pulse oximetry include decreased signal from motion, external light, anemia, and decreased perfusion and pulsatility. - Methemoglobin is the oxidized, ferric (Fe3+) state of hemoglobin, as opposed to the normal ferrous (Fe2+) state. It does not have oxygen-carrying capacity, and thus does not unload oxygen to the tissues. The oxidation of hemoglobin to methemoglobin is caused by topical anesthetics, gastroenteritis, exposure to nitric oxide, or ingestion of nitrite-containing well water. Methemoglobin levels higher than 20% can lead to symptoms of fatigue, dizziness, and nausea. Life-threatening manifestations, such as arrhythmias, seizures, and altered mental status, may occur at levels greater than 45%. Treatment includes removal of the inciting agent, and administration of methylene blue, which is a cofactor of nicotinamide adenine dinucleotide phosphate (NADP)-methemoglobin reductase. Pulse oximetry may provide inaccurate information about SpO2 in methemoglobinemia because pulse oximetry recognizes all types of hemoglobin as either oxyhemoglobin or deoxyhemoglobin. Methemoglobin absorbs light in both the oxygenated and deoxygenated wavelengths. As a result, the reported SpO2 by pulse oximetry is typically around 85%, irrespective of the true SpO2. Inaccurate information may also be provided by pulse oximetry in the presence of carboxyhemoglobin because it absorbs light in the same (red) wavelength as oxyhemoglobin, leading to overestimation of the oxygen saturation. Diagnosis of methemoglobinemia and carboxyhemoglobinemia is best established by co-oximetry, which involves blood gas analysis of oxyhemoglobin, deoxyhemoglobin, methemoglobin, and carboxyhemoglobin levels.

A 6-year-old girl tearfully discloses to her mother that an adult male cousin has, on multiple occasions, put his finger in her vagina. The last episode occurred approximately two weeks prior to the disclosure. The mother calls the police and the pediatrician, and Child Protective Services is made aware of the situation. The pediatrician completes a thorough review of systems and physical examination with no significant medical findings. Which of the following is appropriate management for this scenario?

*ongoing psychosocial support for victim and family* In prepubertal children, forensic evidence can be collected up to 72 hours after the most recent alleged incident. A thorough physical examination by a child abuse medical expert or the patient's primary pediatrician is indicated. When examining the genital area, young children can be examined in the "frog-leg position" and in the lateral decubitus position, with the patient's knees tucked in toward the chest, to examine the anorectal area. In prepubertal children who are asymptomatic, prophylactic treatment for sexually transmitted infections and human immunodeficiency virus is typically not indicated unless there is significant concern from the physician. The abused child's physical safety needs to be guaranteed to prevent recurrences. To address the child's emotional well-being, a social worker who is experienced in child sexual abuse should be available during the initial evaluation. Referral to a mental health specialist or counselor to provide ongoing psychosocial support for the victim and family is essential, as psychological sequelae is common.

A 10-year-old girl presents for poor linear growth and slow weight gain. In the last year, her linear growth has stopped completely, and she continues to struggle to gain weight. Review of systems reveals frequent fatigue, anorexia, and a decreased energy level. She has also had occasional episodes of emesis in the morning that are becoming more frequent. Physical examination shows some darkening of her skin and mucous membranes. The remainder of her examination is unremarkable. Initial laboratory testing shows a serum sodium concentration of 131 mEq/L (131 mmol/L), potassium concentration of 5.8 mEq/L (5.8 mmol/L), and a glucose level of 60 mg/dL (3.3 mmol/L). The rest of her chemistry results are unremarkable. Complete blood cell count shows eosinophilia. Of the following, the BEST next test to determine her underlying diagnosis is

*serum cortisol* The child in the vignette has Addison disease, the most common cause of adrenal insufficiency in the industrialized world. Classic chronic symptoms include weakness, fatigue, anorexia, nausea, abdominal pain, and diarrhea. Poor growth can also occur, as seen in this child. Hyperpigmentation of the skin and mucous membranes can develop over time. Acute symptoms can include muscle, joint and abdominal pain, and hypotension. Laboratory evaluation may reveal hyponatremia, hyperkalemia, and hypoglycemia if the child becomes ill and develops an adrenal crisis. Sometimes the complete blood cell count (CBC) will reveal anemia and eosinophilia as well. Serum cortisol will be very low, revealing the diagnosis of adrenal insufficiency. Other supportive testing includes elevated adrenocorticotropic hormone (ACTH) levels (frequently > 100 pg/mL [22 pmol/L]), and ACTH stimulation testing can reveal a low cortisol response (< 18 μg/dL [497 nmol/L]). Mineralocorticoid deficiency is confirmed with a relatively low aldosterone value in the face of hyperreninemia. Adrenal antibodies can also be measured to confirm the diagnosis of Addison disease as the underlying cause of adrenal insufficiency.

You are taking care of a newborn in the neonatal intensive care unit who has had bluish discoloration since birth 1 hour ago. He is the product of an uneventful pregnancy and normal spontaneous term vaginal delivery. Apgar scores were 7 at one min and 8 at five min. Vital signs show a temperature of 37°C, pulse rate of 140 beats/min, blood pressure of 70/30 mm Hg, respiratory rate of 30 breaths/min, and SpO2 of 75% on room air. Physical examination shows a well-appearing newborn who has cyanotic lips, trunk, and extremities. He has good tone, normal cry, and moves all extremities. He is breathing comfortably, and lungs are clear to auscultation bilaterally. He has warm and well-perfused extremities. His heart has a regular rate and rhythm. When he is placed on 100% supplemental oxygen, his pulse oximetry reading does not change. Of the following, the MOST likely cause of his cyanosis is

*transposition of great arteries* - The newborn in the vignette has cyanosis from transposition of the great arteries (TGA), which is the most common cyanotic congenital heart lesion presenting at birth. As the newborn was breathing comfortably and placement in 100% oxygen did not improve his oxygen saturation, the cyanosis is more likely cardiac in origin. Nonpulmonary causes of cyanosis are listed in Item C250. - Cyanosis is a bluish discoloration of the skin that is visible centrally in the presence of more than 5 g of deoxygenated hemoglobin per dL of blood. When deoxygenated systemic venous or pulmonary arterial blood is shunted to the systemic arterial system without getting oxygenated, it can originate from a pulmonary cause or a nonpulmonary cause. Intrapulmonary shunting is typically caused by ventilation-perfusion mismatching in which areas of the lung are perfused but not ventilated. This can be caused by pneumonia, increased diffusion barrier, atelectasis, and pulmonary edema. The oxygen saturation in most instances of intrapulmonary shunting can be improved by increasing the fraction of inspired oxygen (FiO2). By increasing the alveolar pressure of oxygen (PO2), a higher driving force for diffusion of oxygen into the pulmonary capillaries will increase the arterial PO2. In contrast, increasing the FiO2 in a patient with an intracardiac shunt without significant lung disease will not compensate for amounts of blood that are directly shunted right to left, and the patient will remain cyanotic. Thus, increasing the FiO2 can help differentiate these 2 causes. - Transposition of the great arteries is the most common form of cyanotic congenital heart disease that presents at birth. Oxygenated pulmonary venous blood is not readily able to enter the systemic circulation because it is ejected back into the pulmonary circulation from the left ventricle. In order for survival, mixing must occur at the atrial level, ventricular level, or via the ductus arteriosus to allow oxygenated blood to be sent to the systemic circulation. An infusion of prostaglandin E1 is necessary for newborns with TGA to maintain ductal patency. Other conditions that can present with cyanosis at birth include pulmonary valve atresia and severe Ebstein malformation. Chest radiography can be helpful in distinguishing among these conditions because TGA may show evidence of increased pulmonary blood flow, whereas pulmonary atresia and Ebstein malformation typically show decreased pulmonary blood flow. Primary pulmonary hypertension in the newborn with right-to-left shunting across the ductus arteriosus can also cause cyanosis. This condition, in the absence of structural heart disease, can be caused by sepsis, asphyxia, meconium aspiration, diaphragmatic hernia, and pulmonary hypoplasia. Pulmonary hypertension can also be a cause of cyanosis because of decreased pulmonary blood flow. It can be primary in nature or secondary to increased pulmonary vascular congestion from heart failure, left ventricular outflow tract obstruction, or large left-to-right shunts.

A 14-year-old boy presents with concern for osteomyelitis of the femur. Initial evaluation includes radiographs, inflammatory markers, a complete blood count, and blood cultures. His mother is concerned about the possibility of bone biopsy and hopes that a pathogen can be identified from the blood culture. Which of the following is most strongly associated with increased blood culture yield of a pathogen?

*volume of blood in the culture* - The yield of blood cultures is highly dependent on the volume of blood in cultures. The blood culture yield increases significantly if more than 5 mL are inoculated into blood culture bottles. - Larger children and adolescents have larger blood volumes and can safely provide blood cultures of much higher volumes, sometimes up to 30 mL per culture bottle. - In addition, the yield of pathogens increases with the number of cultures collected.

Blood volume of a child

*~80 mL/kilo* The TBV of a child is around 75-80 ml/kg and is higher in the neonatal period (from 85 ml/kg it rises to a peak of 105 ml/kg by the end of the first month and then drops progressively over ensuing months).

Powder formula - preparation: - stability prepared: - stability opened/unprepared:

- 2oz of water + 1 unpacked level scoop of formula (US formula) --> ex. could do 4oz water + 2 unpacked level scoops of formula - 24 hours (refrigerated) - 1 month (room temp)

Recognize allergic contact dermatitis

- A linear distribution of lesions supports the diagnosis but is not always present. - The presence of a persistent dermatitis despite topical corticosteroid treatment should raise suspicion of allergic contact dermatitis. The allergen responsible often results in a rash with a characteristic location. - The treatment of mild allergic contact (Rhus) dermatitis is aimed at reducing pruritus.

Initiation of feedings, their volume, and the rate of advance of feedings are related to birth wt, gestational age, and how the infant has tolerated feeds to date. General guidelines include:

- Initial volume is 2 cc/kg per feeding with minimal absolute volume of 2 cc - don't advance feedings faster than 20 cc/kg/d - bolus feedings preferable to continuous feedings - goals for "full feedings" are: volume of 150-160 cc/kg/d, calories of 110-120 kcal/kg/d

Liquid concentrate formula - preparation: - stability prepared: - stability opened/unprepared:

- diluted 1:1 with water - 24 hours (refrigerated) - 48 hours (refrigerated)

Softners/surfactants used for constipation

- docusate (colace) - glycerin suppository

colace

- ducosate - stool softener - Relief of occasional constipation (OTC products; FDA approved in ages ≥2 years and adults; consult specific product formulations for appropriate age groups)

Children with moderate to severe traumatic brain injury require intracranial pressure monitoring and treatment in the critical care unit to prevent progressive swelling. First-line therapies include:

- elevation of the head of the bed - ensuring midline positioning of the head - controlled mechanical ventilation - sedation and analgesia (i.e., benzodiazepines and narcotics)

Goals of norwood (for hypopolastic left heart syndrome)

- establish reliable way for blood to get rest of body - atrial septum open - reliable blood flow to lungs - stage 2 is glenn procedure (at 6-9 mos) ~ O2 sat n 80s - be weary of giving them albuterol because of risk of arrhythmias - fontan (3-5 yo) ~ normal O2 sat - stage 4 = transplant

RSI - induction agents

- etomidate 0.3 mg/kg (better for cardiac issues) --> quick acting and can get exam after very quickly; hemodynamically stable; use sedative with it because can get myoclonus - ketamine 2.0 mg/kg - propofol 1.5 mg/kg - midazolam 0.2-0.3 mg/kg IV push

The apnea test assesses the function of the medulla in driving ventilation. It is performed by first ensuring the adequacy of hemodynamics and temperature and the absence of apnea-producing drug effects or significant metabolic derangements. The test is performed by ...

- first preoxygenating the patient with 100% oxygen for approximately 10 min - adjusting ventilation to achieve a PCO2 of about 40 mm Hg

Urea cycle disorders present with

- immediate decompensation in the first 24 to 72 hours after birth - progressive respiratory alkalosis - obtundation - hyperammonemia in the presence of a normal anion gap

What indicators in an infant with hypoxic ischemic encephalopathy is associated with the worst prognosis?

- initial cord or initial blood pH < 6.7 - Apgar scores of 0-3 at 5 min - high base deficit (>20-25 mmol/L) - decerebrate posture - lack of spontaneous activity

umbilical artery catheter on imaging...

- left side - loop b4 coming up

Osmotics used for constipation

- magnesium - sorbitol - lactulose - polyethylene glycol (miralax)

What are some methods of testing for exogenous testosterone exposure?

- urinary ratio of testosterone glucuronide to epitestosterone glucuronide - determining the carbon 13/carbon 12 ratio - measuring the urine testosterone to luteinizing hormone ratio

Lab abnormalities in prerenal AKI

- urine specific gravity is greater than 1.020 - urine sodium level is less than 10 mEq/L - fractional excretion of sodium (FENa) less than 1% - urine to plasma osmolality is greater than 1.5

Recognize cold panniculitis

- via direct contact of skin or mucosal folds to cold --> local trauma - erythematous plaque with areas of induration - can have bluish hue - resolves on its own over 2-3 weeks, but hyperpigmentation can last for several months - most common locations = cheeks and chin

The diagnostic criteria (Rome IV) developed by the Rome Foundation for pediatric IBS include

1. Abdominal pain more than 4 times monthly associated with defecation, change in frequency of stool, and/or change in stool appearance 2. Continued abdominal pain despite treatment of constipation (when constipation is present) 3. After appropriate evaluation, the symptoms cannot be fully explained by another medical condition.

Stop bottle feeding at what age?

12-15 months

Biting incidents decrease after what age?

2.5 years of age

What OCP to start a female patient on usually...

30 mcg estrogen pill (low dose)

Overweight BMI for kids

>85-94%

Which race has the highest incidence of preauricular pits?

Asians

In neonate, from a clinical perspective, a chest radiograph with high degree of cardiomegaly and decreased pulmonary blood flow, should raise the suspicion for

Ebstein anomaly

Imaging for kid with.... bronchiectasis

HRCT

Imaging for kid with.... measuring airwar thickness

MDCT

What gene is responsible for Rett syndrome?

MECP2

PPD testing can interfere with what vaccine?

MMR - delay TST screening at least 4 weeks after MMR vaccine

What is the first-line abortive treatment for migraine headaches?

NSAIDs or Tylenol

Recognize respiratory papillomatosis

Respiratory papillomatosis - via vertical transmission of human papillomavirus (types 6 or 11) from the genital tract of a mother to her child - hoarseness, stridor, and abnormal cry; severe forms can lead to airway obstruction - low potential for malignant transformation.

Most frequently implicated community respiratory viral pathogen causing the common cold in children and adults.

Rhinovirus - Occurs throughout the year, with peak activity from autumn through spring. - In addition to the common cold, rhinoviruses can cause otitis media, bronchiolitis, and pneumonia in infants and children.

What is the definition of cholestasis?

Serum conjugated bilirubin ≥ 2 mg/dL.

A 12 yr old tall afebrile girl presents with hip pain and an extremely rotated right leg. On examination, internal rotation is limited. The most likely diagnosis is:

Slipped capital femoral epiphysis (SCFE) - The classic patient presenting with an SCFE is an obese, African-American boy between the ages of 11 and 16 yr. - Girls present earlier, usually between 10 and 14 yr of age. - Patients with chronic and stable SCFEs tend to present after weeks to months of symptoms. Patients usually limp to some degree and have an externally rotated lower extremity. - Physical examination of the affected hip reveals a restriction of internal rotation, abduction, and flexion. - Commonly, the examiner notes that as the affected hip is flexed, the thigh tends to rotate into progressively more external rotation. - Most patients complain of groin symptoms, but isolated thigh pain or knee pain is a common presentation from referred pain along the course of the obturator nerve. - Children typically refuse to allow any range of motion of the hip; much like a hip fracture, the extremity is shortened, abducted, and externally rotated.

Wasting vs Stunting

Wasting = low weight for age or height Stunting = shorter tha normal

Although some children can perform spirometry as young as 3 years old, consistent results are more likely ....

after 6 years old

90% of alveolar development occurs when during development?

after birth

What type of shock is septic shock

distributive shock - more vasomotor changes - lose tight junctions = signals from cytokines --> let go --> increased capacitance

Peripherally Inserted Central Catheters (PICC) are inserted through...

arm vein (cephalic, basilic, brachial vein) with tip at SVC-RA junction or leg vein with tip at IVC-RA junction - PICCs tend to be small fine lines (3 French) and can easily fracture and embolize

The most common reason for people with intellectual disability to be placed out of the home is:

behavioral disturbance - Most children with an intellectual disability do not have a behavioral or emotional disorder as an associated impairment, but challenging behaviors (aggression, self-injury, oppositional defiant behavior) and mental illness (mood and anxiety disorders) occur with greater frequency in this population than among children with typical intelligence. - These behavioral and emotional disorders are the primary cause for out-of-home placements, reduced employment prospects, and decreased opportunities for social integration.

For ingestions that cause seizure, treat with...

benzos unless otherwise specified

What is the most common site of infection in patients with fever and chemotherapy-induced neutropenia?

bloodstream

In an older child with galactosemia, what type of mineral supplementation is needed after the child is weaned off soy formula?

calcium

In a child without congenital heart disease, what are the signs of a pulmonary hypertensive crisis?

cardiovascular collapse - Without a "pop-off" such as a PFO or a VSD, then increased PVR will lead to cardiovascular collapse as there will be insufficient pulmonary blood flow and thus insufficient LV preload. - In order to have hypoxemia, you would either have to have an intracardiac lesion to shunt R to L or increasing intrapulmonary shunt. ETCO2 decreases, not increases, with pulmonary hypertension as the dead space fraction increases.

What is the treatment of choice for meningococcemia?

ceftriaxone

Most common cause of persistent hypoglycemia in neonatal period

congenital hyperinsulinism - group of congenital disorders - presentation = macrosomia, poor feeding, +/- neuro delays, persistent hyperinsulinemic hypoglycemia, high GIR

Typical albuterol dosages in peds asthmatics

continuous nebulization = 5-20 mg/hr nebulization = 2.5 mg MDI = 2-8 puffs (4-6 puffs = 2.5 mg neb)

Most VLBW and almost all ELBW infants will require an umbilical arterial catheter for blood sampling and blood pressure measurement. Hypotension is common. The most effective therapy is...

dopamine - usually starting dose = 5 mcg/kg/min

Dose of alkali for treatment of metbaolic acidosis can be calculated by...

dose of alkali (mEq) = base deficit x 0.3 x body wt (kg) - administer alkali IV at rate not exceeding 1 mEq/kg/min - usual alkali used in newborns is NaHCO3 and conc is 0.5 mEq/mL, so it is hyperosmolar (900 mOsm/L)

In most circumstances, arterial catheters are safer and more useful than venous catheters. However, in some cases, an umbilical venous catheter (UVC) is desirable, like...

exchange transfusion measurement of central venous pressure

The most common route of transmission of nosocomial infection is:

hands

Normal thymus appearance on US

homogenous echotexture - appearance = similar to that of liver

Excess accumulation of fluid in fetus

hydrops fetalis - mostly caused by severe erythroblastosis fetalis 2ndary to Rh iso-immunization

What is the factor most associated with mortality in pediatric burns?

inhalation injury - has been found to be the single most important factor associated with mortality for pediatric burns

vaccines that can interact with PPD

live attenuated

For post-natal management, perform this if infant is distended and interfering with ventilation...

paracentesis - inset needle/cannula in lower left abdomen lateral to rectus muscle to avoid puncturing possible enlarged spleen - remove enough fluid to decompress abdomen thus allowing diaphragm to move easily

Crackles or rales suggests...

parenchymal disease - pneumonia - pulmonary edema

noncardiac cause of obstructive shock in kid

pneumothorax pulmonary embolism

Pain with hip flexion and rotation suggests

possible interarticular process in the hip --> prompt a low threshold for imaging - patients with Legg-Calvé-Perthes disease should be made non-weight bearing while awaiting orthopedic evaluation

Should be considered in patients who experience effortless and painless vomiting for 2 months.

rumination syndrome

The bismuth component of bismuth subsalicylate may react with small amounts of sulfur in the gastrointestinal tract and result in black stools. Ingestion of bismuth subsalicylate in kids can result in

salicylism, especially with chronic ingestions, and children with varicella may be at risk for Reye syndrome - Over-the-counter cough and cold medications have not been shown to be effective in young children, and can pose a significant risk to children younger than 6 years

The most common müllerian anomaly is:

septate uterus / uterine septum - most common of all müllerian anomalies, accounting for just over half of all abnormalities = most common structural uterine anomaly = after the 2 müllerian ducts fuse in the midline, resorption must occur to unify the endometrial cavities; failure of this process results in some degree of uterine septum - can vary in length from just below the fundus to beyond the cervix, depending on the amount of caudal resorption - has a normal external uterine contour, which is what distinguishes it from a bicornuate or didelphic uterus - MRI can help delineate between a predominantly fibrous septum and a muscular or myometrial septum

PPN can be used for how long?

short term = <2 week - as bridge

What test is used to confirm a positive newborn screen for cystic fibrosis?

sweat chloride test

Another name of prograf...

tacrolimus = FK506 - Binds FK binding protein (FKBP12) which then then inhibits calcineurin, which is responsible for transcription of IL-2, IL-3, IL-4, IL-8, and various chemotactic factors - absorption occurs in the duodenum and jejunum - Absorption not influenced by presence of bile, which is advantageous in cholestatic patients or those with biliary diversion or ileus - Food reduces bioavailability, so TAC should be taken on an empty stomach - Metabolism occurs in the liver via the cytochrome P450-3A

Children with unknown time of coin ingestion, ingestion >24 hours prior, or those who are symptomatic require...

urgent endoscopy - ex. *flexible endoscopy*

Administration of topiramate in the setting of which other anticonvulsant may lead to hypothermia?

valproate

Most infants require only enough O2 to maintain SpO2 between...

87-92% - usually achieved with PaO2 of 40-60 mmHg, if pH is normal - pts with pulm HT may require much higher PaO2

Preparation of UVC is similar to those for UAC. The vein is the...

large, thin-walled vessel in the cord

Low birth weight infants are those born weighing less than

*2500 g*

A 9-year-old girl with cystic fibrosis presents to the emergency department with acute worsening of abdominal pain. She has had on and off cramping abdominal pain for the past week, however, two days ago it acutely worsened. The pain is localized to the right lower quadrant of her abdomen. Her last bowel movement was three days ago, and she reports it was nonbloody diarrhea. She denies fever or vomiting. She has a history of pancreatic insufficiency for which she takes pancreatic enzyme supplements. Her vital signs are T 37.6° C, HR 92 beats/min, RR 20 breaths/min, BP 106/74 mm Hg, and her oxygen saturation is 96% on room air. On physical examination, her abdomen appears mildly distended, there is a palpable mass in the right lower quadrant, and she is tender to palpation over the region. Bowel sounds are hyperactive, and there is no rigidity or guarding. An abdominal radiograph is obtained and shows stool accumulation on the right side.What is the best next step in the management of this patient?

*Administer diatrizoate sodium via nasogastric tube* - This girl with a history of cystic fibrosis and pancreatic insufficiency is suffering from abdominal pain that has acutely worsened in the past two days. Her abdominal exam and radiograph indicate a mass in the right lower quadrant, at the ileocecal junction. This presentation is most consistent with distal intestinal obstruction syndrome, which is unique to patients with cystic fibrosis and occurs when inspissated intestinal contents form an incomplete or complete blockage of the bowel, usually at the ileocecal junction. This most commonly occurs in cystic fibrosis patients with pancreatic insufficiency. - Symptoms usually include crampy abdominal pain that localizes to the right lower quadrant. The severity of symptoms depends on the degree and length of time of the blockage. - Peritoneal signs including rigidity and guarding are concerning for perforation and necessitate immediate intervention. Abdominal radiograph shows granular stool in the right lower quadrant but may have more concerning signs like air-fluid levels or distention of the small bowel. Management depends on the severity of the obstruction, but generally includes laxatives and hyperosmolar enemas. - In this girl whose last bowel movement was three days ago but who does not exhibit peritoneal signs, the best next step would be to administer diatrizoate sodium (gastrografin) via nasogastric tube. If this is unsuccessful, further management with nasogastric tube decompression and enemas may be required.

A full-term newborn who was born yesterday and is in the mother-infant unit has become less vigorous over the past several hours. Due to maternal preference, the baby has been formula fed with standard infant formula since birth. Initially, feeds were going well, but the baby now seems disinterested in feeding and has been sleeping for the past six hours. On examination, the infant is lethargic with poor suck, low tone, and weak primitive reflexes. A point-of-care glucose is 78 mg/dL, and a lab evaluation reveals a venous pH of 7.39 and a serum ammonia level of 493 mcg/dL. Which of the following is the most likely diagnosis?

*Argininosuccinate synthetase deficiency* - Organic acidemias and urea cycle defects are both disorders of toxic accumulations. They are the most likely metabolic disorders to present in the early newborn period, often within the first two to three days of consuming protein-containing feeds. Babies will develop lethargy, obtundation, and possibly seizure activity, as inappropriate protein metabolism causes the buildup of toxic metabolites. - Urea cycle defects, such as argininosuccinate synthetase deficiency, result from absence or deficiency of one of the enzymes of the urea cycle, which is responsible for the removal of nitrogenous wastes and ammonia from the bloodstream. This leads to the accumulation of ammonia as the toxic metabolite (rather than an organic acid by-product). There is no associated hypoglycemia or ketosis in a urea cycle defect, but ammonia levels are typically greater than 260 mcg/dL.

Newborn child with ambiguous genitalia. One month later has vomiting & ↓Na ↑K and acidosis.

*Congenital adrenal hyperplasia*

LTBI Treatment

12 weeks of isoniazid + rifapentine, once a week (>2 yo) OR 4 mo of riafampin once a day OR 9 mo of isoniazid once a day

In chronic granulomatous disease (CGD), which system is generally the first to become symptomatic?

*skin* - Abscesses of the scalp and perianal region are common. - Diarrhea, osteomyelitis, and pulmonary abscesses are among the systemic findings.

The standard dose for nebulized albuterol in pediatric pts is...

0.15 mg/kg (minimum 2.5 mg; maximum 5 mg) - Nebulized albuterol can be administered every 20 to 30 minutes for three doses Beyond that, frequency of therapy may be limited by side effects, such as tachycardia, hypertension, or tremors. - Patients who have shown little or no improvement after three doses and who are not experiencing significant adverse effects may be treated every 30 to 45 minutes or switched to continuous therapy. - Drug delivery is maximized by having a total solution volume of 3 to 4 mL and an oxygen flow rate of 6 to 8 L/min, tapping the sides of the reservoir to renebulize droplets, and having older children use a mouthpiece to avoid nasal deposition of drug

Baby Johns weighs 1.5 kg and is receiving: a morphine drip 0.7 ml/hour, dopamine at 0.3 ml/hour KVO 0.5 ml/hour, TPN 2.4 ml/hour, IL at 1 g/kg/day, feeds at 5 ml q 3 hours. What are his total fluids?

0.7 (morphine) *24 (hours) /1.5 (weight) = 11.2 ml/kg/day of morphine 0.3 (dopamine)*24/1.5 k.g= 4.8 ml/kg/day of dopamine 0.5 (KVO)*24/1.5 kg= 8 ml/kg/day of KVO 2.4 (TPN)*24/1.5 kg = 38.4 ml/kg/day of TPN 1 (1 g/kg/day of IL) *5 (using the shortcut) = 5 ml/kg/day IL 11.2+4.8+8+38.4+5= 67.4 = 67 ml/kg/day of IVF Feeds 5 ml * 8 feeds in a day = 40 ml/1.5 (weight) = 26.6 = 27 ml/kg/day of feeds 27 ml/kg/day of feeds + 67 ml/kg/day IVF = 94 ml/kg/day all fluids

Baby Johns weighs 1.5 kg and is receiving: a morphine drip 0.7 ml/hour, dopamine at 0.3 ml/hour KVO 0.5 ml/hour, TPN 2.4 ml/hour, IL at 0.3 ml/hour feeds at 5 ml q 3 hours. What are his total fluids?

0.7 (morphine) + 0.3 (dopa) + 0.5 (KVO) + 2.4 (TPN) + 0.3 (IL) = 4.2 ml/hour IVF * 24 hours/1.5 (weight) = 67.2 = 67 ml/kg/day in IVF Feeds 5 ml * 8 feeds in a day = 40 ml/1.5 (weight) = 26.6 = 27 ml/kg/day of feeds 27 ml/kg/day of feeds + 67 ml/kg/day IVF = 94 ml/kg/day all fluids

Normal ratio of FEV1/FVC =

0.80 - suggestive of airway obstruction = <0.86

Depending on their clinical condition, premature infants typically require 105 to 130 kcal/kg per day, with a weight gain goal of

15 to 20 g/day - Premature infants typically require 105 to 130 kcal/kg per day, with a weight gain goal of 15 to 20 g/kg per day, until the infant reaches approximately 2 kg. - The preferred milk for most premature infants is human milk, though human milk by itself may not provide an adequate amount of calories, protein, minerals, and vitamins without supplementation. - Fortification should be individualized to optimize growth trajectory over the first year after birth. For formula-fed infants, preterm infant formulas mixed to 22 to 24 kcal/oz provide the higher vitamin content these infants require

What is the earliest age that the American College of Obstetricians and Gynecologists recommends initiating cross-gender puberty?

16

A 2 wk old male infant is eating poorly, has repeated emesis, and has not regained his birth weight. On physical examination he appears dehydrated and is noted to have increased pigmentation of skin creases and genitalia. In addition to measuring serum electrolytes, assay of which of the following hormones would be most informative?

17-hydroxyprogesterone - Patients with salt-losing disease have typical laboratory findings associated with cortisol and aldosterone deficiency, including hyponatremia, hyperkalemia, metabolic acidosis, and often hypoglycemia, but these abnormalities can take 10-14 days or longer to develop after birth. - Blood levels of 17-hydroxyprogesterone are markedly elevated. - Diagnosis of 21-hydroxylase deficiency is most reliably established by measuring 17-hydroxyprogesterone before and 30 or 60 min after an intravenous bolus of 0.125-0.25 mg of cosyntropin (ACTH 1-24).

At what age does pretend play with others begin for typical social and emotional development?

18 months

Toilet training discussions start at WCC visits?

18-24 month well-visits

Commonly used for management of skin and soft tissue infections caused by susceptible strains of S. aureus and group A streptococcus.

1st generation cephalosporins - cefazolin, a parenteral formulation - cephalexin, an oral equivalent

For growing VLBW infants (birthwt </= 1500 g), if formula fed, change to ...

2 calorie/oz Neosure Advance - If breast fed, fortify supplemental breast milk to 22 calorie/oz with Neosure Advance powder

At what age should an infant be able to fixate and follow (bright object) and have visual directed reaching

2 months

The American Academy of Pediatrics states that it is safe to use DEET-containing products on children as young as

2 months of age - Higher concentrations of DEET provide longer protection. - Concentrations of DEET greater than 30% provide little added benefit for protection from ticks and mosquitos

rear facing car safety seat until...

2 yo

Primary routine vaccination against Haemophilus influenzae type b is given at

2, 4, and 6 months of age or at 2 and 4 months of age, depending on the manufacturer, with a booster at 12 to 15 months of age

Child has chronic wet cough with no other abnormal findings, which suggests persistent bacterial bronchitis; the most appropriate management is

2- to 4-week course of amoxicillin/clavulanate or equivalent narrow-spectrum antibiotic appropriate for respiratory flora - Chronic cough is a cough that has been present for at least 4 continuous weeks. - Specific cough pointers (signs and/or symptoms) are findings from the history or physical examination that suggest a specific etiology for the cough and help direct management. - Nonspecific cough has no findings on history or physical examination that suggest an etiology. - Appropriate evaluation includes chest radiography and pulmonary function testing (in children >6 years old); no additional testing is needed unless an abnormality is suggested by the results of these tests.

In girls, the breast bud (thelarche) is usually the 1st sign of puberty (10-11 yr), followed by the appearance of pubic hair (pubarche) 6-12 mo later. The interval to the onset of menstrual activity (menarche) is usually ...

2-2.5 yr but may be as long as 6 yr - In the United States, at least 1 sign of puberty is present in approximately 95% of girls by 12 yr of age and in 99% of girls by 13 yr of age. - Peak height velocity occurs early (at breast stage II-III, typically between 11 and 12 yr of age) in girls and always precedes menarche. - The mean age at menarche is about 12.75 yr. There are, however, wide variations in the sequence of changes involving growth spurt, breast bud, pubic hair, and maturation of the internal and external genitalia.

Most episodes of acute coughing other than foreign body aspiration resolve on their own within

2-3 weeks without the need for diagnostic or therapeutic interventions

Epiglottitis is most common in what age range?

2-7 years old - equal incidence in males and females - medical emergency - *controlled nasotracheal intubation should be performed by experienced personnel*

You have a 20 kg child that comes in with 30% TBSA burns. What is your initial fluid rate per the Parkland formula?

210 ml/hr The Parkland formula is Total fluids in first 24 hours =4(Wt in Kg)(%TBSA Burn) with the first half given in the first 8 hours and the other half given in the second 8 hours. Hence, the total fluid to be given in the first 24 hours= 4(20kg)(30)= 2400 ml. 1200 ml should be given in the first 8 hours. Hence 1200/8=150 ml/hr. However, you also have to add the basic maintenance rate which is 60 ml/hr leading to an initial fluid rate of 150+60 =210 ml/hr. This can then be titrated based on urine output and hemodynamic parameters.

Fortifying feedings not only provides more calories but also improved intake of calcium, phosphorus, and protein. Fortify feedings (breast milk and formula) as follows: - When infants is tolerating >/= 100 cc/kg/d, feedings may be fortified to .... - When infant has been tolerating >/= 150 cc/kg/d for at least 2d, feedings may be fortified to ....

22 cal/ox 24 cal/oz

1 unit will drop your blood sugar 50 points (mg/dl) and the high blood sugar correction factor is 50. Pre-meal blood sugar target is 120 mg/dl. Your actual blood sugar before lunch is 220 mg/dl. Now, calculate the difference between your actual blood sugar and target blood sugar:

220 minus 120 mg/dl = 100 mg/dl To get the high blood sugar correction insulin dose, plug the numbers into this formula: Correction dose = Difference between actual and target blood glucose (100mg/dl) ÷ correction factor (50) = 2 units of rapid acting insulin So, you will need an additional 2 units of rapid acting insulin to "correct" the blood sugar down to a target of 120 mg/dl

What percent of children with autism spectrum disorder have macrocephaly?

25%

GI motor activity present before 24 wks but organized peristalsis not established until ...

29-30 wks - facilitated by antenatal corticosteroid tx

A 16-year-old girl presents to clinic for a well-child visit. The girl has not yet received vaccination against human papillomavirus, and her mother would like to start the series today. How many doses of the 9-valent human papillomavirus vaccine will this girl need?

3 Human papillomavirus - 9-valent human papillomavirus vaccine protects against human papillomavirus types 6, 11, 16, 18, 31, 33, 45, 52, and 58, which cause a significant proportion of warts and these cancers. - recommended that vaccination against human papillomavirus be initiated between ages 11 and 12. - In children ages 14 and younger, two doses separated by 6-12 months are recommended. - In children 15 years and older, three doses are recommended. These doses should be given at zero, one to two, and six months.

Coordinated sucking and swallowing develops at how many wks?

32-34 wks - by term, fetus swallows ~ 150 cc/kg/day of amniotic fluid, which has 275 mOsm/L, contains carbs, protein, fat, electrolytes, immunoglobulins, growth factors --> plays important role in dev't of GI function

Age: throws ball overhead

36 mos

A 2-day-old neonate is nearly ready to be discharged. He was born at 39 weeks' gestation without any complications during pregnancy or delivery. He is feeding well and has voided and passed stools. His vital signs, including oxygen saturation, have been normal since birth. Despite several attempts, he failed the otoacoustic emission test. Before discharge, repeat otoacoustic emission testing is arranged. Of the following, based on current care guidelines, the MOST appropriate timeframe for the completion of this testing is within

3 month - Infants with hearing loss should be identified by 3 months of age. - Cytomegalovirus is the most common cause of acquired sensorineural hearing loss. - Hearing loss is associated with language, social, and motor developmental delays; intervention should begin by 6 months of age.

When screening for developmental disorders, if child is younger than 24 months at the time of screening, adjust the age for prematurity if the child was born...

3 or more weeks premature - adjusted age = child's age in weeks - number of weeks premature (term = 40 wks) Ex. 6 months old born at 32 wks - baby was 8 weeks premature - 24 - 8 wks = 16 weeks = 4 month ASQ interval used

Total serum bilirubin peaks at what age in infants?

3-5 days (later in Asian infants) - mean peak total serum bilirubin is 6 mg/dL (higher in Asian infants)

Severe traumatic brain injury is characterized by a Glasgow Coma Score (GCS) of:

3-8 - The hallmark of severe TBI is coma (GCS score 3-8). - In some cases, such as with an epidural hematoma, a child may be alert at presentation but the condition may deteriorate after a period of hours. - Clinicians also should not be lulled into underappreciating the potential for deterioration of a child with moderate TBI (GCS score 9-12) with a significant contusion, because progressive swelling can potentially lead to devastating complications. - In the comatose child with severe TBI, the second key clinical manifestation is the development of intracranial hypertension. I - Significantly raised ICP (>20 mm Hg) can occur early after severe TBI, but peak ICP generally is seen at 48-72 hr. - Need for ICP-directed therapy may persist for longer than a week.

Name 4 pictures by what age?

36 mos

Handedness is usually determined by age...

36-48 mos - usually established by 3 yo

On the 1st day of life, preterm infants should receive restricted fluids, appx....

60-80 mL/kg/d - for ELBW infants, fluid intake should be higher (100-125 mL/kg/d) - follow intake and output closely, at least q12h for first several days

The AAP recommends developmental screenings at what age?

9, 18, 24, and 30 months of age - using formal tool such as the Ages and Stages Questionnaire

A male newborn infant is noted to have increased pigmentation of his skin and genitalia, perineal hypospadias with bifid scrotum, and a 2-cm phallus with chordee. Gonads are palpable in the inguinal canal, and no uterus is visualized on ultrasound examination. The most likely diagnosis is:

3β-HSD deficiency - Because cortisol and aldosterone are not synthesized in patients with the classic form of the disease, infants are prone to salt-wasting crises. - Because androstenedione and testosterone are not synthesized, boys are incompletely virilized. - Varying degrees of hypospadias may occur, with or without bifid scrotum or cryptorchidism.

At what age should babies' ocular alignment be stable with ability to look from near to far and back again.

4 months

Benign GER peaks when?

4 months of age - when two-thirds of infants are reported to spit up daily - resolution in nearly 95% of infants by 1 year

Age: grasps rattle

4 mos

Normal BMI for kid

5-84%

Holliday-Segar method Electrolyte Requirements Na - 3 MEq per 100 cc water K - 2 MEq per 100 cc water Example, 25 kg kid, 1600 cc/d ...

48 Meq Na, 32 Meq K 48/1600 = X/1000; X = 30 (Remember that quarter NS has 38.5 MEq/L Na) 32/1600 = Y/ 1000; Y= 20 D5 quarter NS with 20 MEq/L KCl (as Cl is your anion to fill with)

Typically, patients with uncomplicated community-acquired bacterial pneumonia show response to therapy, with improvement in clinical symptoms (fever, cough, tachypnea, chest pain), within

48-96 hr of initiation of antibiotics

A 1-year-old girl is being evaluated for unusual facies, growth deficiency, significant developmental delays, hypotonia, hearing loss, and a previously diagnosed generalized tonic-clonic seizure disorder. Her facies are remarkable for microcephaly, "Greek warrior" helmet nose, high anterior hairline with prominent glabella, hypertelorism, epicanthus, high-arched eyebrows, downturned mouth, small jaw, and small ears with bilateral ear pits. Prenatal history is remarkable for intrauterine growth restriction. Evaluation for other anomalies includes echocardiography, which shows an atrial septal defect, and brain magnetic resonance imaging, which shows thinning of the corpus callosum. A diagnosis is made using a high-resolution karyotype. Of the following, the MOST likely diagnosis for this girl is

4p-deletion/ Wolf-Hirschhorn syndrome - unusual facies - prenatal and postnatal growth deficiency - significant developmental delays - hypotonia - hearing loss - antibody deficiencies (69%) - cardiac defects (50%) - urinary tract anomalies (30%) - central nervous system malformations (80%) - epilepsy or electroencephalographic abnormalities Typical craniofacial dysmorphology includes microcephaly, "Greek warrior" helmet nose, high anterior hairline with prominent glabella, hypertelorism, epicanthus, high-arched eyebrows, downturned mouth, small jaw, and small ears with bilateral ear pits.The most common congenital heart defect is an atrial septal defect followed by pulmonic stenosis, ventricular septal defect, tetralogy of Fallot, and patent ductus arteriosus. The most common central nervous system anomaly is thinning of the corpus callosum followed by ventricular enlargement, cortical atrophy, and cerebellar hypoplasia. A few affected children have hematopoietic dysfunction or hepatic adenomas

Per AAP Bright Futures, for middle childhood (ages 5 - 10), when are hearing and vision screenings done?

5, 6, 8, 10

A 10 yr old girl (body surface area of 1.0 m2) with chronic renal insufficiency is seen in your clinic and undergoes a 24-hr urine collection for measurement of creatinine clearance. The results are as follows: urine creatinine 144 mg/dL; serum creatinine 1.7 mg/dL; urine volume 700 mL. Based on these measurements, this patient's standard creatinine clearance (mL/min/1.73 m2) is:

71 mL/min/1.73 m2 - The precise measurement of the GFR is accomplished by quantitating the clearance of a substance that is freely filtered across the capillary wall and is neither reabsorbed nor secreted by the tubules. Clearance is represented by the following formula: Cs(mL/min) = Us(mg/mL) × V(mL/min)/Ps(mg/mL) - Cs equals the clearance of substance s - Us reflects the urinary concentration of s - V represents the urinary flow rate - Ps equals the plasma concentration of s To correct the clearance for body surface area, the formula is: Corrected clearance (mL/min/1.73 m2) = Cs(mL/min) × 1.73/ Surface area (m2) The calculation becomes: (144mg/dL × 700 mls)/(1.7 mg/dL) = 59294 mls/day = (59294 mls/day) / (1440 min/day) = 41.2 ml/min. 41.2 ml/min × 1.73/1 (BSA correction) = 71 ml/min/1.73m2

ECMO is temporary support of heart and lung function by partial cardio-pulmonary bypass - up to ....

75% of cardiac output - used for pts who have: reversible cardiopulmonary failure from pulmonary, cardiac, or other disease

In newborn, saturation goal at 4 minutes...

75-80%

An infant has the following findings at 5 min of life: pulse, 130 beats/min; cyanotic hands and feet; good muscle tone; and a strong cry and grimace. This infant's Apgar score is:

8 - This infant loses one point for color and one point for grimacing in response to the catheter in the nostril but otherwise appears vigorous.

Should have car seat for kids until...

8 yo or 4 ft 9inches

Goal prograf/tacrolimus/FK506 trough level immediately after transplant

8-10 - after time out from transplant, goals decrease

18 month old should have how many words?

8-10 words - if not, think hearing, autism

Neonates are near-sighted, having a fixed focal length of

8-12 in - approximately the distance from the breast to the mother's face - as well as an inborn visual preference for faces

A female newborn has a single perineal orifice. The chance that this baby has associated urologic problems is:

90% - More than 90% of the time the diagnosis in girls can be established on perineal inspection. - The presence of a single perineal orifice is a cloaca. - Patients with a persistent cloaca have a 90% risk of urologic defects.

Photoscreening is commonly covered under 2 CPT codes...

99174 (remote view of images) 99177 (on-site review of images)

Extremely Low Birth Weight (ELBW): Birth weight ...

<1,000 g

Very Low Birth Weight (VLBW): Birth weight ...

<1,500 g

For infants, always use an ET tube with an internal diameter (I.D.) that is....

<1/10 of the infant's gestational age (i.e., if gestational age is 35 wks, use a 3.0 I.D. tube, not a 3.5 tube)

low voltage

<1000 volts - standard household outlets = 110 V

Suggested frequency of measurements of electrolytes, including calcium for infants receiving only IV fluids.... < 750 g --> 750 - 1500 g --> > 1500 g -->

<750 g = q8-12h x 3-4d, then daily 750-1500 g = q12h x 3-4d, then daily >1500g = daily

Change in FEV1 by how much after bronchodilator is considered reversible?

>10% - Reversibility is determined either by an increase in FEV1 of at least 12% from baseline or by an increase of at least 10% of predicted FEV1 after inhalation of a shortacting bronchodilator. - change in FEF 25-75% >25% = significant response too

CDC defines an elevated blood lead level (BLL) as ...

>5 mcg/dL

A 10 yr old girl has grown 4 cm and gained 7 kg in the past year. She is diffusely obese with violaceous striae on her trunk and extremities. Her 24-hour urinary free cortisol is increased. The next step in her evaluation is:

A 2-step dexamethasone suppression test - After the diagnosis of Cushing syndrome has been established, it is necessary to determine whether it is caused by a pituitary adenoma, an ectopic ACTH-secreting tumor, or a cortisol-secreting adrenal tumor. - ACTH concentrations are usually suppressed in patients with cortisol-secreting tumors, are very high in patients with ectopic ACTH-secreting tumors, but may be normal in patients with ACTH-secreting pituitary adenomas. - The 2-step dexamethasone suppression test consists of administration of dexamethasone, 30 and 120 μg/kg/24 hr in 4 divided doses, on consecutive days. In children with pituitary Cushing syndrome, the larger dose, but not the smaller dose, suppresses serum levels of cortisol. Typically, patients with ACTH- independent Cushing syndrome do not show suppressed cortisol levels with dexamethasone.

Compression injury of distal radial and/or ulnar metaphysis

Torus or buckle fracture - often due to FOOSH - buckling of periosteum - stable - children may be safely treated with removable splint

What form of testing for chlamydia and gonorrhea is preferred in victims of sexual assault?

A nucleic acid amplification test, or NAAT, from vaginal or urine specimens, is preferred over a cervical culture test.

Mnemonic for managing coma

ABC DON'T Airway (with cervical spine immobilization) Breathing Circulation D-stick - D25W, 2 to 4 mL/kg IV bolus if hypoglycemia present Oxygen Naloxone - 0.1 mg/kg IV, IM, SQ, or ETT (max dose, 2 mg) - repeat as necessary, given short half-life (in case of opiate intoxication) Thiamine - 100 mg IV (before starting glucose in adolescents, in case of alcoholism or eating disorder)

A 10 yr old boy is brought to the emergency department after being kicked in the abdomen by a horse. Pulse is 80/min; respiratory rate is 18 breaths/min; and blood pressure is 110/70 mm Hg. His abdomen is diffusely tender. The most appropriate diagnostic study for this patient would be:

Abdominal CT with IV contrast - rapidly identifies structural and functional abnormalities and is the preferred study in a stable child - excellent sensitivity and specificity for splenic, hepatic, and renal injuries but is not as sensitive for diaphragmatic, pancreatic, or intestinal injuries - Although focused assessment with sonography in trauma (FAST) examination helps detect hemoperitoneum, the variably low sensitivity of this test in children suggests that it should not be used to exclude intra-abdominal injury in patients with a high pretest probability for injury

A 12-year-old girl is being evaluated for tooth pain that started a few days ago, but worsened yesterday. She has left upper molar pain with localized gingival swelling; the pain is making it difficult to eat. Her vital signs include a temperature of 38.3°C, a heart rate of 105 beats/min, and a respiratory rate of 17 breaths/min. Her mouth has an area of redness, swelling, and fluctuance along the maxillary left molar, which is tender to the touch. Of the following, the MOST likely cause of this infection is

Actinomyces israelii - Anaerobic bacteria are the cause of most dental infections. - Good routine dental hygiene should be reviewed at health supervision visits.

Rare neurologic disorder presenting with acute paralysis of 1 or more limbs, preserved sensation, and normal mental status, typically within a week of viral symptoms.

Acute flaccid myelitis - Treatment of acute flaccid myelitis is supportive; recovery is variable and often incomplete. - Suspected cases of acute flaccid myelitis should be reported to the Centers for Disease Control and Prevention with samples sent for viral testing.

A 15 yr old boy is admitted to your care after the intentional ingestion of 2 g of his own amitriptyline in a suicide gesture. He received activated charcoal for gastrointestinal decontamination. The patient is placed in the ICU for monitoring and remains stable. He is receiving only 0.45% normal saline. You observe a change in his cardiac monitor display with a widening of his QRS complex to 0.12 second and occasional ectopic beats. The most appropriate next step in management is to:

Add sodium bicarbonate to his IV fluids to try to raise his serum pH above 7.4 Sodium bicarbonate - antidote of choice for TCA toxicity and works via overcoming the sodium channel blockade by providing a sodium load and via inducing an alkalosis to decrease drug binding to sodium channels. Indications for sodium bicarbonate - QRS duration >100 msec - ventricular dysrhythmias - hypotension. An initial bolus of 1-2 mEq/kg of sodium bicarbonate is given followed by initiation of a continuous infusion. - Additional boluses may be given if the QRS duration continues to widen, with the goals of therapy being a serum pH of 7.45-7.55, improved hemodynamic stability, and narrowing of the QRS complex.

A 7 year old boy has a history of moderate persistent asthma. His mother is concerned that he has been sleeping poorly. He snores loudly w/ noted pauses and snorting that often wakes him. He has behavior issues at school. His BMI is greater than 95itle and his BP is 128/80mHg. His tonsil are 4+ in size. Vital signs and physical exam are normal. PSG results show AHI of 27 events/hr w/ SpO2 nadir of 77% and normal wake SpO2. Of the following, the most accurate statement regarding this boy's OSA is

Adenotonsillectomy requires inpatient overnight monitoring postop - He has severe obstructive sleep apnea that is complicated by his OSA and asthma. He is at high risk for post-op compromise after his T&A based on his polysomnogram findings and of an apnea-hyponea (AHI) index greater than or equal to 24 events/hour and an SpO2 less than 80%. - This child in this vignette demonstrates several features of OSA including snoring, observed apnea, and snorting. he is obese with tonsillar hypertrophy and hypertension. - AAP guidelines consider children younger than 3 years and children with severe OSA, obesity, craniofacial anomlies, neuromuscular disorders, cardiac complications, or failure to thrive as being at higher risk for postsurgical complications including worsening OSA, pulm edema, or death. Therefore guidelines recommends that high risk patients who undergo adenotonsillectomy receive inpatient post-op monitoring.

A 16 yr old, 165-lb patient reports consuming 20-40 325-mg capsules containing acetaminophen 1 hr ago. The most appropriate approach to treatment is to:

Administer activated charcoal immediately and measure the plasma level of acetaminophen 4 hr after ingestion - Initial treatment should focus on the ABCs and consideration of decontamination with activated charcoal in patients who present within 1-2 hr of ingestion. - The antidote for acetaminophen poisoning is NAC, which works primarily via replenishing hepatic glutathione stores. - NAC therapy is most effective when initiated within 8 hr of ingestion. However, there is no demonstrated benefit to giving NAC before the 4 hr postingestion mark. Thus, patients who present early after ingestion should have a 4-hr level drawn and decision to initiate NAC should be based on this level.

A 10-month-old girl presents with six days of fever, five days of bilateral red eyes, four days of swelling of the hands and feet, two days of red, cracked lips, and today a diffuse rash. Which of the following is a risk factor for coronary artery aneurysm associated with her Kawasaki disease?

Age under one year - made with a combination of a least five days of fever and fulfillment of at least four of five clinical criteria. - also has four clinical criteria, including bilateral nonexudative conjunctivitis, swelling of the hands and feet, mucous membrane changes, and a rash. - does not meet the fifth clinical criteria, unilateral cervical lymph node swelling. - fulfillment of four criteria is sufficient for a diagnosis of Kawasaki disease. - age under one year is of particular concern, as infants less than one year of age are at increased risk of coronary artery aneurysms. Other risk factors for coronary artery aneurysm include delayed diagnosis, failed therapy with intravenous immune globulin, age over nine years, male sex, certain supportive laboratory findings, and genetic polymorphisms.

For children with mild asthma exacerbation, we suggest the following...

Albuterol inhalation therapy - administered via small volume nebulizer (SVN) at a dose of 0.15 mg/kg (minimum 2.5 mg and maximum 5 mg per dose) or metered-dose inhaler with spacer (MDI-S) at a dose of one-quarter to one-third puff/kg (minimum two puffs and maximum eight puffs per dose). - If repeated doses are needed, they should be given every 20 to 30 minutes for three doses

1 pill can kill list for children

Alpha-adrenergic blockers (clonidine) Antimalarials (chloroquine, quinine) Beta blockers Buprenorphine (Suboxone®, Subutex®) Calcium channel blockers Camphor Carbamates/organophosphates Caustics Imidazolines (oxymetazoline, tetrahydrolazine) Opioids Sulfonylureas (e.g., glipizide, glyburide, glimepiride) Toxic alcohols (ethylene glycol, isopropanol, methanol) Tricyclic antidepressants http://missouripoisoncenter.org/wp-content/uploads/2015/02/2011-One-Pill-Can-Kill.pdf

Which of the following is the recommended treatment for neonatal listeriosis?

Ampicillin with or without an aminoglycoside - The recommended therapy is ampicillin (100-200 mg/kg/day divided every 6 hr intravenously [IV]; 200-400 mg/kg/day divided every 6 hr IV if meningitis is present) alone or in combination with an aminoglycoside (5.0-7.5 mg/kg/day divided every 8 hr IV). - The aminoglycoside enhances the bactericidal activity and is generally recommended in cases of endocarditis and meningitis.

The radioallergosorbent test (RAST) and related as-IgE assay determine:

Antigen-specific serum IgE concentrations - can be documented in vivo by skin testing or in vitro by the measurement of allergen-specific IgE (as-IgE) levels in the serum - first test for documenting the presence of as-IgE was called the radioallergosorbent test (RAST) because it used a radiolabeled anti-IgE antibody - RAST has been replaced by an improved generation of as-IgE assays that use enzyme-conjugated rather than radiolabeled anti-IgE - these assays use solid-phase supports to which allergens of an individual allergen extract are bound - next, the allergen-coated support to which the patient's as-IgE is bound is incubated with enzyme conjugated antihuman-IgE that then binds to the patient's as-IgE - incubation of this complex with a fluorescent substrate of the conjugated enzyme results in the generation of fluorescence that is proportional to the amount of as-IgE in the serum sample

A 6 yr old girl with a history of asthma presents to your clinic with status asthmaticus. You give her 3 separate albuterol treatments and IM dexamethasone. She has improved aeration, expiratory wheezes, and a respiratory rate of 40 breaths/min. You advise admission to the hospital, which is 30 miles away. The mother would like to drive the girl. The most appropriate response would be:

Arrange for an ambulance to transport the patient after arranging the admission - This patient has acute respiratory distress and has a high likelihood of requiring medical intervention during transportation (e.g., oxygen therapy, additional respiratory treatments). - Ultimately, the legal responsibility for a patient lies with the referring physician, until responsibility of care is officially transferred to another medical provider.

A 15-year-old girl presents with fever, chills, abdominal pain, and emesis. The pain is constant and is located in the right upper abdomen and back. She has had small, frequent urine output despite her poor oral intake. She has mild suprapubic tenderness and percussive tenderness over the costovertebral angle. What is the most likely etiology of her pain and fever?

Ascension of Enterobacteriaceae up the ureter Pyelonephritis - urinary frequency, fever, chills, abdominal pain, emesis, suprapubic pain, and costovertebral angle tenderness. - most commonly caused by ascension of Enterobacteriaceae up the ureter - incidence of urinary tract infection in women is four-fold higher than in men, which is presumed to be secondary to the shorter length of the female urethra. - Recognition and proper treatment of pyelonephritis is important in the prevention of renal scarring and its consequences, such as end-stage renal disease and hypertension.

Example: 160 lbs In this example: Correction Factor =

Assume your total daily insulin dose(TDI) = 160 lbs ÷ 4 = 40 units Correction Factor = 1800 ÷ TDI(40 units) = 1 unit insulin will drop reduce the blood sugar level by 45 mg/dl

A 3 mo old infant has had progressively worsening biphasic stridor. Her parents report two brief episodes of croup. A 1-cm-diameter hemangioma is present on her chin. Airway radiographs are most likely to show:

Asymmetric subglottic narrowing Congenital subglottic hemangiomas - symptomatic within the 1st 2 mo of life, almost all occurring before 6 mo of age - stridor is biphasic but usually more prominent during inspiration - infant may be hoarse, have a barking cough, and present with croup - fifty percent are associated with facial lesions - radiographs classically delineate an asymmetric subglottic narrowing - diagnosis is made by direct laryngoscopy

You are caring for a 3 year old with vomitting and diarrhea. The child's pulses are palpable but faint, and the child is now lethargic. The HR is variable (range, 44/min to 62/min). You begin bag-mask ventilation with 100% oxygen. When the HR does not improve, you begin chest comnpressions. The rhythm shown here is seen on the cardiac monitor (bradycardia). Which would be the most appropriate therapy to consider next?

Atropine 0.02 mg/kg IV

An 18-month-old girl presents to establish care with a new pediatrician. She was born on time, has had no major illnesses, and is immunized. Her mother is concerned that she has not yet begun speaking and seems disinterested in other children. She is able to walk and run and scribbles with crayons. In the exam room, the toddler is sitting on the floor spinning a wheel on a truck and becomes inconsolable when her mother removes the truck from her hand. What is the most likely diagnosis?

Autism spectrum disorder - constellation of speech and social delays in the setting of normal motor development raise concern for autism spectrum disorder - abnormal social interactions may include poor eye contact, inability to share joint attention, poor understanding of nonverbal communication, lack of empathy, inability to participate in discussion, limited adaptation to social environments, and inability to develop or maintain friendships

Event occurring in infant younger than 1 year when observer reports sudden, brief, and now resolved episode of >/= 1 of the following: - cyanosis or pallor - absent, decreased, or irregular breathing - marked change in tone - altered level of responsiveness

BRUE: Brief Resolved Unexplained Event

A term baby girl has 2 episodes of bile-stained emesis at 24 hr after birth. There is a history of excessive amniotic fluid volume. The most appropriate diagnostic test is:

Barium upper gastrointestinal x-ray series with small bowel follow-through - Bile-stained emesis suggests intestinal obstruction beyond the duodenum but also may be idiopathic. - Abdominal radiographs (kidney-ureter-bladder [KUB] and cross-table lateral views) should be performed in neonates with persistent emesis and in all infants with bile-stained emesis to detect air-fluid levels, distended bowel loops, characteristic patterns of obstruction (double bubble: duodenal atresia), and pneumoperitoneum (intestinal perforation). - A contrast swallow radiograph with small bowel follow-through is indicated in the presence of bilious emesis.

The 500 rule

Based on assumption that average person consumes (via meals and snacks) and produces (via the liver) a total of 500 grams of carbohydrate daily --> Divide 500 by average number of units of insulin you take daily (basal insulin plus mealtime insulin) = I:C ratio Example - If you take a total of 25 units of insulin in a typical day, each unit of insulin should cover approximately 20 grams of carbohydrate (500 ÷ 25 = 20). - If you take 60 units daily, your I:C ratio would be 1 unit per 8 grams of carb (500 ÷ 60 ≅ 8).

A 4 yr old boy is brought to your office because of a circular reddish rash on the right side of his chest. The child has been afebrile and has had no other systemic symptoms. The rash is not pruritic. The child's parents state that they have recently returned from a vacation in Massachusetts on Cape Cod and that a small tick had been removed from the same area where the rash is now. The only abnormality on the examination is the circular, flat, erythematous rash, which is about 6 cm in diameter and is not tender. The most appropriate next step in treating this patient is to:

Begin treatment with amoxicillin - The 1st clinical manifestation of Lyme disease in most patients is erythema migrans. - Although it usually occurs 7-14 days after the bite, the onset of the rash has been reported from 3 to 30 days later. - Without treatment, the rash gradually expands (hence the name migrans) to an average diameter of 15 cm and typically remains present for 1-2 wk. - Erythema migrans may be associated with systemic features, including fever, myalgia, headache, or malaise. - In the appropriate epidemiologic setting, typical erythema migrans is virtually pathognomonic. - At this phase of illness, patients may be appropriately treated with an oral antibiotic without further laboratory evaluation. Young children are generally treated with amoxicillin.

A 9 year old presents to his primary care physician for a well child check. Mom tells you he snores at night and is extremely restless. Patient undergoes a sleep study which reveals mild obstructive sleep apnea. The most likely clinical presentation of this child is:

Behavior issues, mood swings, attention-deficit/hyperactivity disorder like symptoms - Children, especially children with mild OSA, unlike adults with OSA, are most likely to present with behavioral issues, mood swings, and attention-deficit/hyperactivity disorder (ADHD)-like symptoms. - Adults, not children, usually present with excessive daytime sleepiness. - Children with OSA have short sleep latency. The main risk factors for OSA in children are adenotonsillar hypertrophy and obesity. - Children with OSA may present with bed-wetting, but it is not the most common presentation.

2 brands of tracheostomy tubes

Bivona ​ - Neonatal​: Shorter inner cannula ​ - Pediatric - Bivona has a specific cuff trach called TTS: when balloon is deflated, it is contracted tightly around shaft to decrease resistance around the tube.​ ​ Shiley ​ - Pediatric ​ - Adult

A 6-year-old previously healthy boy is brought to your office by his mother for concerns of adult body odor that she first noticed a few months ago. He takes no medication and has had no exogenous exposure to androgen. A review of systems shows unremarkable findings. There is no family history of precocious puberty. On physical examination, his temperature is 37°C, heart rate is 89 beats/min, blood pressure is 98/56 mm Hg, weight is 25 kg (90th percentile), and height is 125 cm (97th percentile). He appears older than his chronologic age, and has comedonal acne on his nose. Examination of his genitalia reveals a normal phallus, pubic hair at sexual maturity rating 2, and testes measuring approximately 6 mL bilaterally. Thinning and reddening of the scrotum are noted. He has a small amount of axillary hair bilaterally. The remainder of the physical examination findings are unremarkable. Of the following, the test MOST likely to reveal this boy's diagnosis is

Brain MRI - Testicular size is an important discriminator between central and peripheral precocious puberty in boys. - Testes are pubertal in volume (≥4 mL) in central precocious puberty because they are stimulated by luteinizing hormone (LH). - A pubertal LH level of 0.3 IU/L or more is consistent with central precocious puberty. - Central nervous system pathology is much more common in boys with central precocious puberty than in girls.

7 days old, bili @ 12, direct is 0.5. dry mucous membranes, not gaining weight.

Breast feeding jaundice - decreased feeding = dehydration = retain meconium and reabsorb deconjugated bili

Inhaled steroids --> name some

Budesonide (Pulmicort) Fluticasone propionate (Flovent) Mometasone (Asmanex) Beclomethasone (Qvar) Ciclesonide (Alvesco) Flunisolide (Aerospan) Fluticasone furoate (Arnuity Ellipta)

A 3 yr old girl presents to your office with acute onset of lethargy and pallor. The child's mother reports that the child had bloody diarrhea for 5 days that cleared 1 day before presenting to your office. She also notes acute onset of cola-colored urine. On examination, the patient is pale and lethargic. Blood pressure is 120/80 mm Hg. The most appropriate next step in diagnosis would be:

CBC Hemolytic-uremic syndrome (HUS) - triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency - most common form of HUS is caused by toxin-producing Escherichia coli that causes prodromal acute enteritis and is commonly termed diarrhea-associated HUS - diagnosis is made by the combination of microangiopathic hemolytic anemia with schistocytes, thrombocytopenia, and some degree of kidney involvement - anemia, mild at presentation, rapidly progresses. - Coombs test is negative, with the exception of pneumococci-induced HUS, in which the Coombs test is usually positive - leukocytosis is present and significant

A previously healthy 7-year-old boy is brought to the emergency department (ED) for evaluation. He has had diarrhea for the last 8 days and bloody diarrhea for 2 days. He has become increasingly listless over the past several hours. He has no known previous medical problems. There is no history of similar illness in other family members. A week ago the boy visited an apple orchard on a school trip. His family does not know if any other children on the trip had similar symptoms. Physical examination reveals an ill-appearing child, with marked pallor and periorbital edema. His temperature is 38.4°C, heart rate is 120 beats/min, respiratory rate is 28 breaths /min, and blood pressure is 90/50 mm Hg. He has normal growth parameters. Of the following, the test MOST likely to confirm the boy's diagnosis is

CBC + smear Hemolytic uremic syndrome (HUS) - one of the most common causes of acute renal failure in children - should be considered in all children presenting with bloody diarrhea - complete blood cell count (CBC) with review of the peripheral blood smear (PBS) is most likely to reveal the diagnosis for the boy in the vignette - characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury - enteric infection with organisms such as Salmonella, Campylobacter, Yersinia, amebiasis, or Clostridium difficile also present with abdominal pain and bloody diarrhea - evaluation in such patients may reveal elevated serum creatinine and blood urea nitrogen levels, indicating acute renal failure (prerenal) secondary to dehydration

An 8-year-old girl is being evaluated for repeated episodes of pneumonia. During these events, the girl is usually afebrile, but develops cough and sometimes spits out pink, frothy mucus. Chest radiographs have shown diffuse multifocal infiltrates. These episodes are usually treated with antibiotics and corticosteroids, and improve over several days. The girl has no history of asthma or wheezing, chronic or recurrent upper respiratory congestion with rhinorrhea, or kidney disease. Physical examination findings are normal today, and the point-of-care urinalysis is normal. Of the following, the laboratory test MOST likely to determine this girl's diagnosis is

CBC+diff - The girl in the vignette has pulmonary hemosiderosis characterized by repeated episodes of subclinical pulmonary hemorrhage that may present with cough and pink frothy sputum. Chest radiography will demonstrate multifocal infiltrates. The characteristic finding, other than the radiographic infiltrates, is anemia, which may be variable and exacerbated by episodes of hemorrhage. - Pulmonary hemosiderosis is characterized by repeated episodes of subclinical pulmonary hemorrhage that may present with cough and pink frothy sputum; chest radiography will demonstrate multifocal infiltrates.

Labs to establish severity of acute pancreatitis and guide management...

CMP serum Ca CBC serum triglycerides lactate

Imaging for kid with.... airspace disease

CT scan

Imaging for kid with.... investigating extent and pattern of bronchiectasis

CT scan

Imaging for kid with.... lung abscess

CT scan

Imaging for kid with.... anterior or middle mediastinal mass

CT scan PET for lymphoma

Also called infantile cortical hyperostosis. Is a rare musculoskeletal disorder that most often occurs in infants. Excessive new bone formation (hyperostosis), which mainly affects the mandibles, scapulae, clavicles, and long bone diaphyses. Infants often develop swelling of the joints and soft tissues, with pain and erythema in the affected areas. Signs and symptoms are generally apparent within the first few months after birth and often resolve by early childhood.

Caffey disease

Step-up approach for controller medications for asthma

Can start at flovent or Qvar (40/44/low-dose --> 100/110/medium-dose) --> advair: fluticasone/salmeterol)

A 10 mo old Ashkenazi Jewish girl manifests hypotonia and macrocephaly. By 18 mo of age, she has hyperreflexia and optic atrophy. Additional problems include failure to thrive and swallowing difficulties. MRI demonstrates diffuse white matter degeneration of the cerebral cortex. The most likely diagnosis is:

Canavan disease - autosomal recessive disorder characterized by spongy degeneration of the white matter of the brain, leads to a severe form of leukodystrophy - deficiency of the enzyme aspartoacylase leads to the accumulation of N-acetylaspartic acid in the brain, especially in white matter, and massive urinary excretion of this compound - excessive amounts of N-acetylaspartic acid also are present in the blood and cerebrospinal fluid - more prevalent in individuals of Ashkenazi Jewish descent than in other ethnic groups. - infants usually appear normal at birth and may not manifest symptoms of the disease until 3 to 6 mo of age when they develop progressive macrocephaly, severe hypotonia, persistent head lag, and delayed milestones - as the disease progresses, there is spasticity, joint stiffness, and contractures - optic atrophy and seizures develop - feeding difficulties, poor weight gain, and gastroesophageal reflux may occur in the 1st yr of life; swallowing deteriorates, and nasogastric feeding or permanent gastrostomy may be required

Rule of 3s regarding crying in infants

Normal/behavioral/dev'tal crying in infants Begins at 3 weeks of age Ends at 3 mos of age Duration commonly 3 hours a day Often starts around 3pm in afternoon

A 4-year-old boy is seen in the emergency department for 6 hours of progressive restlessness, abnormal movements of the extremities, blurred vision, and drooling. His parents report that the boy was in good health until he felt something sharp touch his right great toe as he put on his shoes. His parents examined his toe at the time but were unable to see any marks. Over the course of the next hour he complained of a "funny feeling" in his right leg. Shortly thereafter, the other symptoms commenced. On physical examination, the boy's temperature is 38.2°C, heart rate is 140 beats/min, respiratory rate is 22 breaths/min, blood pressure is 110/60 mm Hg, and his room air oxygen saturation is 100%. He is alert and oriented and able to answer questions appropriately. The boy appears uncomfortable and is intermittently writhing on the stretcher. His pupils are equal, round, and reactive to light; however, he has abnormal extraocular movements. His mucous membranes are moist and he has minimal drooling. On auscultation, his heart has a normal rhythm with no murmurs, scattered expiratory wheezes can be heard; and his abdominal examination findings are normal. On skin examination, no marks are found and there is no bruising. Palpation of his right great toe produces intense pain. Of the following, the creature MOST likely to have caused this child's symptoms is

Centruroides sculpturatus (Arizona bark scorpion) - Scorpion stings present with a mixture of sympathetic, parasympathetic, and neuromuscular symptoms. - Supportive care is the mainstay of management for scorpion stings. - Scorpion antivenom can be considered for use in severe cases.

Leading cause of morbidity and mortality during drowning

Cerebral hypoxia - injury likely begins 3-5 minutes after submersion ~ multifactorial ~ not reversible

Recommended management for a mother with active genital HSV infection during labor is:

Cesarean section within 4 hr of rupture of membranes - For pregnant women with active genital herpes at the time of delivery, the risk for mother-to-baby transmission can be reduced but not eliminated by delivering the baby via a cesarean section (within 4-6 hr of rupture of membranes).

Nonfatal drowning is a common injury in pediatrics. Children may be asymptomatic after drowning or they may have severe respiratory symptoms, anoxic brain injury, or multi-organ dysfunction due to hypoxia. When can children be discharged home safely?

Children with a normal chest X-ray and no symptoms eight hours after arrival to emergency department can be discharged home safely - Most pediatric patients with respiratory compromise will develop symptoms within six to eight hours of the drowning event - They should be discharged in the company of a responsible adult with strict instructions to return if the child develops any difficulty breathing. - Children who cannot be discharged in the company of a responsible adult should be admitted for further monitoring.

A 12-year-old girl presents to the emergency department with a firm anterior neck mass. Her mother first noticed it this morning, but the patient says it has been slowly growing over the last few weeks. The patient is short for her age but denies weight changes, palpitations, constipation, diarrhea, or difficulty with temperature changes. Her exam is only remarkable for an enlarged and firm but nontender goiter. Laboratory results show normal free T4 and thyroid-stimulating hormone values. Which of the following is the most likely diagnosis?

Chronic lymphocytic thyroiditis (i.e., chronic autoimmune thyroiditis, Hashimoto thyroiditis) - common childhood illness that mostly affects girls and Caucasian patients, with 1 in 20 of all children showing serologic evidence of this disease. - especially common in children with Down syndrome or Turner syndrome. - rarely occurs in infants and is common after 6 years of age but most often presents in adolescents. - organ-specific autoimmune disease that results in infiltration of lymphocytes into the thyroid. - Many patients will have high levels of antithyroid peroxidase antibodies and antithyroglobulin antibodies. - Most patients with chronic lymphocytic thyroiditis are euthyroid and only present with a firm and nontender goiter found on a routine examination without other symptoms, as in the patient above. - An ultrasound is indicated if the thyroid is nodular on exam. - If nodules are large or suspicious for cancer, fine-needle aspiration should be arranged. - most common cause of hypothyroidism in children even though most cases are euthyroid.

Individual treatment for PTSD involves transforming the child's concept of himself or herself as victim to that of survivor and can occur through play therapy, psychodynamic therapy, or cognitive behavioral therapy (CBT). Group work is also helpful for identifying which children might need more intensive assistance. What may be helpful for sleep disturbance, persistent arousal, and exaggerated startle response?

Clonidine or guanfacine - Comorbid depression and affective numbing might respond to a selective serotonin-reuptake inhibitor (SSRI). - As for many other anxiety disorders, CBT is the psychotherapeutic intervention with the most empirical support.

During a high school football game, a 17-year-old boy collides with another player, causing a forceful collision of the two players' heads. The boy presents later in the afternoon to the urgent care where you are working. He reports some mild nausea, dizziness, and fatigue, but is alert and appropriately interactive, with a nonfocal neurological exam. He and his father provide you with the history that he was wearing a helmet at the time of injury. He sustained no loss of consciousness following the injury, and he was able to walk off the football field unassisted to sit out the rest of the game on the sideline. The boy is eager to play in his football team's game next weekend and is requesting your medical clearance to do so. Of the following, which is the most appropriate to recommend?

Complete resolution of symptoms before return to play - Current guidelines in managing concussions include ensuring complete resolution of concussion signs and symptoms before clearing a patient to return to competing in their sport(s) of choice. - Thus, any athlete with a suspected sport-related concussion should be removed from sport-related activity on the day of injury and monitored closely for concussive signs and symptoms. - Cognitive and physical rest is indicated for typically at least 24 to 48 hours. - Thus, both cognitive and physical rest are indicated for full recovery from a concussion. - When the athlete is cleared to return to play their sport, a progression from low-intensity to normal sport activity should be undertaken before the athlete's return to competition. Clearance for an athlete to return to play is also dependent on the athlete not having recurrence of concussion symptoms with sport participation.

A 15 yr old girl who had new-onset seizures was started on carbamazepine 4 wk ago. She now has a diffuse erythematous macular rash, fever, lymphadenopathy, eosinophilic leukocytosis, and elevated values on liver function tests. The most likely cause of these abnormalities is:

DRESS syndrome / drug rash with eosinophilia and systemic symptoms - formerly called anticonvulsant hypersensitivity syndrome and pseudolymphoma syndrome - classically seen 1-6 wk after initial exposure to an aromatic anticonvulsant or other drugs (allopurinol, minocycline, sulfonamides, other antibiotics) and often manifest as the triad of fever, rash, and hepatitis. - rash is characterized by a pruritic, diffuse, erythematous to urticarial eruption of coalescing plaques - eosinophilia (≥500/μL) occurs in up to 30% of patients - hepatitis ranging from mild elevation of liver transaminase values to frank hepatic failure may also be accompanied by interstitial nephritis, pneumonitis, myocarditis, shock, and encephalitis - late-onset (several months) thyroiditis and hypothyroidism may occur as a result of antimicrosomal antibodies directed against thyroid peroxidases involved in drug metabolism

A 13 yr old girl with hyperthyroidism has chronic infection of her skin and nails with Candida albicans. She has never required hospitalization or had life-threatening infection. She had chickenpox at age 11 mo from which she had an uneventful recovery, and has received her other routine childhood vaccinations without problems. Which of the following tests often yields an abnormal result in a child with this condition?

Delayed-type hypersensitivity testing against Candida - Chronic mucocutaneous candidiasis is a rare immunodeficiency associated primarily with T-cell dysfunction. - These patients might not demonstrate delayed hypersensitivity to skin tests for Candida antigen despite having chronic superficial infection with yeast, but they do not appear to be at increased risk for systemic yeast infections.

A 4 yr old girl is admitted to the hospital for her third evaluation for vaginal bleeding. The mother noted bright red blood on the child's underwear. Previous examinations revealed a normal 4 yr old girl, Tanner stage 1, with normal external genitalia. Pelvic ultrasound results were normal, as was the serum estradiol level. The hemoglobin and platelet counts were normal, as were the bleeding time and coagulation studies. Findings on pelvic examination conducted under anesthesia also were normal. The next step in the examination is to:

Determine the blood type of the blood on the underwear - Consideration of factitious disorder by proxy should be triggered when the reported symptoms are repeatedly noted by only one parent, appropriate testing fails to confirm a diagnosis, and seemingly appropriate treatment is ineffective. - Preverbal children are usually involved. Bleeding is a particularly common presentation. - This may be caused by adding dyes to samples, adding blood (e.g., from the mother) to the child's sample, or giving the child an anticoagulant (e.g., warfarin).

A newborn in the NICU is intubated and on total parenteral nutrition, including lipid emulsions. A blood culture yields Malassezia furfur. The most important element of treatment for this infection is:

Discontinuing intravenous lipid infusion - Symptoms of catheter-associated fungemia are indistinguishable from other causes of cathether-associated infections but should be suspected in patients, especially neonates, receiving intravenous lipid infusions. - Compared with other causes of fungal sepsis, it is unusual for catheter-related Malassezia fungemia to be associated with secondary focal infection. - Fungemia caused by M. furfur can be successfully treated in most cases by immediately discontinuing the lipid infusion and removing the involved catheter. - For persistent or invasive infections, amphotericin B (desoxycholate or lipid-complex formulations), fluconazole, and itraconazole are effective.

What helps infants with pulmonary parenchymal etiologic origins + those with pulm HT as causes of cyanosis?

Hyperoxygenation

What fluids are recommended for pediatric patients who are considered to be at high-risk of antidiuretic hormone (ADH) secretion (e.g. post-surgical patients, respiratory infections, neurologic disease)?

Isotonic saline (e.g. D5 NS) for maintenance requirements

A 13 yr old girl develops ptosis, diplopia, and difficulty swallowing but is afebrile and otherwise in good health. On examination, her ptosis is noted to increase with sustained upward gaze for 30 sec. No weakness of the extremities or limb girdles is detected, and she has no myalgias or tenderness of muscles to palpation. The initial diagnostic test most likely to confirm the diagnosis is:

Edrophonium (Tensilon) test - A clinical test for myasthenia gravis is administration of a short-acting cholinesterase inhibitor, usually edrophonium chloride. - Ptosis and ophthalmoplegia improve within a few seconds, and fatigability of other muscles decreases.

Typical dosing of epinephrine in pediatric asthmatic pts

Epinephrine 1:1,000 = 0.01mg/kg IM Q20min x 3

What are the four forms of neonatal brachial plexus injury?

Erb palsy involves C5-C6 Klumpke palsy involves C8-T1 total arm paralysis involves C5-T1 Horner syndrome involves the sympathetic outflow of the T1 nerve root only

Which syndrome has autoimmune hemolytic anemia with concomitant immune thrombocytopenic purpura?

Evans syndrome

A 4 yr old girl sustained a 40% 2nd- and 3rd-degree total body surface area (BSA) burn from scalding hot water. Of the following, the most therapeutic approach is:

Excision of burn wounds and grafting - Deep 2nd-degree burns of >10% of BSA benefit from early excision and grafting. - To improve outcome, sequential excision and grafting of 3rd-degree and deep 2nd-degree burns is required in children with large burns. - Prompt excision with immediate wound closure is achieved with autografts, which are often meshed to increase the efficiency of coverings.

A 14-year-old previously healthy boy presents with an episode of not being able to move his right arm and right leg. This started about two hours ago. He is also having headache and vomiting. He says his vision was blurry earlier but is now back to normal. He has no fever, head trauma, rash, or neck pain. He denies any ingestions. His mother has had several similar episodes in the past. On exam, you note a tall, thin boy in no acute distress. There is mild weakness in the right upper extremity. He says he now feels better and is asking for food. What is the most likely etiology of this episode?

Familial channelopathy unilateral weakness in conjunction with headache, vomiting, and visual changes + also a family history of similar episodes = diagnosis of familial hemiplegic migraine - caused by a familial channelopathy, specifically a mutation in a protein which codes for sodium and calcium channels. - weakness is considered a motor aura. - neuroimaging such as magnetic resonance imaging is typically normal. - treatment includes similar medications to those used for other types of migraine with aura, including corticosteroids and valproic acid.

Similar to the microbiology of AOM, the most common etiologic agent of acute mastoiditis is

Streptococcus pneumoniae - Intravenous antimicrobial agents should be targeted against pathogens causing AOM, especially S pneumoniae and S aureus (including methicillin-resistant S aureus), for example, vancomycin plus ampicillin-sulbactam. In the setting of recurrent AOM and recent antibiotic use, some experts prefer a combination of vancomycin plus ceftazidime or cefepime (to cover P aeruginosa).

Placed in those children who cannot safely feed orally or who cannot take feed directly into stomach. They may be used as alternative to fundoplication or in cases of failed fundoplication.

GJ tubes - both GJ and NJ tubes are subject to intussusceptions

A 4 mo old girl presents with developmental delay, an exaggerated startle response to loud noise, and macrocephaly. On physical examination, the child has decreased eye contact and a cherry-red spot in each retina. The most likely diagnosis is:

GM2 gangliosidosis - include Tay-Sachs disease and Sandhoff disease; each results from the deficiency of β-hexosaminidase activity and the lysosomal accumulation of GM2 gangliosides, particularly in the central nervous system - clinical manifestations of Sandhoff disease are similar to those for Tay-Sachs disease - diagnosis of infantile Tay-Sachs disease and Sandhoff disease is usually suspected in an infant with neurologic features and a cherry-red spot - affected infants usually develop normally until 4 to 5 mo of age when decreased eye contact and an exaggerated startle response to noise (hyperacusis) are noted - Macrocephaly, not associated with hydrocephalus, may develop - In the 2nd yr of life, seizures develop that may be refractory to anticonvulsant therapy - Neurodegeneration is relentless, with death occurring by the age of 4 or 5 yr

Generalized #/volume of bottle feedings for infants

General rule of thumb: increase by 1oz/feed each month before leveling off at 6-8 months Birth - 1 week = 8-12 feeds/day - 1-3 ounces/feed 1 month - 3 months = 7-8 feeds/day - 2-4 ounces 3 months - 6 months = 5-7 feeds/day - 4-6 ounces 6 months - 9 months = 3-5 feeds/day - 6-8 ounces 10 months - 12 months = 3-4 feeds/day - 6-8 ounces/feed

The recommended treatment for tularemia is administration of:

Gentamicin or streptomycin - All strains of F. tularensis are susceptible to gentamicin and streptomycin. - Gentamicin (5 mg/kg/day divided bid or tid IV or IM) is the drug of choice for the treatment of tularemia in children because of the limited availability of streptomycin (30-40 mg/kg/day divided bid IM) and the fewer adverse effects of gentamicin.

Small, papular periareolar projections that may play a role in lactation. They can become occluded, leading to accumulation of fluid and the formation of a periareolar mass that may remain asymptomatic or become superinfected and lead to mastitis.

Glands of Montgomery - The ultrasonographic appearance of a cyst of Montgomery is that of a simple subareolar cyst. - Expectant management is recommended since the vast majority of cysts resolve spontaneously over weeks to months, with few cases resolving by two years.

A 16-year-old adolescent boy presents to the emergency department for evaluation of a painful skin lesion on his left lower extremity that has been progressing over the last 3 days. Three days ago, he scratched his left lower calf while climbing over a fence in the playground. The next day, he noticed some redness surrounding the scratch site, but he did not seek medical attention. Over the next 24 hours, he noted a marked increase in pain (9 out of 10) at the site. He also began to feel warm, had a decrease in appetite, and complained of diffuse achiness and malaise. He has been previously well and is not taking any medication. His vital signs show a temperature of 39.2°C, a heart rate of 110 beats/min, a blood pressure of 120/76 mm Hg, and a respiratory rate of 22 breaths/min. Physical examination in the emergency department shows an ill-appearing adolescent. The left lower extremity has a 4 × 5 cm erythematous area surrounding a scratch mark with bluish discoloration. The area is exquisitely sensitive to touch. There is no swelling or discharge at the site. The remainder of the examination findings are unremarkable. Of the following, the MOST likely cause of this lesion is infection with

Group A Streptococcus PREP Pearls · Necrotizing fasciitis is clinically characterized by an exquisitely painful, tender lesion with bluish discoloration at the site of infection. · Surgical debridement is a critical component of the management of necrotizing fasciitis. · Group A streptococcus is the leading cause of necrotizing fasciitis in the previously normal host.

Recurrent erythema multiforme is associated with:

HSV - Among the numerous factors implicated in the etiology of erythema multiforme (EM), infection with herpes simplex virus (HSV) is the most common. - Presence of the human leukocyte antigens A33, B62, B35, DQB1*0301, and DR53 is associated with an increased risk of HSV-induced EM, particularly the recurrent form. - Most patients experience a single self-limited episode of EM. - Lesions of HSV-induced recurrent EM (HA-EM) typically develop 10-14 days after onset of recurrent HSV eruptions, have a similar appearance from episode to episode, but may vary in frequency and duration in a given patient.

most common childhood cancer in the 15- to 19-year-old age group

Hodgkin lymphoma - present with painless lymphadenopathy, which is usually cervical, supraclavicular, axillary, or inguinal. - typically feel rubbery and more firm than inflammatory adenopathy. - may be sensitive to palpation if they have grown rapidly. - may present with nonspecific systemic symptoms, including fatigue, anorexia, and weight loss. - classified to have "B" symptoms when they present with fever (> 38.0°C), drenching night sweats, and weight loss (≥ 10 percent loss within six months before diagnosis).

While rounding on the inpatient pediatric floor as the attending hospitalist, your medical student asks if it is better for healthcare providers to wash their hands with soap and water or to cleanse them with an alcohol-based hand disinfectant before and after examining patients. The use of which of the following is both most advisable and practical to prevent the spread of infection?

Hand washing with alcohol-based hand rub before and after patient encounter - entails using either an alcohol-based hand rub or washing hands with soap and water before and after each patient encounter to prevent the direct transmission of pathogens. - the alcohol-based disinfectants come in gel, rinse, or foam types. - use of either method of hand cleansing is acceptable for most types of patient encounters, such as performing hospital rounds, but the hands must be cleaned both before and after patient contact. - as hand cleaning with an alcohol-based hand rub is just as effective as hand washing with soap and water in most situations for preventing nosocomial microbe transmission, and is more convenient and quicker than hand washing with soap and water, the use of alcohol-based hand rubs is the preferred method of hand cleansing before and after most patient encounters.

The parents of a 3 yr old girl report that "she ran before she walked," "she is never hungry at the same time," and "she goes from toy to toy." This child is best described as:

Having developmental pervasive disorder Classic theory of Thomas and Chess proposes 9 dimensions of temperament --> 3 common constellations: (1) the easy, highly adaptable child, who has regular biologic cycles (2) the difficult child, who withdraws from new stimuli and is easily frustrated (3) the slow-to-warm-up child, who needs extra time to adapt to new circumstances. Various combinations of these clusters also occur.

A 2-day-old male in the newborn nursery is found to have a left preauricular pit. He is the product of a full-term uncomplicated pregnancy. Physical exam is otherwise normal, and he has been doing well in the nursery. Which of the following is the most appropriate next step?

Hearing screen - Preauricular pits are present in up to 10% of newborns, depending on race. They are typically unilateral but may be bilateral in 25-50% of cases. Permanent hearing loss occurs in these patients at a rate five times higher than the general population, thus, a hearing screen is recommended in all affected patients. - Preauricular pits may be associated with other anomalies, as in branchio-oto-renal syndrome, which can also present with sensorineural hearing loss, branchial cysts/tracts, renal abnormalities, and malformation of the ears. CHARGE syndrome and DiGeorge syndrome also may be associated with ear anomalies. - Preauricular pits may have drainage and may become infected, and in the setting of repeated infection or discharge, surgical excision may be considered.

Symptoms of carbon monoxide poisoning/carboxyhemoglobin are nonspecific and can include altered mental status, headache, malaise, nausea, and emesis. Carbon monoxide poisoning is diagnosed by using blood gas carbon monoxide oximetry. What is the mainstay of treatment for carbon monoxide poisoning. Severe poisoning may necessitate hyperbaric oxygen therapy.

High-flow oxygen administration via non-rebreather mask - The US Consumer Product Safety Commission recommends that a carbon monoxide detector with alarms be installed on each level of every home; if battery-operated, the batteries should be changed biannually at daylight saving time.

What common cause of respiratory alkalosis leads to temporary signs of hypocalcemic carpopedal spasm?

Hyperventilation from a panic attack.

The boy in this vignette—with a history of prior sore throat, cola-colored urine, hematuria, proteinuria, hypertension, azotemia, elevated level of antistreptolysin O, and a low complement 3 level—has acute poststreptococcal glomerulonephritis (PSGN). The presence of edema and hypertension reveal that he has fluid retention. The most appropriate treatment is to administer

IV furosemide - Poststreptococcal glomerulonephritis can present with subclinical features or clinically apparent acute glomerulonephritis. - Poststreptococcal glomerulonephritis is characterized by a low serum complement C3 value and a normal serum complement C4 value. - Hypertension and edema in children with poststreptococcal glomerulonephritis, resulting from sodium and fluid retention, are best treated with a potent diuretic such as furosemide.

Infant has severe thrombocytopenia. Perinatal thrombocytopenia can be productive (eg, a genetic bone marrow failure syndrome such as congenital amegakaryocytic thrombocytopenia), consumptive (eg, disseminated intravascular coagulation), or destructive (ie, maternal alloantibodies to the neonate's platelets). In this infant, mother's new onset of severe thrombocytopenia, the most likely cause of the neonate's thrombocytopenia is a maternal immune thrombocytopenic purpura (ITP). Maternal ITP results from the passive transplacental transmission of a maternally derived antibody to a surface antigen found on both maternal and neonatal platelets, resulting in thrombocytopenia in both the mother and the neonate. Both maternal ITP and NAIT are self-limiting, requiring only temporary support. Transmission of the alloantibody in both cases ceases at birth, and the antibody titer drops as it binds to neonatal platelets. Maternal ITP can be temporized through the administration of

IVIG

Troubleshooting ventilator settings...

If patient fighting ventilator and desaturating immediate measures include: -DOPE TROUBLE SHOOTING- D-Displacement -check tube placement. - when in doubt take ET Tube out and start manual ventilation with 100% O2 and with bag and mask. O-Obstruction -is the chest rising. - are breath sounds present and equal? - changes in examination? - atelactasis, treat bronchospasm/tube block/malposition/pneumothorax(consider needle thoracocentesis). Examine circulation:?Shock, ?Sepsis. P-Pneumothorax -check ABG, saturation and CXR for pneumothorax and worsening lung condition. E-Equipment failure -examine ventilator, ventilator circuit/humidifier/gas source. - if no other reason for hypoxemia :- increase sedation/muscle relaxation, put back on the ventilator.

For all patients with Stage 2 hypertension confirmed on at least two visits, what are the next steps?

If symptomatic and/or BP is markedly elevated (ie BP>30 mmHg above the 95th percentile), refer to the emergency department If asymptomatic, perform the following evaluation: - Check upper and lower extremity blood pressure - Order the following: Chem 10, urinalysis, CBC, renin, aldosterone, TSH, serum catecholamines and renal ultrasound - Place urgent referral to pediatric nephrology and specify need for Ambulatory Blood Pressure Monitoring

A 6-year-old boy presents to the emergency department with muscle spasms. Over the past four days, he has had progressively worsening bouts of pain and stiffening of his body. Initially, he mainly complained of bilateral hand and arm pain, but the bouts are now more diffuse. He became febrile yesterday, and he slept poorly last night, sweating through the bedsheets. His parents report that he is overall healthy, but he did have a few deep splinters in his right hand from playing in the sandbox in their home 10 days ago, which they removed with tweezers. He has not received childhood vaccinations and has no known sick contacts. His vital signs reveal a temperature of 38.3°C, HR 124 beats per minute, respiratory rate 24 breaths per minute, BP 108/72 mm Hg. On physical examination, he appears tearful and is diaphoretic. During his oropharyngeal exam, his masseters spasm when his gag reflex is elicited. Abdominal exam is positive for rigidity, making palpation difficult, and auscultation reveals hyperactive bowel sounds. On musculoskeletal exam, it is difficult to perform passive flexion of his extremities. His skin exam shows a partially healed lesion on his right hand which is tender to palpation, but no obvious foreign body is appreciated. On neurologic examination, he is alert but irritable. He has diffuse hypertonicity. On pupillary examination, he suddenly has an episode where he clenches his fists, abducts his arms, extends his legs, and arches his back, crying out in intense pain. Which of the following is the next best step in management?

Immunoglobulin should be administered to bind any free toxin Tetanus - organism does not invade tissue but instead produces a potent toxin called tetanospasmin or tetanus toxin. - variety of forms including neonatal tetanus, cephalic tetanus, localized tetanus, and generalized tetanus. - other common symptoms include stiff neck, opisthotonus (arching of the head, neck, and spine), sardonic smile, and rigid abdomen. - autonomic hyperactivity is common as well, including irritability, restlessness, sweating, tachycardia, fever, and labile blood pressures. - on exam, he develops reflex spasm with use of tongue blade and is hypersensitive to light from the ophthalmoscope, both of which are typical in tetanus. - wound management is essential: debriding any devitalized tissue and ensuring no nidus for further propagation. - Human tetanus immune globulin should be given as soon as possible in order to bind any free toxin that may still be present. - Antibiotics, generally metronidazole, are part of the standard treatment regimen.

A 6 yr old child with meningococcal meningitis develops a swollen left knee on the 5th day of antibiotic treatment. Which of the following is the most likely cause for this finding?

Immune complex deposition resulting from production of anti-meningococcal antibodies - Nonsuppurative complications of meningococcal disease appear to be immune complex mediated and become apparent 4-9 days after the onset of illness. - Arthritis and cutaneous vasculitis (erythema nodosum) are most common. The arthritis usually is monarticular or oligoarticular, involves large joints, and is associated with sterile effusions that respond to nonsteroidal anti-inflammatory agents. - Long-term sequelae are uncommon. - Because most patients with meningococcal meningitis become afebrile by the 7th hospital day, persistence or recrudescence of fever after 5 days of antibiotics warrants evaluation for immune complex-mediated complications.

A 3-year-old boy presents to the emergency department for a skin lesion. Over the past several days, he has developed swelling of his right forearm. This morning a yellowish fluid spontaneously drained from the site. The lesion is overlying a particularly dry and irritated area of skin, which his parents note he has been scratching at for the past week. He has not been febrile. They routinely use emollients and topical steroids on his skin. His history is significant for recurrent Staphylococcus aureus abscesses and necrotizing pneumonia, which required surgical intervention at 12 months of age. He has had a pruritic rash mainly affecting his face, upper extremities, and trunk that has been difficult to control since a few weeks after he was born. His family history is positive for skin infections and atopic dermatitis in multiple individuals. His vital signs are T of 37.8° C, HR 106 beats/min, RR 28 breaths/min, and BP 94/60 mm Hg. On physical examination, he has coarse facial features with a prominent nose and broad nasal bridge. His right arm has a 4 cm area of swelling on the extensor forearm without surrounding erythema. Palpation further expels purulent yellow fluid. He has erythematous maculopapular plaques at the extensor surfaces of his elbows, upper arms, shoulders, and right cheek, some of which have silvery scales overlying them. Laboratory data are shown: White blood cell count: 8,000/microL Hemoglobin: 12.4 g/dL Platelet count: 340 x 103/microL Absolute neutrophil count: 3,200/microL Absolute lymphocyte count: 1,000/microL Absolute eosinophil count: 3,800/microL Total IgE: 10,500 units/mL What is the most likely underlying pathophysiology in this patient?

Impaired neutrophil chemotaxis - This patient has a skin abscess in the context of recurrent skin abscesses and eczematous skin lesions. Family history is positive for family members with a similar history. - His physical exam reveals coarse facial features, lichenified eczematous skin rash, and an abscess without surrounding erythema. - Laboratory evaluation shows a normal neutrophil count, marked eosinophilia (~50% of differential), and a very elevated IgE count. Hyperimmunoglobulin E syndrome, also known as Job syndrome, is an autosomal dominant condition characterized by eczematous skin rashes and frequent skin and respiratory infections. - The onset of the skin rash is typically shortly after birth and involves the extensor surfaces and face. - Coarse facial features are a common part of the phenotype of these individuals. - Infections may be invasive and require surgical management. Candidal infections of the nails are common as well. - These findings are secondary to defects in the Janus-activated kinase-signal transducer and activator of transcription (JAK-STAT) pathway, which leads to poor T helper cell differentiation and function. This leads to a number of abnormalities, including impairment in neutrophil chemotaxis, making the patient susceptible to bacterial infections. - Patients may have "cold abscesses" that do not show expected inflammatory signs yet develop pus and have a normal level of neutrophils on laboratory examination. - Osteoporosis and increased risk of fractures are secondary to increased osteoclastic activity by monocytes. - Laboratory abnormalities include a markedly elevated IgE, the degree of which is not directly related to disease severity. - Eosinophilia, often 40-50% of the differential, is another associated finding that is more suggestive of hyperimmunoglobulin E syndrome as it is generally higher than seen in atopic dermatitis.

The laboratory calls at 8:45 AM to inform you that a blood culture obtained in the emergency department yesterday from a 10 mo old febrile girl who is followed in your practice is growing Streptococcus pneumoniae. You contact her parents, and they report that she is "doing well." The most appropriate next step in management would be to:

Instruct the parents to bring the child to your office as soon as possible to be re-examined - If blood cultures are obtained and S. pneumoniae is isolated from the blood, the child should return to the physician as soon as possible after the culture results are known. - If the child appears well, is afebrile, and has a normal physical exam, a 2nd blood culture should be obtained and the child should be treated with 7-10 days of oral antimicrobial therapy. - If the child appears ill and continues to have fever with no identifiable focus of infection at the time of follow-up, or if H. influenzae or N. meningitidis is present in the initial blood culture, the child should have a repeat blood culture, be evaluated for meningitis (including lumbar puncture), and receive treatment in the hospital with appropriate intravenous antimicrobial agents.

An 8-year-old boy presents to the emergency room with a two-week history of polyuria and polydipsia and lethargy for one day. On physical examination, he appears dehydrated with dry mucous membranes and doughy skin. The initial laboratory findings are significant for a serum sodium of 164 mEq/L, potassium of 4 mEq/L, chloride of 120 mEq/L, bicarbonate of 23 mEq/L, serum glucose of 90 mEq/L and blood urea nitrogen of 46 mg/dL. The urinalysis is normal with a urine specific gravity of 1.004. The child receives IV fluids and then a four-hour water deprivation test is performed. Throughout the test, the urine output rate is 5 to 6 mL/kg per hour without any change in rate. After vasopressin injection, the urine output decreases to 2 mL/kg per hour and urine osmolality increases to over 100 mosmol/kg. Which of the following is the next best step in the management of this patient?

Intranasal desmopressin central diabetes insipidus - occurs due to lack of secretion of antidiuretic hormone from the posterior pituitary - since antidiuretic hormone works to concentrate urine, these children have large quantities of dilute urine. The treatment of choice is intranasal desmopressin, which is a potent diuretic that is similar in structure to antidiuretic hormone. - boy in this vignette continues to produce excessive urine despite water deprivation. However, after the vasopressin injection the urine output decreased and the urine osmolality increased to 100 mosmol/kg, which is diagnostic for central diabetes insipidus. - Vasopressin is given to differentiate central from nephrogenic diabetes insipidus.

In the United States, how common is suicide as the cause of death in children and adolescents?

It is the third-leading cause of death.

Should be suspected in a young child (typically ages 1-7 years) with intermittent and painless rectal bleeding.

Juvenile polyp(s) - Most found in the distal colon (sigmoid and rectum) - Once resected, follow-up colonoscopy is not indicated. However, if more than 5 polyps are detected, or if the location of the polyps is in the proximal colon, juvenile polyposis syndrome should be suspected. Evaluation should include genetics and oncology consultation, and repeat colonoscopy every 1 to 3 years.

Characterized by a rapidly enlarging lesion, thrombocytopenia, microangiopathic hemolytic anemia, and coagulopathy as a result of platelet and red blood cell trapping and activation of the clotting system within the vasculature of the hemangioma. This syndrome has been shown to be associated with kaposiform hemangioendotheliomas or tufted angiomas.

Kasabach-Merritt syndrome

Guidelines recommend considering a statin in those with risk factors who have

LDL persistently 160 mg/dL or higher despite dietary management

A 7 mo old boy has been healthy and developing normally since birth. His mother now reports that he has decreased eye contact with her, even during feedings. The infant also startles very easily when there is a loud noise in the house. Of the following, the most appropriate diagnostic test to confirm the etiology of these findings is the measurement of:

Leukocyte β-hexosaminidase A activity Patients with the infantile form of GM2 gangliosidoses have clinical manifestations in infancy including loss of motor skills, increased startle reaction, and macular pallor and retinal cherry-red spots. - Affected infants usually develop normally until 4 to 5 mo of age when decreased eye contact and an exaggerated startle response to noise (hyperacusis) are noted. - Macrocephaly, not associated with hydrocephalus, may develop. - Definitive diagnosis is made by determination of β-hexosaminidase A and B activities in peripheral leukocytes. - The two GM2 gangliosidoses are distinguished by the enzymatic assay, because in Tay-Sachs disease only the β-hexosaminidase A isozyme is deficient, whereas in Sandhoff disease both the β-hexosaminidase A and B isozymes are deficient.

A standard concentration of a microorganism is inoculated into serially diluted concentrations of antibiotic, and the minimum inhibitory concentration (MIC) in μg/mL, the lowest concentration of antibiotic required to inhibit growth of the microorganism, is determined. Dilution testing also permits determination of the minimum bactericidal concentration (MBC), the lowest concentration of antibiotic required to kill the organism. The MBC is sometimes determined to exclude the possibility of bacterial tolerance, which is defined as...

MBC > 4 times the MIC

Most common type of skull injury after head trauma from both unintentional and inflicted mechanisms.

Linear, parietal skull fractures - Skull fractures in children younger than 2 years of age without a plausible history of injury should raise the clinician's concern and prompt a more thorough evaluation for inflicted trauma. - Well-appearing children with linear skull fractures and no associated intracranial injury may be considered for discharge after a period of observation in the emergency department if child abuse is not suspected.

A male neonate, born at 34 weeks' gestation, is being evaluated for respiratory distress shortly after delivery. The mother reports no medical problems during the pregnancy; prenatal care records are not available. Maternal group B Streptococcus status is unknown. The mother was febrile, with a maximum temperature of 39°C, for 6 hours before delivery. The neonate was delivered vaginally through green amniotic fluid. On physical examination, he is noted to have grunting and tachypnea. The remainder of his examination findings are unremarkable. He is started on continuous positive airway pressure +5 cm H2O and 30% oxygen in the delivery room. Chest radiography shows bilateral streaky infiltrates. Of the following, the pathogen MOST likely to be responsible for this neonate's findings is

Listeria monocytogenes - Neonates born to mothers with Listeria infection are often delivered prematurely with brown- or green-stained amniotic fluid. Among premature infants, meconium-stained amniotic fluid is uncommon. - Early-onset neonatal Listeria is uncommon, with a high rate of mortality. These neonates may present with respiratory distress and an erythematous papular rash. - Late-onset neonatal Listeria typically presents with nonspecific findings among term infants at 14 days after birth. Meningitis is more common with late-onset neonatal Listeria.

A 13 yr old boy is undergoing evaluation for Cushing syndrome. His cortisol level is unchanged following dexamethasone 30 µg/kg/day in 4 divided doses but decreases to 4 µg/dL following 120 µg/kg/day in 4 divided doses. The next step(s) in his evaluation should include:

MRI of his head with and without contrast - 2-step dexamethasone suppression test consists of administration of dexamethasone, 30 and 120 μg/kg/24 hr in 4 divided doses, on consecutive days - in children with pituitary Cushing syndrome, the larger dose, but not the smaller dose, suppresses serum levels of cortisol - CT detects virtually all adrenal tumors larger than 1.5 cm in diameter. MRI may detect ACTH-secreting pituitary adenomas, but many are too small to be seen; the addition of gadolinium contrast increases the sensitivity of detection

What is the most common ovarian neoplasm in adolescents?

Mature cystic teratoma (dermoid cyst) - most are benign and contain mature tissue of ectodermal (skin, hair, sebaceous glands), mesodermal, or endodermal origin - occasionally well-formed teeth, cartilage, and bone are found - calcification on an abdominal radiograph is often a hallmark of a benign teratoma - if the major component of the dermoid is thyroid tissue (struma ovarii), hyperthyroidism can be the clinical presentation - benign teratomas should be carefully resected, preserving as much normal ovarian tissue as possible - oophorectomy (and salpingoophorectomy) for this benign lesion is excessive treatment - during surgery, both ovaries should be evaluated, and if there is any question about the nature of the lesion, the specimen should be evaluated by a pathologist - an association of dermoid tumors with neural elements and anti-NMDA receptor encephalitis has been reported - excision of the ovarian tumor has led to improvement in neurologic symptoms in some patients

A 3 yr old girl presents to the emergency department with anasarca. The urinalysis shows 4+ proteinuria and is negative for blood. Serum albumin is 1.2 mg/dL and serum creatinine is 0.4 mg/dL. The most likely diagnosis is:

Minimal change nephrotic syndrome - 85% of total cases of nephrotic syndrome in children - urinalysis reveals 3+ or 4+ proteinuria, and microscopic hematuria is present in 20% of children - spot urine protein:creatinine ratio exceeds 2.0, and urinary protein excretion exceeds 40 mg/m2/hr - serum creatinine value is usually normal, but it may be abnormally elevated if there is diminished renal perfusion from contraction of the intravascular volume - serum albumin level is < 2.5 g/dL, and serum cholesterol and triglyceride levels are elevated - serum complement levels are normal

Of the following diagnostic tests, the most sensitive for detecting recurrent airway aspiration is:

Modified barium swallow with video fluoroscopy - A modified barium swallow study with video fluoroscopy (video fluoroscopic swallowing study) is generally considered the gold standard for evaluating the swallowing mechanism. - This study is preferably done with the assistance of a pediatric feeding specialist and a caregiver in the attempt to simulate the usual feeding technique of the child. The child is seated in normal eating position, and various consistencies of barium or barium impregnated foods are offered. - This study is more sensitive for demonstrating aspiration than bedside assessment or a traditional barium swallow study. The sensitivity of the modified barium swallow study is such that it occasionally detects aspiration in patients without apparent respiratory abnormalities.

A 15 yr old wrestler sustains a traumatic blow the left ear, resulting in a large hematoma. Appropriate treatment is:

Needle aspiration of the hematoma or Incision and drainage of the hematoma - Auriclar trauma is common in certain sports, and quick drainage of a hematoma can prevent irreversible damage. - Hematoma, with accumulation of blood between the perichondrium and the cartilage, can follow trauma to the pinna and is especially common in teenagers related to wrestling or boxing. - Immediate needle aspiration or, when the hematoma is extensive or recurrent, incision and drainage and a pressure dressing are necessary to prevent perichondritis, which can result in cartilage loss and a cauliflower ear deformity. - Sports helmets should be worn when appropriate during activities when head trauma is possible.

A history of cough and dyspnea 24 hr after working in a corn silo. Examination reveals a normal-appearing young man who is mildly tachypneic with normal findings on lung auscultation. There are no ill contacts. Silo filler disease is typically a result of:

Nitrogen dioxide toxicity Silo filler disease - also referred to as silage gas poisoning or silo filler pneumoconiosis - typically caused by nitrogen dioxide toxicity - nitrogen dioxide is produced in silos (particularly corn silos) within a few hr of filling and reaches a maximum concentration within about 2 days - dangerous concentrations of gas can remain in a closed silo for as long as 2 wk - after entering a silo within this time frame without proper protection, a person may experience various degrees of silo filler disease

ROS (Areas of Emphasis with an Adolescent)

Nutrition: Dietary habits, including skipped meals, special diets, purging methods, recent weight gain or loss Cardiac: syncopal or presyncopal events, chest pain on exercise, history of heart murmur Respiratory: Wheezing/asthma, dyspnea during exercise Neuro: History of significant head trauma/concussion, numbness, tingling, seizures Derm: Acne, moles, rashes, warts GU: Dysuria, urgency, frequency, discharge, bleeding Gync: Menarche, LMP, frequency/regularity, longest interval between periods, duration, dysmenorrhea, menometrorrhagia, reproductive life plan Psychiatric: Assessment for symptoms or feelings of depression

After blunt facial trauma, a 12 yr old appears to have a missing incisor. There is no evidence of the tooth at the scene, and the child does not remember swallowing the tooth. The next step in evaluating the child is to:

Obtain a dental film to look for intrusion - Intruded teeth are pushed up into their socket, sometimes to the point where they are not clinically visible. - Intruded primary incisors can give the false appearance of being avulsed (knocked out.) - To rule out avulsion, a dental radiograph is indicated.

Most common complication of acute hypophosphatemia ...

Rhabdomyolysis - usually in the setting of an acute transcellular shift of phosphorus into cells in a child with chronic phosphorus depletion (anorexia nervosa) - rhabdomyolysis is actually somewhat protective, in that there is cellular release of phosphorus

A teenage girl presents in the emergency department with the story that she got upset with her boyfriend and swallowed a "handful of aspirin" about 4 hr previously. One hour afterward, after she began vomiting, she confessed to her mother what she had done. On examination the patient has normal vital signs and is asymptomatic except for the complaint of nausea. A serum salicylate level is ordered, but the laboratory reports no salicylates in her blood. The most appropriate next step in management is to:

Order an acetaminophen level - Acetaminophen is a widely available medication and a commonly detected co-ingestant with the potential for severe toxicity. - Given that patients might initially be asymptomatic and might not report acetaminophen as a co-ingestant, an acetaminophen level should be checked in all patients who present after an intentional exposure or ingestion.

A 12-year-old girl is brought to the emergency department for new-onset clumsiness. Her parents report that over the past week she has been tripping over small things, like steps into their house or curbs. The girl says it is especially hard to walk up stairs. Her lower back aches all over, but there is no shooting pain into her legs or pain in her calves or feet. Her feet feel a little numb and tingly, but her hands and arms feel normal. She denies urinary or stool incontinence. She had a fever and cough 2 weeks ago, and missed several days of school, but otherwise has been well. The girl's vital signs are normal. Her neurologic examination shows normal eye movements, and she can puff out her cheeks and purse her lips normally. She has full strength in both upper extremities. She is able to rise from a chair quickly without using her hands, but has difficulty standing on her tiptoes and on her heels. She can feel light touch on her face, arms, and legs, and all over her back. No reflexes are elicited from her arms or legs. When walking, she lifts her thighs up high; her little brother says she looks like a pony when she walks. The remainder of her physical examination findings are unremarkable. A negative inspiratory force measurement is obtained, and it is normal. Of the following, the next BEST management step for this girl is to:

Perform a lumbar puncture - The girl in the vignette has Guillain-Barré syndrome (GBS). The best next step is to perform a lumbar puncture to obtain a cerebrospinal fluid (CSF) specimen. Typically, the CSF results show cytoalbuminologic dissociation, with a high protein and normal white blood cell count. Early in the course of GBS, CSF studies may be normal. - Symptoms of GBS include ascending paralysis, low back pain, and distal dysesthesias. - Cerebrospinal fluid white blood cells more than 50 per high-power field should prompt a search for concurrent infection. - Treatment for pediatric GBS is typically intravenous immunoglobulin. Supportive care required for GBS includes close monitoring for progression of weakness, especially of the respiratory muscles, and monitoring for evidence of dysautonomia including cardiac arrhythmias, hypertension, bladder dysfunction, and ileus.

In female patients with documented anti-NMDA receptor encephalitis, which imaging test should be considered?

Pelvic imaging such as ultrasound or MRI should be considered, as there exists an association between ovarian teratomas and anti-NMDA receptor encephalitis, presumably via a paraneoplastic mechanism.

A 3-year-old boy is seen for a health supervision visit. His parents express concern that their son is not speaking at the same level as his 3-year-old cousin. They state that he can "understand everything," but his language does not include combinations of more than 2 words. His parents understand approximately half of what he says. The boy enjoys running around with the other children at his preschool. He can draw lines but not circles and can stack up to 4 blocks. He needs assistance with removing his clothing. The boy is friendly, makes good eye contact, and smiles on being greeted. He looks confused when asked to hand over his jacket, but does so willingly when the jacket is pointed to and a hand held out for it. He looks to his parents for reassurance during the physical examination. The boy's growth has been appropriate. His vital signs and physical examination findings are unremarkable. When offered a choice of stickers, he points to the one he wants and says "Thank you." Of the following, the BEST next management step for this boy is to

Perform a general development screen - For a child with speech or language delay, a general developmental screen should be administered to determine if he/she has any additional delays in development and if a full developmental evaluation is needed. - A formal audiology evaluation is needed for a child with significant delays in language development. An office-based hearing screen is not sufficient because of the risk for false-negative results; milder cases of clinically significant hearing impairment may not be identified. - Children whose language delay resolves are at risk for language-based learning problems (eg, reading).

A 14 yr old healthy girl has a urinalysis as part of her well child visit. Urine dipstick testing shows specific gravity 1.014, pH 6.0, and 2+ proteinuria and is negative for blood. Microscopic examination of the urine is unrevealing. The most appropriate next step in diagnosis is to:

Perform urine dipstick testing on a 1st morning-voided sample Persistent asymptomatic proteinuria - initial evaluation should include an assessment for orthostatic proteinuria, a condition in which the 24-hr urinary protein excretion rarely exceeds 1 g - begins with the collection of a 1st morning urine sample, with subsequent testing of any urinary abnormalities by a complete urinalysis and determination of a spot protein:creatinine (Pr:Cr) ratio - child must fully empty the bladder before going to bed and then collect the 1st voided urine sample immediately on arising in the morning - absence of proteinuria (dipstick negative or trace for protein and urine Pr:Cr ratio < 0.2) in the 1st morning urine sample for 3 consecutive days confirms the diagnosis of orthostatic proteinuria - no further evaluation is necessary, and the patient and family should be reassured of the benign nature of this condition - however, if there are other abnormalities of the urinalysis (e.g., hematuria) or the urine Pr:Cr ratio is > 0.2, the patient should be referred to a pediatric nephrologist for a complete evaluation

Useful in infants in whom UACs cannot be used

Peripheral arterial catheters - can often be used for several days

A 2-year-old girl presents with dark urine for the past week. Her mother denies visible blood in the urine or any dysuria. The girl had a recent upper respiratory infection and has been fatigued since the illness. On examination, she is jaundiced with scleral icterus, and she has a II/VI systolic ejection murmur over the left sternal border. Which of the following is the most likely laboratory abnormality?

Positive direct antiglobulin (Coombs) test This patient has an autoimmune hemolytic anemia following a viral illness, with signs of hemolysis and anemia on physical exam. Immune-mediated hemolysis occurs secondary to the development of antibodies against red blood cell surface antigens. Most cases are idiopathic, and the majority of patients have a preceding illness (e.g. Mycoplasma, EBV, CMV, and HIV). Other triggers include medications, immunologic disease, immunodeficiency disease, and neoplasms. Patients present with profound anemia (usually hemoglobin <6 g/dL), weakness, fatigue, jaundice, and/or dark urine (hemoglobinuria). Laboratory studies are significant for a positive Coombs test. In most pediatric cases, signs and symptoms last for 3-6 months. However, in infants and adolescents, the clinical course may be months to years. Treatment consists of blood transfusion, and in severe cases, glucocorticoids. Refractory cases may be treated with IV immunoglobulin or rituximab.

A newborn in the delivery room cries immediately after delivery but has poor tone and irregular respiratory effort. She is the product of a full-term vaginal delivery to a mother who was started on antibiotics for fever shortly after delivery. The infant is brought to the warmer where her heart rate is measured at 60 beats per minute, and she demonstrates no significant respiratory effort. What is the next best step in management?

Positive pressure ventilation - If the baby does not have adequate spontaneous respirations or a heart rate greater than 100 beats per minute, then positive pressure ventilation should be initiated within one minute following birth.

Less likely than other polydactylies to be associated with other abnormalities.

Postaxial polydactyly - A genetics referral should be considered for patients who have postaxial polydactyly without a family history and for patients with preaxial or central polydactyly.

Total daily dose (TDD) of insulin starting dose

Prepubertal child: start ~0.5 units/kg/day Pubertal child: start ~0.7 units/kg/day - TDD/2 = units basal insulin per day - Insulin to Carbohydrate Ratio (ICR): aka CARBOHYDRATE FACTOR = 500/TDD = g of CHO per 1 unit insulin

A 6 mo old male infant with persistent wheezing is diagnosed with tracheomalacia by bronchoscopy. He has no history of atopy. What is the most appropriate treatment recommendation?

Prescribe ipratropium bromide as needed during acute respiratory illness - Primary tracheomalacia and bronchomalacia are principally disorders of infants, with a male:female ratio of 2:1. - The dominant finding, low-pitched monophonic wheezing, is most prominent over the central airways. - Parents often describe persistent respiratory congestion even in the absence of a viral respiratory infection. - Postural drainage can help with clearance of secretions. - Nebulized ipratropium bromide may be useful. - Continuous positive airway pressure (CPAP) via tracheostomy may be indicated for severe cases.

A 2 mo old Bangladeshi boy, born in the United States, has been coughing since birth. The coughing episodes are particularly pronounced during feeding. His mother also mentions occasional choking and gagging episodes. His immunizations are up-to-date and there is no history of travel. Which of the following will be the most appropriate diagnostic test?

Pressure-injection barium swallow - Barium fluoroscopy is optimal for evaluating for structural anomalies, such as duplications, strictures, or external esophageal compression by an aberrant blood vessel, or for causes of dysmotility, such as achalasia. - Modifications of the routine barium fluoroscopic study are used in special situations. - When an "H- type" tracheoesophageal fistula is suspected, the test is most sensitive if the radiologist, with the patient prone, distends the esophagus with barium via a nasogastric tube. - The videofluoroscopic evaluation of swallowing performed with varying consistencies of barium ("modified barium swallow," oropharyngeal videoesophagogram, or "cookie swallow") optimally evaluates children with dysphagia by demonstrating incoordination of the pharyngeal and esophageal phases of swallowing and any associated aspiration.

Stop dz before it occurs

Primary prevention

A 5 yr old girl with a 6-mo history of excessive urination and thirst is seen for an evaluation. She has no history of trauma, recent illness, or medication use. Physical examination demonstrates a well-appearing child with normal vital signs, stable weight, and normal findings on the neurologic examination.You suspect diabetes insipidus.The most appropriate next step in the management of this patient is:

Quantification of daily fluid input and output - Cause of pathologic polyuria or polydipsia (exceeding 2 L/ m2/24 hr) may be difficult to establish in children. - Patients with suspected DI should have a careful history taken, which should quantify the child's daily fluid intake and output and establish the voiding pattern, nocturia, and primary or secondary enuresis. A - If pathologic polyuria or polydipsia is present, the following should be obtained: serum for osmolality, sodium, potassium, blood urea nitrogen, creatinine, glucose, and calcium; urine for osmolality, specific gravity, and glucose determination. - Diagnosis of DI is established if the serum osmolality is > 300 mOsm/kg and the urine osmolality is < 300 mOsm/kg. - If the patient's serum osmolality is < 300 mOsm/kg (but > 270 mOsm/kg) and pathologic polyuria and polydipsia are present, a water deprivation test is indicated to establish the diagnosis of DI and to differentiate central from nephrogenic causes.

A 4-year-old boy presents with difficulty in weight-bearing and pain in his right shin. Radiographs of the tibia and fibula are normal. You find a study of children with limp that demonstrates that radiographs have a specificity of 85% and a sensitivity of 30% for detecting osteomyelitis during the first week of illness. What is the most appropriate interpretation of the patient's negative radiographs?

Radiographs will detect 30% of patients with osteomyelitis during the first week of illness - Sensitivity measures the ability of a test to identify those with a disease of interest such as osteomyelitis. Sensitivity is calculated as the number of true positive test results divided by the sum of true positives plus false negatives. - In other words, the denominator of the equation represents all patients who truly have the disease. - The study cited in the question identifies the sensitivity of first-week radiographs in detecting osteomyelitis as 30%. This means that the radiographs will detect only 30% of patients with osteomyelitis during the first week of illness.

Which of the following is an advantage of skin testing over serum assays such as RAST to determine specific IgE?

Skin testing has greater sensitivity than RAST - primary advantages of the serum assays (as-IgE and RAST) in comparison with allergen skin testing are their safety and that the results are not influenced by skin disease or medications - in patients with histories of life-threatening reactions to foods, insect stings, drugs, or latex, skin testing is still required because of its higher sensitivity even if the as-IgE assay result is negative

A 6 yr old child is brought to your office because a tiny tick was found and removed from his forearm. The parents are unsure how long the tick had been attached, although they thought that it probably had not been there for more than 1 day. They live in an area in which Lyme disease is common. The next step in the proper treatment of this patient should be to:

Reassure the parents that the risk of infection is small and have them observe the area around the bite for the development of a rash - If a deer tick attachment is noted, the tick should be grasped at the mouthparts with a forceps or tweezers; if these are not available, the tick should be covered with a tissue. - The recommended method of tick removal is to pull directly outward without twisting; infection is usually preventable if the tick is removed before 48 hours of attachment. - The overall risk for acquiring Lyme disease after a tick bite is low (1-3%) in most endemic areas. - Patients and families can be advised to watch the area for development of erythema migrans and to seek medical attention if the rash or constitutional symptoms occur. - A study of prophylaxis after a tick bite found that a single dose of doxycycline in adults (200 mg PO) was 87% effective in preventing Lyme disease; data in children using this strategy are lacking. - Most people are not able to identify the species or the stage of the tick (in some instances reported "tick" bites prove not to be from ticks), and therefore administration of antimicrobial prophylaxis is not recommended routinely. - The routine testing of ticks that have been removed from humans for evidence of B. burgdorferi is not recommended, because the value of a positive test result for predicting infection in the human host is unknown.

Most common type of atelectasis in children

obstructive atelectasis - caused by complete or partial obstruction of large airways - typically caused by mucus plugging, particularly in children with asthma in whom it often involves the right middle lobe - other causes of obstructive atelectasis include cystic fibrosis, bronchial stenosis, bronchogenic cyst, or compression of the airway from external structures such as vascular malformations or cardiac disease

The triad of arthritis, conjunctivitis, and urethritis is referred to as

Reiter syndrome - Additional symptoms can include fever, axial spine tenderness, and corneal ulcerations. - Reactive arthritis is more common in adults but can occur in children. - It is more common in males with a 3:1 predominance. - The pathophysiology is not completely understood, but symptoms typically occur days to 6 weeks after an infection of the genitourinary, gastrointestinal, or upper respiratory system. - In the United States, Chlamydia trachomatis is the most commonly associated organism. Other known associated organisms include: Neisseria gonorrhoeae, Shigella, Salmonella, Yersinia, Campylobacter, and Streptococcus pyogenes. - Approximately 75% of individuals with reactive arthritis have HLA-B27 and are more likely to develop more severe symptoms. - Management is primarily supportive with nonsteroidal anti-inflammatory medications, such as ibuprofen, cold packs to the affected joints, and rest.

On routine office ophthalmoscopic examination of a 14 mo old girl, you are unable to see the fundus. The extraocular muscles appear intact, and the remaining physical findings are normal. A red reflex was reportedly present at birth. The most appropriate next step in management would be to:

Refer the patient to an ophthalmologist for examination performed with the child under general anesthesia - Retinoblastoma classically presents as leukocoria, a white pupillary reflex, which often is 1st noticed when a red reflex is not present at a routine newborn or well child examination or in a flash photograph of the child. - Indirect ophthalmoscopy with slit-lamp evaluation can detect retinoblastomas, but a complete evaluation requires an examination under general anesthesia by an experienced ophthalmologist to obtain complete visualization of both eyes, which also facilitates photographing and mapping of the tumors.

An 11-year-old girl presents with her mother because her mood has changed over the past year since her family moved to a new town. She no longer seems to enjoy doing things she previously liked, including playing on her soccer team or going to birthday parties. She often complains of abdominal pain in the morning before school. In the interview, she is difficult to engage, with poor eye contact. She says she is very worried about what other kids think about her. Her weight has decreased 0.5 kg in the last year. On exam, you note that she is quiet and shy. She is thin but her examination is otherwise normal. What is the best next step for this patient?

Referral to a psychologist for social anxiety disorder - anxiety disorders = most common psychiatric prob of childhood and adolescence = more common in girls

West Zone 1 is an example of what type of VQ matching?

Relative avleolar dead space - West Zone 1 is more aerated with relatively less perfusion. Hence, it is an example of relative alveolar dead space. - Zone 2 is an example of VQ matching with Zone 3 an example of relative intrapulmonary shunt, with blood flow in excess of avleolar ventilation.

Recognize respiratory papillomatosis

Respiratory papillomatosis that occurs before 18 years of age is considered juvenile-onset disease. - It is typically diagnosed between the ages of 2 and 5 years. - This condition is thought to result from vertical transmission of human papillomavirus (HPV; types 6 or 11) from the genital tract of a mother to her child. - Risk factors include being a firstborn child, vaginal delivery, and birth to a teenage mother with genital papillomatosis. - On endoscopy, the lesions have a verrucous or polypoid appearance. - Symptoms of respiratory papillomatosis include hoarseness, stridor, and abnormal cry. Severe forms can lead to airway obstruction. - The lesions are managed with surgical excision.

A 3 mo old patient hospitalized for bronchiolitis is noted on admission laboratory studies to have a serum bicarbonate of 14 mmol/L when obtained by heel stick. Of the following, which is the most appropriate next step in the patient's management?

Repeat serum electrolyte determination via venipuncture - Serum electrolytes, blood urea nitrogen, calcium, phosphorus, creatinine, and pH should be obtained by venous puncture. - Traumatic blood draws (e.g., heel stick specimens), small volumes of blood in "adult-size" specimen collection tubes, or prolonged specimen transport time at room temperature can lead to falsely low bicarbonate levels, often in association with an elevated serum potassium value.

Moderate to severe hypernatremic dehydration is best corrected by:

Repletion of intravascular volume with normal saline, followed by D5 ½ normal saline at 1.25 times maintenance rate - In the child with hypernatremic dehydration, as in any child with dehydration, the first priority is restoration of intravascular volume with isotonic fluid. - Normal saline is preferable to lactated Ringer solution because the lower sodium concentration of the latter can cause the serum sodium to decrease too rapidly, especially if multiple fluid boluses are given. - Repeated boluses of normal saline (10-20 mL/kg) may be required to treat hypotension, tachycardia, and signs of poor perfusion (peripheral pulses, capillary refill time). - Most patients with hypernatremic dehydration do well with a fluid sodium concentration of approximately half-normal saline, but with a fluid rate that is only 20-30% greater than maintenance fluid. - Use of this concentration prevents excessive delivery of free water and too rapid a decrease in the serum sodium level.

Neuromuscular blocking (NMBA) agents / paralytic

Rocuronium vs Succinylcholine Rocuronium - high dose = 1.2 mg/kg - use 1 mg/kg for both Succinylcholine - 1-2 mg/kg superior to roc at 0.6 mg/kg - avoid in kids with cardiac arrest risk, muscular dystrophies - usually fasciculate when you give; may have myalgias after

A 5-year-old boy has a complex medical history including hypoxic-ischemic encephalopathy, cerebral palsy, severe intellectual disability, gastrostomy tube dependence for feeds and medications, and epilepsy. He was admitted with hypoxemia secondary to recurrent aspiration pneumonia of the right lower lobe. His mother is concerned that his secretions have been increasingly difficult to manage and are leading to the aspiration events. His dose of glycopyrrolate was recently increased, and secretions have been thick and difficult to suction. Which of the following options is most likely to benefit the child?

Salivary botulinum toxin injection Sialorrhea - external drooling, or secretions may pool in the posterior pharynx. - First-line pharmacotherapy for sialorrhea includes cholinergic antagonists such as glycopyrrolate and scopolamine. Low doses of glycopyrrolate can be gradually uptitrated. However, uptitration may be limited by thickening of secretions, making secretions increasingly difficult to swallow or suction. Other side effects of anticholinergic agents include headaches, blurred vision, and urinary retention. - Some children benefit from salivary botulinum toxin injection, which provides a local anticholinergic effect that lasts for several months. The toxin may be injected into the submandibular or parotid glands, and injections are typically repeated at six-month intervals. Similar to oral anticholinergic agents, botulinum toxin may cause significant thickening of secretions. In addition, extravasation of the toxin into surrounding musculature may cause difficulty with chewing and swallowing. Thus, it may increase the risk of aspiration with feeds for children who are able to feed by mouth. - Because the described child is gastrostomy tube-dependent, salivary botulinum toxin is not likely to increase his aspiration risk. - An additional consideration for children with sialorrhea is surgery, which may be used to ligate, reposition, or remove the salivary glands.

A 20 mo old child develops hemolytic anemia, anuria, azotemia, and thrombocytopenia after a bout of febrile bloody diarrhea. The most likely etiologic agent of this illness is:

Shiga toxin-producing Escherichia coli - 5-10% of children with STEC (Shiga toxin-producing E. coli) hemorrhagic colitis go on within a few days to develop systemic complications such as hemolytic-uremic syndrome (HUS), characterized by acute kidney failure, thrombocytopenia, and microangiopathic hemolytic anemia.

Can cause exocrine pancreatic insufficiency (EPI). Autosomal recessive condition associated with skeletal abnormalities, neutropenia, and short stature. Can have findings of neutropenia and multiple infections, anemia, thrombocytopenia, short stature, and low fecal pancreatic elastase level (indicative of EPI).

Shwachman-Diamond syndrome

Although most lung opacities in the young child with cough and fever are viral there are features that are highly suggestive of bacterial infection...

Spherical pneumonia (now most commonly due to Streptococcus pneumoniae) Bulging Fissure Large pleural effusion, especially when loculated Lobar consolidation without volume loss Cavitation

A 12 yr old gymnast presents with back pain, reduced lumbar lordosis, and sacral kyphosis. The buttocks are flattened, and a step-off is felt in the lumbosacral region. Findings on neurologic examination are normal. The most likely diagnosis is:

Spondylolisthesis with spondylolysis - usually present with mechanical low back pain that can radiate to the buttocks and thigh but rarely below the knee - pain is exacerbated by spinal hyperextension, which exerts compression on the posterior elements - useful provocative test, with the patient standing, involves placing the spine in hyperextension and holding the position for up to 10-20 seconds. This compresses the posterior elements and might reproduce the symptoms. - In contrast, pain emanating from disk space pathology is exacerbated by spinal flexion. - Physical examination might also reveal tenderness over the spinous process of the involved vertebra. - Hamstring spasm, with or without contracture, is commonly observed. - The buttocks appear flattened (sacrum is more vertically inclined, lumbosacral kyphosis), and the abdomen might appear more protuberant (hyperlordosis above the lumbosacral kyphosis). There is often a palpable step off between the spinous processes at the involved levels, especially with a high-grade slip. - Hamstring spasm or contracture is usually more pronounced than in spondylolysis. - Gait disturbance is also observed, typically characterized by mild crouching, a short stride length, and an incomplete swing phase. - In addition to back pain, neurologic symptoms (radiculopathy or bowel or bladder dysfunction) can result from compression of the cauda equina or nerve roots.

A 2-year-old girl is brought to the emergency department with a 2-day history of fever, irritability, malaise, and emesis. Five days before presentation, she completed a 10-day course of amoxicillin for acute otitis media. On physical examination, she is ill-appearing, with a temperature of 39.4°C, heart rate of 136 beats/min, respiratory rate of 34 breaths/min, and blood pressure of 104/54 mm Hg. There is redness and swelling above the right ear, with downward and outward displacement of the pinna. Her right tympanic membrane is bulging, immobile, and opaque. The remainder of her physical examination findings are normal. Laboratory findings are notable for leukocytosis and an elevated C-reactive protein. Tympanocentesis is performed and specimens are sent for culture. Of the following, the MOST likely pathogen to be isolated in this girl is

Streptococcus pneumoniae - The child has a clinical picture suggestive of acute mastoiditis following a recent episode of acute otitis media (AOM). - Acute mastoiditis is an unusual complication of otitis media in the current antibiotic era, with an incidence of 1.2 to 4.2 cases per 100,000 children each year. - Mastoiditis can result in serious intracranial and extracranial complications. - Similar to the microbiology of AOM, the most common etiologic agent of acute mastoiditis is Streptococcus pneumoniae, even in the era of the pneumococcal conjugate vaccine (PCV). Other bacterial causes include Staphylococcus aureus (including methicillin-resistant S aureus), group A Streptococcus, nontypeable Haemophilus influenzae, Moraxella catarrhalis, and Streptococcus anginosus. - Acute mastoiditis occurs most commonly in children younger than 2 years. - The middle ear cavity is connected to the mastoid air cells via the aditus ad antrum. After AOM, there is inflammation of the middle ear and mastoid mucosa which typically resolves with antibiotic therapy. In rare instances, obstruction of the aditus ad antrum may result in accumulation of serous and then purulent fluid in the mastoid cavity, resulting in periostitis of the bony septa. Destruction of the thin trabecular system leads to coalescent mastoiditis and formation of a subperiosteal abscess. Infection can extend beyond the mastoid cavity and cause various intracranial (eg, lateral or sigmoid sinus thrombosis, meningitis, epidural, subdural, or brain abscess) and extracranial complications (eg, Bezold abscess along sternocleidomastoid muscle sheath, Bell palsy, labyrinthitis, and occipital osteomyelitis). - Treatment of mastoiditis includes systemic antibiotics and, in many cases, surgical drainage. An otolaryngology consultation should be obtained. - It is prudent to obtain a middle ear fluid specimen by tympanocentesis for culture and antimicrobial sensitivities. - Intravenous antimicrobial agents should be targeted against pathogens causing AOM, especially S pneumoniae and S aureus (including methicillin-resistant S aureus), for example, vancomycin plus ampicillin-sulbactam. In the setting of recurrent AOM and recent antibiotic use, some experts prefer a combination of vancomycin plus ceftazidime or cefepime (to cover P aeruginosa).

Child has signs and symptoms that are consistent with infective endocarditis (IE). The most likely pathogen would be

Staphylococcus aureus. - Although endocarditis is rare in children, it is associated with significant morbidity and mortality. - Most common pathogens associated with IE are streptococcus viridans group (including Streptococcus sanguis, Streptococcus mitis, Streptococcus oralis, and Streptococcus anginosus) and S aureus. - The streptococcus viridans group has been associated with infecting abnormal valves as seen in congenital heart disease and rheumatic fever. - Staphylococcus spp. have been seen in both structurally normal and abnormal valves. - The AACEK organisms (Aggregatibacter parainfluenzae [formerly Haemophilus parainfluenzae], Aggregatibacter actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens, and Kingella species) are also known to cause IE, although less frequently. - Candida and Aspergillus species can also cause IE, particularly in hospitalized patients or those with central venous catheters. - Additionally, culture-negative IE is not uncommonly seen. - The initial management of IE includes broad-spectrum bactericidal antibiotics at high serum levels (ie, vancomycin and gentamicin). - Surgical procedures are sometimes warranted and should be considered in those with heart failure symptoms that cannot be managed medically, prosthetic valves, embolic events, or positive blood cultures for more than 5 to 7 days.

A 3-year-old child with unknown muscular dystrophy is scheduled to undergo a muscle biopsy for diagnostics purposes. Anesthesia is induced using sevoflurane and you start injecting succinylcholine in preparation for intubation. You immediately notice masseter spasm and his temperature is 41°C.After immediate discontinuation of inhaled anesthesia and succinylcholine, which one of the following subsequent steps is the most appropriate?

Start 100% oxygen with high flow rate to wash out residual sevoflurane malignant hyperthermia (MH) - pts with muscular dystrophy and myotonia are at increased risk of MH when exposed to inhalational anesthetic agents like halothane, sevoflurane, isoflurane, and/or succinylcholine - muscle rigidity, fever, increased carbon dioxide production, acidosis, and tachycardia - important to recognize this potentially fatal condition immediately - immediate intervention in order of sequence includes discontinuation of offending agents, oxygenation with 100% oxygen to wash out the offending inhalational agent, intravenous injection of dantrolene, use of cold normal saline if temperature above 30°C

When does a child who has been diabetic for at least three years require an ophthalmologic exam?

Starting at age 10 years or onset of puberty, whichever is first.

Connective tissue disorder. Is associated with midfacial hypoplasia; cleft palate; Pierre Robin sequence (PRS); hearing loss; and abnormalities of the eye, including high-grade myopia, cataracts, and increased risk for vitreous abnormalities and retinal detachment.

Stickler syndrome - High risk for hearing loss in addition to eye abnormalities. - Health care providers should be mindful of the high risk of hearing loss in children with Stickler syndrome because this can have an impact on cognitive and speech development.

A 13-year-old adolescent girl is brought to the emergency department 1 hour after sustaining a laceration to her right leg during a street football game. She was running alongside parked cars when she lost her footing, tripped into a car, and sustained a laceration to her right lower leg from a broken bumper. Hemostasis was achieved with pressure at the scene, and her parents brought her directly to the emergency department. She can ambulate without assistance, although she is limping. There is a 5-cm jagged laceration slightly oozing on the lateral aspect of her right lower leg. The wound is cleaned and repaired. Prophylactic antibiotics are administered, and the parents are asked about their child's tetanus immunization status. They produce her immunization card that shows a complete primary series. The last dose of tetanus vaccine was 7 years ago. Of the following, the MOST appropriate tetanus prophylaxis for this patient is

Tdap only - In a child who has received 3 or more doses of tetanus toxoid, a clean wound requires postexposure prophylaxis with a tetanus vaccine if the last dose was more than 10 years prior, and a dirty wound requires postexposure prophylaxis if the last dose was more than 5 years prior. - If a child has not received 3 or more doses of tetanus toxoid, any wound requires postexposure prophylaxis with a tetanus vaccine, while a dirty wound also warrants tetanus immune globulin. - If a tetanus vaccine is required, the preferred vaccine is DTap for children younger than 7 years, Td for children aged 7 to 10 years, and Tdap for children aged 11 years and older.

A 4 year old previously healthy boy presented in DKA and new onset DM. He was initially managed on insulin infusion at 0.1 units/kg/hr and is now down to 0.05 units/kg/hr, requiring D10 fluids to maintain his BG in the 100s. He is hungry this morning and wants to eat breakfast. He weighs 25kg and is Tanner Stage 1. You need to come up with a plan before you consult Endocrinology...

Total Daily Dose (TDD) = 0.5 units x 25kg = 12.5 units - TDD starting point for young children = 0.5 units/kg/day 50% basal = 6.25 units glargine or detemir (daily or div BID) Insulin-to-Carbohydrate Ratio (ICR) = 500/12.5 = 40g - ICR = 500/TDD Insulin Sensitivity Factor (ISF) = 1800/12.5 = 144 mg/dL - ISF = 1800/TDD A reasonable starting plan: detemir 3 units BID aspart ICR 1:40g, ISF 140 mg/dL, BG target 150 mg/dL BG insulin dose= 1 unit:140 mg/dL > 150 mg/dL

Fluid management: parkland formula

Total body surface area burned (%) x wt (kg) x (2-4 mL) - prior to initiating Parkland formula, please contact local burn center for planning and consultation - give half of volume (NS or LR) in first 8 hours, give remainder in next 16 hours - this volume does no account for maintenance fluids (D% 1/2 NS or D5 1/4 NS) - titrate to urine output >0.5 mL/kg/hr while awaiting for transfer to burn center

A 9-month-old boy is admitted for his fourth episode of bronchiolitis. He required intubation during two prior episodes and had been referred to an immunologist with concern for the frequency and severity of his viral infections. Between episodes of bronchiolitis, he is vigorous and feeds well. He has dropped off his growth curve during admissions and then regained weight after each episode. His development is normal. During his admission, several tests return. He has low immunoglobulins A, G, and M. He has a normal number of B lymphocytes and T lymphocytes and a normal distribution of T lymphocyte subtypes. What is the most likely diagnosis?

Transient hypogammaglobulinemia of infancy - theorized to represent a prolonged nadir of immunoglobulins that typically occurs at three to six months of age. - infant must demonstrate at least low immunoglobulin G with improvement in levels over time. Immunoglobulins M and A may also be low. B cell counts and cellular immunity are normal. During the period of hypogammaglobulinemia, affected infants most commonly demonstrate upper and lower respiratory tract infections. - Recurrent infections typically decrease between 9 and 15 months of age, and immunoglobulin levels typically normalize by 4 years of age. - Intravenous immune globulin is occasionally administered to children with transient hypogammaglobulinemia of infancy if infections are particularly frequent or severe.

Autosomal dominant disorder; the classic facial dysmorphology is characterized by underdevelopment of the zygomatic bones and mandible, downslanting palpebral fissures, prominent nose, micrognathia and retrognathia, external ear abnormalities, coloboma of the lower eyelid, absence of the lower eyelashes, and anterior hair displacement onto the lateral cheekbones.

Treacher Collins syndrome - Conductive hearing loss caused by underlying hypoplasia of the middle ear cavities and malformation of the ossicles is seen in 40% to 50% of children with Treacher Collins syndrome. - Careful attention to airway management and feeding difficulties in children with Treacher Collins syndrome is important in infancy and early childhood.

only two live born trisomies apart from trisomy 21 (Down syndrome)

Trisomy 18 & Trisomy 13 - Mean survival time (MST) for trisomy 18 is 14.5 days, and MST for trisomy 13 is 7 days - Substantial number of these patients are living longer than 1 year of life - Cardiac defects typically being more severe in trisomy 13 Trisomy 18 - Clenched hand, with overlap of the 2nd and 5th fingers, over the 3rd and 4th - Intrauterine growth retardation (IUGR) - Rocker bottom feet - Micrognathia, prominent occipital, micro-ophthalmia - Low set ears - Cardiac defects, such as ventricular septal defect (VSD), atrial septal defect (ASD) and patent ductus arteriosus (PDA) - "Strawberry shaped" calvarium - Generalized muscle spasticity - Renal anomalies - Mental retardation Trisomy 13 - Major midline dysmorphic features due to a defect in the fusion of the midline prechordial mesoderm in the first three weeks of gestation. - Tends to present with more severe craniofacial and midline defects - Holoprosencephaly - Polydactyly - Seizures - Deafness - Microcephaly - Midline Cleft Lip - Midline Cleft palate - Abnormal ears - Sloping forehead - Cutis aplasia ("punched out lesions" of the scalp) - Omphalocele - Cardiac and renal anomalies - Mental retardation

A football coach calls you about a 15 yr old who just had a central incisor knocked out on the field. You tell the coach to:

Try to reimplant the tooth and take the child to the dentist - If avulsed permanent teeth are replanted within 20 min after injury, good success may be achieved; if the delay exceeds 2 hr, however, failure (root resorption, ankylosis) is common. - The likelihood that normal reattachment will follow replantation of the tooth is related to the viability of the periodontal ligament. Parents confronted with this emergency situation can be instructed to do the following: 1. Find the tooth. 2. Rinse the tooth. (Do not scrub the tooth. Do not touch the root. After plugging the sink drain, hold the tooth by the crown and rinse it under running tap water.) 3. Insert the tooth into the socket. Gently place it back into its normal position. Do not be concerned if the tooth extrudes slightly. If the parent or child is too apprehensive for replantation of the tooth, the tooth should be placed in cold cow's milk or other cold isotonic solution. 4. Go directly to the dentist. (In transit, the child should hold the tooth in its socket with a finger. The parent should buckle a seatbelt around the child and drive safely.) - After the tooth is replanted, it must be immobilized to facilitate reattachment; endodontic therapy is always required. - The initial signs of complications associated with replantation can appear as early as 1 wk after trauma or as late as several years later. - Close dental follow-up is indicated for at least 1 yr.

During a routine well-child visit, a mother mentions that her son is undergoing evaluation for dyslexia. History of which of the following would significantly increase his risk for diagnosis?

Type 1 diabetes - estimated that about 4.5% of the total student population has a learning disability. - children with chronic medical conditions such as type 1 diabetes mellitus have double the risk of learning disability when compared to children without chronic conditions. - Other chronic conditions that increase risk include chromosomal syndromes (such as Klinefelter, Turner, fragile X), neurologic disorders (including seizures, tuberous sclerosis, neurofibromatosis), and developmental and mental health diagnoses (such as ADHD, autism, depression, anxiety). - Poverty, prematurity, and family history of learning disability are also considered to be risk factors. - Additionally, children with a history of neurologic insult such as irradiation, infection, or traumatic brain injury have increased prevalence of learning disability.

A 15 yr old boy presents with frequent episodes of flushing and watery diarrhea. The laboratory test that would suggest the presence of a carcinoid tumor is assay of:

Urinary 5-hydroxyindoleacetic acid (5-HIAA) - Carcinoids are neuroendocrine tumors of enterochromaffin cells that can occur throughout the GI tract but in children they are typically found in the appendix. This is often an incidental diagnosis at the time of appendectomy. - Complete resection of small tumors (<1 cm) with clear surgical margins is curative. - Appendiceal tumors > 2 cm mandate further bowel resection. - Carcinoid tumors outside the appendix (small intestine, rectum, stomach) are more likely to metastasize. - Metastatic carcinoid tumor within the liver can give rise to the carcinoid syndrome. - Serotonin, 5-hydroxytryptophan (5-HT), or histamine are elaborated by the tumor, and elevated serum levels cause cramps, diarrhea, vasomotor disturbances (flushing), bronchoconstriction, and right-sided heart failure. - The diagnosis is confirmed by elevated urinary 5-hydroxyindoleacetic acid (5-HIAA) levels. - Symptomatic relief of carcinoid symptoms may be achieved with administration of somatostatin analogs (octreotide).

The best approach for parents to help a preschool child overcome monster fears is to:

Use "great power" like monster spray to keep monsters away - More than 80% of parents report at least 1 fear in their preschool children. - Attempts to demonstrate rationally that there are no monsters in the closet often fail, inasmuch as the fear arises from prerational thinking. However, this same thinking allows parents to be endowed with magical powers that can banish the monsters with "monster spray" or a night light. - Parents should acknowledge the fears, offer reassurance and a sense of security, and give the child some sense of control over the situation.

A 7 mo old boy manifests muscle weakness and cardiomyopathy in addition to hypoketotic hypoglycemia with fasting. An older female sibling also has periods of muscle pain and rhabdomyolysis. Both have a urinary organic acid profile with hypoketotic dicarboxylic aciduria. The most likely diagnosis is:

VLCAD deficiency - second most commonly diagnosed disorder of fatty acid oxidation - usually more severely affected than those with MCAD deficiency, presenting earlier in infancy and having more chronic problems with muscle weakness or episodes of muscle pain and rhabdomyolysis - cardiomyopathy may be present during acute attacks associated with fasting - urinary organic acid profile shows a nonketotic dicarboxylic aciduria

A 15 yr old girl who is active in her school choir is evaluated for symptoms of chronic hoarseness and strained voice, which has been present for several months. The hoarseness is worse in the evening and lessens in the morning. She has no symptoms of airway obstruction. The most likely cause of his symptoms is:

Vocal nodules (screamer's nodes) - most common cause of chronic hoarseness in children - not true neoplasms - chronic vocal abuse or misuse produces nodules at the junction of the anterior and middle thirds of the phonating edge of the vocal cords - symmetric, bilateral swellings interfere with voice production and cause children to strain the voice - can occur in infants and are exacerbated by laryngopharyngeal reflux

Should be considered in children who have recalcitrant sinusitis, pulmonary infiltrates, and evidence of nephritis...

Wegener granulomatosis (WG) - Chest radiography often fails to detect pulmonary lesions, and chest CT may show nodules, ground-glass opacities, mediastinal lymphadenopathy, and cavitary lesions. - The diagnosis is confirmed by the presence of anti-proteinase 3 (anti-PR3)-specific ANCAs (PR3-ANCAs) and the finding of necrotizing granulomatous vasculitis on pulmonary, sinus, or renal biopsy. The ANCA test result is positive in approximately 90% of children with WG, and the presence of anti-PR3 increases the specificity of the test.

On initial physical examination, a 4,500-g neonate is noted to have an umbilical hernia, macroglossia, unusual ear creases, hemihypertrophy, and visceromegaly. Soon after delivery, he develops persistent hypoglycemia warranting treatment with intravenous dextrose. Of the following, this neonate is at INCREASED risk for

Wilms Tumor Beckwith-Wiedemann syndrome (BWS) - overgrowth disorder manifested by macrosomia, macroglossia, neonatal hypoglycemia, ear creases/pits, hemihypertrophy, and visceromegaly - can also have embryonal tumors (Wilms tumor, hepatoblastoma, neuroblastoma), umbilical hernia/omphalocele, nephrocalcinosis, medullary sponge kidney disease, cardiomegaly, and nephromegaly - macrosomia, macroglossia, and hypoglycemia are typically noted in the neonatal period - hemihyperplasia can be noted in segmental regions of the body or specific organs - can present with variable expression, with some affected individuals having multiple clinical features while others have only 1 to 2 phenotypic findings - development and cognitive outcomes are typically normal

Boy in clinic has clinical and laboratory features of nephrotic syndrome (NS). A history of eyelid swelling, pitting edema of the ankles, and a 3+ protein level on spot urinalysis favor this diagnosis. To meet the criteria for idiopathic NS and to differentiate NS from acute glomerulonephritis, the next step in evaluation is to

perform renal function testing and assess serum albumin and complement level Nephrotic syndrome - proteinuria (urine protein to creatinine ratio >2 mg/mg or urine protein >40 mg/m^2/h), hypoalbuminemia (<2.5 gm/dL), edema, hyperlipidemia - classic age = 2-10 yo - starts with periorbital edema and weight gain

Only true contraindications to air or barium enema include

peritonitis perforation

Assume you weigh 160 pounds Basal/background insulin dosen =

Your total daily insulin dose (TDI) = 160 lbs ÷ 4 = 40 units. Basal/background insulin dose = 50% of TDI (40 units) = 20 units of either long acting insulin, (such as glargine or detemir) or rapid acting insulin if you are using an insulin pump (continuous subcutaneous insulin infusion device)

The differential diagnosis for a macrocytic anemia in children is limited to nutritional deficiencies (vitamin B12 and folate), bone marrow failure/myelodysplasia, certain drug exposures (eg, chemotherapeutics), or hypothyroidism. Any time a child exhibits multiple cytopenias, what should be considered?

a bone marrow evaluation

Episodic headache, sweating, tachycardia, and hypertension are common presenting features in a child with

pheochromocytoma - caused by excessive catecholamine secretion - pheochromocytoma is commonly associated with familial syndromes such as von Hippel-Lindau syndrome, multiple endocrine neoplasia type 2, and neurofibromatosis type 1

The combination of hemoptysis with multifocal densities on chest radiography, urinary findings (dark urine and borderline elevated creatinine), anemia, and thrombocytosis suggests

a systemic vasculitis such as granulomatosis with polyangiitis (previously called Wegener syndrome) - Measurement of serum antineutrophil cytoplasmic antibodies (ANCA) would be the appropriate diagnostic test - Other studies that would be helpful in establishing a systemic inflammatory process and evaluating for other causes of hemoptysis include erythrocyte sedimentation rate, C-reactive protein, antinuclear antibody, serum myeloperoxidase, and antiproteinase-3 antibody levels

A newborn infant develops nephrotic syndrome within the 1st 2 wk of life. Which of the following is the most likely cause of this patient's nephrotic syndrome?

abnormality in the nephrin gene Primary congenital nephrotic syndrome - due to a variety of syndromes inherited as autosomal recessive disorders - number of structural and functional abnormalities of the glomerular filtration barrier causing congenital nephrotic syndrome have been elucidated - affected infants most commonly present at birth with edema due to massive proteinuria, and they are typically delivered with an enlarged placenta (>25% of the infant's weight) - severe hypoalbuminemia, hyperlipidemia, and hypogammaglobulinemia result from loss of filtering selectivity at the glomerular filtration barrier

A 2 yr old boy presents with pronounced crossing of his left eye of 2 mo duration. On further examination, he is found to be excessively farsighted for his age. The most likely diagnosis is:

accommodated esotropia - "convergent deviation of the eyes associated with activation of the accommodative (focusing) reflex" - usually occurs in a child who is between 2 and 3 yr of age and who has a history of acquired intermittent or constant crossing - amblyopia occurs in the majority of cases - mechanism of accommodative esotropia involves uncorrected hyperopia, accommodation, and accommodative convergence - image entering a hyperopic (farsighted) eye is blurred. If the amount of hyperopia is not significant, the blurred image can be sharpened by accommodating (focusing of the lens of the eye) - accommodation is closely linked with convergence (eyes turning inward) - if a child's hyperopic refractive error is large or if the amount of convergence that occurs in response to each unit of accommodative effort is great, esotropia can develop - to treat accommodative esotropia, the full hyperopic (far-sighted) correction is initially prescribed - these glasses eliminate a child's need to accommodate and therefore correct the esotropia

Hypermagnesemia inhibits ...

acetylcholine release at the neuromuscular junction - produces hypotonia, hyporeflexia, and weakness - paralysis occurs at high concentrations - neuromuscular effects may be exacerbated by aminoglycoside antibiotics - direct CNS depression causes lethargy and sleepiness; infants have a poor suck - elevated magnesium values are associated with hypotension because of vascular dilation, which also causes flushing. hypotension can be profound at higher concentrations from a direct effect on cardiac functionq

A 4-year-old developmentally normal girl is brought to the emergency department because of an abnormal gait. Her mother reports that she woke that morning with the inability to walk without holding on to something. When sitting, she cannot hold herself up without bobbing her head. Her mother also reports that her speech seems slower than usual and that she has had no fever, headache, sensory changes, vomiting, rash, or weakness. The mother recalls that her daughter had a mild viral upper respiratory infection 1 week ago, from which she has completely recovered. The patient is alert and cooperative. The general physical examination findings and vital signs are normal. Neurological examination reveals scanning speech. Cranial nerve examination findings are normal, with no papilledema and mild end-gaze nystagmus bilaterally. Her strength is normal, with normal deep tendon reflexes and toes downgoing to plantar stimulation. Sensory examination findings are normal. When encouraged to sit up, she exhibits truncal instability with titubation. She has mild dysmetria when reaching for toys bilaterally. She walks holding her mother's hand with a wide-based unsteady gait. Magnetic resonance imaging of the brain with and without contrast has normal findings. Lumbar puncture is performed with normal opening pressure. Cerebrospinal fluid analysis reveals a lymphocytic pleocytosis with normal protein and glucose levels. Cultures of the cerebrospinal fluid are negative. Of the following, the MOST likely diagnosis for this child is

acute cerebellar ataxia - Acute cerebellar ataxia is a self-limited, postinfectious condition in young children that presents with acute onset of ataxia, nystagmus, and unsteady gait. - Diagnostic evaluation of acute cerebellar ataxia is aimed at exclusion of alternate diagnoses and can include toxicology screens, neuroimaging, and cerebrospinal fluid analysis. - Treatment of acute cerebellar ataxia is supportive with a favorable prognosis of recovery over weeks to months in the majority of children.

A 4-year-old developmentally normal girl is brought to the emergency department because of an abnormal gait. Her mother reports that she woke that morning with the inability to walk without holding on to something. When sitting, she cannot hold herself up without bobbing her head. Her mother also reports that her speech seems slower than usual and that she has had no fever, headache, sensory changes, vomiting, rash, or weakness. The mother recalls that her daughter had a mild viral upper respiratory infection 1 week ago, from which she has completely recovered. The patient is alert and cooperative. The general physical examination findings and vital signs are normal. Neurological examination reveals scanning speech. Cranial nerve examination findings are normal, with no papilledema and mild end-gaze nystagmus bilaterally. Her strength is normal, with normal deep tendon reflexes and toes downgoing to plantar stimulation. Sensory examination findings are normal. When encouraged to sit up, she exhibits truncal instability with titubation. She has mild dysmetria when reaching for toys bilaterally. She walks holding her mother's hand with a wide-based unsteady gait. Magnetic resonance imaging of the brain with and without contrast has normal findings. Lumbar puncture is performed with normal opening pressure. Cerebrospinal fluid analysis reveals a lymphocytic pleocytosis with normal protein and glucose levels. Cultures of the cerebrospinal fluid are negative. Of the following, the MOST likely diagnosis for this child is

acute cerebellar ataxia - Her examination findings are notable for features consistent with ataxia: slow scanning speech, intention tremor, titubation (head bobbing) while sitting, and a wide-based, unbalanced ataxic gait. - Normal magnetic resonance imaging findings with mild inflammatory changes in the cerebrospinal fluid analysis exclude a majority of these diagnostic considerations and in the setting of the described clinical presentation are supportive of a postinfectious acute cerebellar ataxia. - Postinfectious condition commonly affecting young children between the ages of 2 and 5 years. - Classically, acute cerebellar ataxia was associated with varicella infection but now a wide range of viral and bacterial causes have been associated with this condition, including Epstein-Barr virus, influenza, echoviruses, mumps, coxsackie B, and Mycoplasma. - An immune-inflammatory process leading to acute demyelination from antibodies cross-reacting to cerebellar epitopes is proposed as the likely pathology. - The ataxia is predominately truncal rather than extremity ataxia, although often a subtle intention tremor is found on finger-to-nose testing. - Treatment is supportive, aimed at safe ambulation, hydration, and, in severe cases, medical and surgical management of increased ICP in the setting of significant cerebellar swelling. - Prognosis is favorable, with over 90% of children having full recovery within a few weeks to months after initial onset.

A 3-year-old unvaccinated girl is brought to the emergency department 16 hours after she was bitten on the right lower arm by an appropriately vaccinated child at her daycare center. The family denies fever, current bleeding, or active discharge from the wound. On physical examination, her temperature is 37°C. There is a 2-cm bite wound on her right lower arm with mild penetration of the epidermal layer, minimal erythema and edema of the immediate surrounding tissue, no induration or fluctuance, no active bleeding or discharge, and mild tenderness to palpation. Of the following, the best NEXT management step for this girl is to

administer diphtheria, tetanus, and pertussis (DTaP) vaccination - The girl in the vignette who was bitten by another child is unvaccinated and therefore should receive a diphtheria, tetanus, and pertussis (DTaP) vaccination. - Because she is unvaccinated, she should concurrently receive human tetanus immune globulin (TIG) within 21 days of the injury. If human TIG is not available, intravenous immune globulin may be used. Human TIG should be administered at a different site from the tetanus toxoid. - In the case of a human bite, children who have received fewer than 3 adsorbed tetanus-toxoid containing vaccines should receive both tetanus vaccination and human tetanus immune globulin.

A 12-year-old boy presents to the emergency department with eye pain and blurry vision after being hit in his right eye with a baseball during gym class. On examination there is a limitation of right upward gaze. CT scan confirms the diagnosis of orbital floor fracture. In which age group do orbital fractures most commonly occur in children?

adolescents - low prevalence of orbital fractures in young children is due to the high cranium-to-face ratio. - cranium-to-face ratio in adults and adolescents is 2:1. - fractures in children tend to involve the upper face and skull. - children have more cancellous than cortical bone, which provides elasticity to the facial bones and makes them harder to fracture.

The boy described in the vignette has precocious puberty, exhibiting signs of puberty before age 9 years. These signs include penile enlargement, pubic hair growth, tall stature, and an advanced bone age. His testicular volume of 2 mL is prepubertal (< 4 mL), indicating a peripheral, noncentral source of his precocious puberty. His undetectable level of luteinizing hormone (LH) level confirms a noncentral source. The boy's markedly elevated dehydroepiandrosterone sulfate (DHEA-S) level makes what the most likely source of his precocious puberty?

adrenal gland - This level is consistent with an androgen-secreting adrenal tumor. - Dehydroepiandrosterone sulfate is made by the adrenal gland and is a marker of adrenal androgen production. - Adrenal androgens can be metabolized to testosterone, the source of his elevated testosterone level.

Measles is a highly communicable acute viral illness that begins with a prodromal stage characterized by fever and at least one of the "3 Cs" (ie, cough, coryza, conjunctivitis), followed by an erythematous maculopapular rash. The diagnosis of measles must be suspected in individuals with a febrile rash illness iho are unvaccinated, have recently traveled to another country, or reside in a community with low vaccine uptake. In hospital or ambulatory settings, patients with suspected measles should be immediately isolated and placed on what precautions?

airborne transmission precautions - Measles is a highly communicable acute viral illness that begins with a prodromal stage characterized by fever and at least one of the "3 Cs" (ie, cough, coryza, conjunctivitis), followed by an erythematous maculopapular rash - The diagnosis of measles must be suspected in individuals with a febrile rash illness who are unvaccinated, have recently traveled to another country, or reside in a community with low vaccine uptake - In hospital or ambulatory settings, patients with suspected measles should be immediately isolated and placed on airborne transmission precautions.

Routine immunization with quadrivalent meningococcal conjugate vaccine (MCV4) is recommended for what ages?

all adolescents 11 through 12 years of age followed by a booster dose at 16 years of age - Adolescents who receive the first dose at age 13 through 15 years should receive a booster dose at age 16 through 18 years - A booster dose is not recommended for adolescents who receive their first dose of meningococcal conjugate vaccine at or after 16 years of age

A 5-year-old girl presents to your office with a rash. Her mother reports the girl started to complain of rectal pain and itching today. Exam shows diffuse erythema of the perianal area. She has been afebrile and her physical exam is otherwise unremarkable. Which of the following is the most appropriate therapy?

amoxicillin Perianal streptococcal dermatitis - intense, well-circumscribed erythematous rash associated with rectal pain and itching - caused by group A beta-hemolytic Streptococcus and is treated with amoxicillin, oral penicillin V, or a first-generation cephalosporin - usually afebrile, but may be febrile if seen in conjunction with streptococcal pharyngitis - diagnosis can be confirmed with a bacterial culture from a perianal swab.

A 2-year-old girl with no underlying medical illness is brought to the emergency department for evaluation of a painful left forearm 12 hours after she was scratched by a pet cat. She has a temperature of 38.2°C and a puncture mark on her left forearm. There is erythema, mild swelling, and tenderness to touch in the area of the puncture mark. Of the following, the BEST choice of antimicrobial therapy for this girl is oral

amoxicillin-clavulanate - The most frequently implicated pathogen in infected cat bite infections is Pasteurella multocida; in addition to Pasteurella, a variety of mixed aerobic-anaerobic bacteria may be found in infected animal bite wounds. - The most common presentation of Pasteurella infection in children is cellulitis at the site of a bite or scratch of a cat; the onset of cellulitis is usually within 24 hours of the injury. - Treatment of choice for animal bite infections in the outpatient setting is amoxicillin-clavulanate.

Which endocrine diagnosis must be considered in a girl with an inguinal hernia?

androgen insensitivity syndrome - Up to 2% of girls who have an inguinal hernia may have complete androgen insensitivity syndrome. - Androgen insensitivity syndrome results from impaired function of the androgen receptor, leading to incomplete virilization of a 46,XY individual. - Partial androgen insensitivity syndrome is an important consideration in newborns with ambiguous genitalia, as phenotypes may include virilized female or undervirilized male. Complete androgen insensitivity was previously known as testicular feminization, and affected individuals typically have female phenotype with normal breast development. They also have a blind vaginal pouch and absent uterus and often come to medical attention and diagnosis in adolescence after presenting with primary amenorrhea. They may have less pubic and axillary hair.

Infant has respiratory distress and physical examination findings that include a gallop and hepatomegaly. These findings are suggestive of congestive heart failure (CHF) and prompted the provider to obtain a chest radiograph, which demonstrates cardiomegaly and pulmonary edema. Electrocardiography (ECG) demonstrates deep Q waves in the inferior leads (II, III, AVF). The child is in compensated cardiogenic shock with evidence of decreased perfusion to the distal extremities. This constellation of findings, particularly the ECG abnormality, is suggestive of

anomalous left coronary artery from the pulmonary artery (ALCAPA) - This anomaly requires prompt surgical repair which is the definitive therapy. Many children will have full recovery of myocardial function after repair. - When the left coronary artery arises from the pulmonary artery and not the aorta, it leads to an ALCAPA. At birth, the condition is typically asymptomatic. The natural history of pulmonary vascular resistance is that it begins to drop in the first few months after birth. As the pulmonary vascular resistance begins to drop, and consequently, the pulmonary artery pressure begins to drop, the perfusion pressure of this coronary artery also drops, resulting in myocardial ischemia and necrosis. In some children who develop collateral vessels from the right coronary artery, this condition will manifest later

A 14 yr old girl presents to your clinic with a 1-mo history of fatigue. Over the past week, she has developed low-grade fevers with temperatures to 100oF, bilateral knee pain, and chest pain with deep inspiration. On examination, she is seen to be a tired-appearing adolescent in no acute distress. Blood pressure is 130/80 mm Hg. Breath sounds are diminished over the right lung base. Cardiac examination findings are normal. Abdominal auscultation/palpation reveals no abnormalities. A urinalysis reveals 3+ hematuria and 3+ proteinuria. Which of the following laboratory studies is the most appropriate next step in confirming this patient's diagnosis?

antinuclear antibody Systemic lupus erythematosus (SLE) - fever, weight loss, dermatitis, hematologic abnormalities, arthritis, and involvement of the heart, lungs, central nervous system, and kidneys - glomerulonephritis is the most important cause of morbidity and mortality in SLE - diagnosis of SLE is confirmed by the detection of circulating antinuclear antibodies and by demonstrating antibodies that react with native double-stranded DNA - in most patients with active disease, C3 and C4 levels are depressed - in view of the lack of a clear correlation between the clinical manifestations and the severity of the renal involvement, renal biopsy should be performed in all patients with SLE --> used to guide the selection of immunosuppressive therapies

The SVC-RA junction is usually where?

at or below the right main bronchus and is anterior to the T6 vertebrae with the patient supine.

A 17-year-old girl presents with several weeks of fatigue, abdominal pain, and decreased appetite. She has lost about 4 kg during this time. She denies fever, respiratory symptoms, or rash. She does note intermittent arthralgias without joint swelling. She denies drug use or sexual activity. She has no known sick contacts. Her vital signs are normal. On exam, she is mildly ill appearing. There is subtle scleral icterus. Heart and lung exams are normal. Her abdomen is diffusely tender with a liver edge palpable 3 cm below the costal margin. Laboratory studies include aspartate aminotransferase (AST) of 740 U/L and alanine aminotransferase (ALT) of 800 U/L. Antinuclear antigen and anti-smooth muscle antibodies are strongly positive. Which of the following is the most likely diagnosis?

autoimmune hepatitis type 1 clinical presentation and her positive antibodies for antinuclear antigen and anti-smooth muscle antibodies strongly suggest autoimmune hepatitis type 1. - liver biopsy in patients with autoimmune hepatitis typically shows interface hepatitis, which refers to inflammatory changes at the portal-parenchymal interface. - treatment for autoimmune hepatitis typically involves immunosuppressants such as corticosteroids or azathioprine.

Metabolic acidosis in infant is defined as...

base deficit >5 mEq/L on first day and >4 mEq/L thereafter

A 16-year-old boy presents for a sports preparticipation physical examination before his junior year in high school. He would like to join his school's wrestling and baseball teams this year. The boy wears glasses and contact lenses for myopia. His corrected vision is 20/20 on the left and 20/70 on the right. Of the following, the MOST appropriate recommendation for this boy is that he may participate in

baseball with appropriate sports goggles, but not wrestling - The adolescent boy in the vignette has corrected vision of worse than 20/40 in 1 eye. Thus, he is considered a functionally 1-eyed athlete. - If he were to sustain an eye injury resulting in vision loss in his better-seeing eye, this could result in significant disability. - Baseball, basketball, hockey, lacrosse, and racket sports are among the activities typically considered high risk for eye injury, and the boy would require protective eyewear for participation in these sports. - Wrestling has historically been considered a sport with a low risk of eye injury. However, a recent study examining high school and college injury databases demonstrated that wrestling had the 4th highest rate of eye injury (after women's basketball, women's field hockey, and men's basketball). There is no commercially available protective eyewear for wrestling. Although custom-made eye protection may be available, the use of eye protection is typically not feasible for competitive wrestlers. Therefore, the boy in the vignette should not participate in wrestling.

Girl in clinic has moderate inflammatory and comedonal acne involving the face. There are several inflammatory papules and open comedones. No scarring is evident, but there are several resolving inflammatory lesions. These lesions may remain erythematous or violaceous for months and are often confused with scars. The most appropriate treatment, therefore, is

benzoyl peroxide (BPO)/clindamycin applied to the face each morning and retinoid (eg, tretinoin or adapalene) applied at bedtime - Benzoyl peroxide, antibiotics (topical or oral), and dapsone address the inflammatory process, topical retinoids improve follicular obstruction, and oral contraceptives lessen the impact of androgens (reducing sebum production and follicular obstruction). - It is important to note that particularly in moderate or severe acne, follicular obstruction is present and should be addressed therapeutically, even if blackheads (open comedones) and whiteheads (closed comedones) are not observed. - Extensive inflammatory acne (ie, involving the trunk, as well as the face) requires treatment with an oral antibiotic.

hyperopia, or farsightedness, is defined as a condition in which the visual images come to focus where?

beyond the retina of the eye = especially defective vision of near objects

The primary pathology in classic Potter syndrome is best characterized as:

bilateral renal agenesis - incompatible with extrauterine life and produces the Potter syndrome - death occurs shortly after birth from pulmonary hypoplasia - eyes are widely separated with epicanthic folds, the ears are low set, the nose is broad and compressed flat, the chin is receding, and there are limb anomalies - should be suspected when maternal ultrasonography demonstrates oligohydramnios, nonvisualization of the bladder, and absent kidneys - incidence of this disorder is 1/3,000 births, with a male predominance, and represents 20% of newborns with the Potter phenotype

What is the disease for which liver transplantation is most commonly indicated?

biliary atresia - most common indication for liver transplantation in children, followed by metabolic and inborn disorders, autoimmune and familial cholestatic disorders, and acute hepatic necrosis - children with biliary atresia (or any other obstructive biliary disorder) who do not achieve successful drainage require liver transplantation within the 1st year of life, yet some patients with successful drainage later develop cirrhosis with portal hypertension (variceal bleeding and ascites)

Preterm infants generally can be vaccinated at the same chronologic age as full-term infants according to the recommended childhood immunization schedule. An exception is

birth dose of HepB vaccine - infants weighing ≥ 2 kg and who are stable may receive a birth dose - HepB vaccination should be deferred in infants weighing < 2 kg at birth until 30 days of age, if born to an HBsAg-negative mother - All preterm, low birthweight infants born to HBsAg-positive mothers should receive HepB IG and HepB vaccine within 12 hr of birth. However, such infants should receive an additional 3 doses of vaccine starting at 30 days of age.

Small gas-containing space within visceral pleura or subpleural lung smaller than 1 cm

bleb - 1 cm or larger = bulla

Type of feeding preferred for MBM

bolus feeding

Infant with corneal clouding, photophobia, and chronic or intermittent tearing is demonstrating the classic presentation of

congenital or early-onset glaucoma - Every infant should be evaluated for a red reflex in a darkened room with an ophthalmoscope. - First, each eye should be examined individually from a distance of 1 to 2 feet, followed by Brückner reflex testing, during which both eyes are examined simultaneously from a distance of 2 to 3 feet. - Immediate referral to an ophthalmologist should occur for findings of asymmetric, absent, dulled, or opaque red reflex; dark spots in the red reflex; or leukocoria (white reflex). - After the age of 3 months, poor tracking, lack of fixation, nystagmus, or squinting should warrant a referral.

A 15-month-old boy is brought to the urgent care center after an episode during which he stopped breathing, turned blue, became limp, and stiffened. The episode lasted for about 1 minute, after which, he was very sleepy. The boy had been playing in a park when he fell, scraped his knee, and immediately cried. He then became apneic, cyanotic, and limp; lost consciousness; and developed stiffness of his upper and lower extremities. He has had no recent illness. There is no known family history of syncope, sudden death, or epilepsy. Of the following, based on this boy's history, the MOST likely diagnosis is

breath-holding spell - common events in children from 6 months to 4 years of age - 90% have their first episode before 18 months of age. - slightly more common in girls - some data support a primary role for dysfunction of the autonomic nervous system or delayed maturation in myelination of the brainstem - family history of BHS is present in approximately 50% of patients, and autosomal dominant inheritance has been reported in some families - iron-deficiency anemia is more prevalent in children with BHS compared with controls and may contribute to the occurrence of BHS and underlying dysautonomia - prognosis for children with BHS is excellent, with remission occuring at a mean age of 4 years; virtually all children with BHS stop having episodes by 8 years of age - because iron deficiency is highly associated with BHS, evaluation with complete blood cell count and serum ferritin is warranted

The finding of parallel lines ("tram track") radiating from the hilum is the hallmark of ...

bronchiectasis on chest radiograph

bumex

bumetanide = loop diuretic with rapid onset and short duration of action

11 yr old boy had been fighting and wrestling with another boy at school and sustained several bruises on the face and neck. The next morning he woke up with inability to speak clearly and weakness on the right side of his body, greater in the arm than in the leg. The most likely cause for his neurologic disorder is:

carotid artery intimal dissection

A 6-year-old boy with a history of penicillin allergy presents to clinic with a sore throat. Parents report that he developed a sore throat and fever about 48 hours ago. He also vomited once and is complaining of a headache. He has no nasal congestion or rhinorrhea. Physical exam reveals a febrile boy with an erythematous oropharynx, strawberry tongue, and palatal petechiae. The rest of his exam is within normal limits. After confirming the diagnosis, which of the following would be the most appropriate treatment?

cephalexin streptococcal pharyngitis - medication of choice is penicillin (or amoxicillin in children due to its preferred flavor), however, this boy has a history of penicillin allergy. - First-line treatment in patients who are penicillin allergic and do not have history of an IgE-mediated reaction is a first-generation cephalosporin, such as cephalexin. - In patients who have a history of an IgE-mediated penicillin reaction, it is recommended to treat with second- or third-generation cephalosporins. - Macrolides, such as azithromycin, are indicated for patients who have a history of anaphylaxis to penicillins or cannot tolerate cephalosporins.

Exposure to pesticides that contain organophosphates can cause symptoms of

cholinergic excess - including bradycardia, miosis, lacrimation, salivation, bronchospasm and bronchorrhea, urination, emesis, and diarrhea - organophosphates are well absorbed through the skin, gut, and respiratory tract - children can be exposed to organophosphate insecticides through accidental ingestion, inhalation of insecticide sprays, ingestion of contaminated food, or transmission on clothing or fabric.

A 9 yr old boy undergoes stem cell transplantation for acute myelogenous leukemia in remission. Approximately 4 mo after transplantation, he has persistent maculopapular rash over 25% of his body, generalized erythroderma, bilirubin 8.4 mg/dL, and diarrhea with > 1,000 mL of feces per day. He complains of xerostomia. The most likely diagnosis is:

chronic graft versus host disease - develops or persists > 3 mo post-transplant and is the most frequent late complication of allogeneic HSCT, with an incidence of ≈25% in pediatric patients - disorder of immune regulation characterized by autoantibody production, increased collagen deposition and fibrosis, and clinical symptoms similar to those seen in patients with autoimmune diseases - ongoing immune reactivity results in clinical features resembling a systemic autoimmune disease with lichenoid and sclerodermatous skin lesions, malar rash, sicca syndrome, arthritis, joint contractures, obliterative bronchiolitis, and bile duct degeneration with cholestasis - chronic GVHD involving only the skin and liver have a favorable course - extensive multiorgan disease may be associated with a very poor quality of life and a high mortality rate

A 5-year-old boy is seen for evaluation of a rash. He has a history of atopic dermatitis that is typically well controlled. He reports falling on the playground and skinning his knee 4 days ago. This morning, he awoke to find redness, swelling, and tenderness surrounding the abrasion. The boy's mother is a physician, and his younger sister has been treated for several skin abscesses in the past. The boy appears well and has age-appropriate vital signs with no fever. He has a 2×1-cm shallow abrasion overlying his left patella with approximately 3 cm of surrounding erythema and edema. The area is tender, with no focal fluctuance. He has full range of motion of his knee. He has several enlarged, nontender lymph nodes at the left inguinal canal. The remainder of his physical examination findings are normal. Of the following, the MOST appropriate treatment is

clindamycin - For hemodynamically stable children with cellulitis and risk factors for methicillin-resistant Staphylococcus aureus, initial antibiotic choice should include coverage for methicillin-resistant Staphylococcus aureus and β-hemolytic Streptococcus. Clindamycin is the preferred first-line agent. - For hemodynamically stable children with cellulitis and no risk factors for methicillin-resistant Staphylococcus aureus, coverage should include methicillin-sensitive Staphylococcus aureus and β-hemolytic Streptococcus. Cephalexin, cefadroxil, and cefuroxime are options for this clinical situation. - Risk factors for methicillin-resistant Staphylococcus aureus infection include skin trauma, frequent exposure to antibiotics, chronic disease, recent surgery or hospitalization, sharing of potentially contaminated items such as razors or towels, history of abscesses or other skin infections in patient or household contact, and history of methicillin-resistant Staphylococcus aureus infection in patient or household contact.

A 13-year-old adolescent girl is seen for a routine health supervision visit. She was diagnosed with Crohn disease at 10 years of age. At 12 years of age she underwent resection of an ileal stricture and had 25 cm of terminal ileum removed. Reanastomosis has since been performed, and she has done very well. She has 1 or 2 formed stools daily, reports no abdominal pain, and has been growing well. Recent endoscopic evaluation (upper endoscopy and colonoscopy) demonstrated no active Crohn disease. She receives adalimumab injections every 2 weeks, and she takes a multivitamin with additional iron and vitamin D supplements daily. She eats a regular diet. Of the following, the dietary component MOST likely to be deficient in this patient is

cobalamin - The adolescent in this vignette has Crohn disease, a subtype of inflammatory bowel disease (IBD) and has had resection of her terminal ileum, which increases her risk for cobalamin (vitamin B12) deficiency. - Cobalamin is found naturally in animal products. Once ingested and in the stomach, vitamin B12 binds to intrinsic factor. The uptake of vitamin B12 bound to intrinsic factor occurs through a specific receptor found only in the terminal ileum. Therefore, diseases affecting the stomach or terminal ileum can result in cobalamin deficiency.

A 2-year-old boy's parents are concerned about significant speech delay. He exhibits unusual dysmorphology, including malar and zygomatic hypoplasia with downslanting palpebral fissures; prominent nose; significant micrognathia and retrognathia; small, posteriorly rotated, and malformed ears; and lower eyelid colobomas with absent eyelashes. His father has similar dysmorphology. Of the following, the MOST likely reason for this boy's speech delay is

conductive hearing loss Treacher Collins syndrome - 40% to 50% will have conductive hearing loss because of underlying hypoplasia of the middle ear cavities and malformation of the ossicles - less commonly, children with TCS will have a cleft palate or choanal stenosis/atresia which could also affect hearing. - typically an autosomal dominant disorder caused by gene mutations in TCOF1 (78%-93%) and POLR1C or POLR1D (8%) - classic findings of TCS include craniofacial dysmorphology (including zygomatic and mandibular enderdevelopment, downslanting palpebral fissures, prominent nose, and micrognathia), airway abnormalities (including unilateral or bilateral choanal atresia and Pierre-Robin sequence), ophthalmologic abnormalities (including lower eyelid coloboma and vision loss), dental anomalies, and ear anomalies - intelligence is usually normal

A study is conducted in a tertiary care center to examine the relationship between morbidity in children with cystic fibrosis and annual household income. Results showed that children of families with incomes below the poverty line were 5 times as likely to have cystic fibrosis-related morbidities compared with children of families whose incomes were above the poverty line. An attempt was then made to increase access to health care for children living in households with incomes below the poverty line. A social worker was assigned to each family and provided gas vouchers and mileage and taxi reimbursement to the parents of patients whose incomes were below the poverty line. Of the following, the type of bias that MOST accurately describes what was corrected in the study is

confounding bias - occurs when extraneous factors or variables are present in the study that can distort the association between exposure and outcome. - the confounding bias in the study described in the vignette was access to health care, which is shown to be more limited in children living in households with incomes below the poverty line versus above it - decreased health care access leads to low compliance for acute-care and routine-care visits in patients with chronic medical conditions such as cystic fibrosis. Therefore, the bias has the potential to affect the results of the study conducted by the tertiary care center by influencing morbidity. - confounding can be addressed in a study design by using techniques such as restriction, matching, and randomization - based on the results of the study described in the vignette, the confounding bias was identified and then addressed by providing participants with resources to help mitigate the cost of transportation to the care center and thereby increasing access to medical care

For U.S. children younger than age 1, what are the leading cause of mortality?

congenital malformations and disorders - For all other pediatric age groups, accidents lead all causes of death.

A 6-year-old boy presents to the emergency department after a motor vehicle collision. The patient was a restrained passenger when his car was rear-ended by another vehicle traveling at a high speed. The patient is complaining of neck pain and numbness in both hands, which is improving. Otherwise, his physical examination is normal. A cervical collar is placed, and subsequent plain radiographs and CT scan of the brain and cervical spine are normal. Which of the following is the most appropriate next step in management?

consult to spine surgery presenting after a motor vehicle collision with symptoms concerning for spinal cord injury without radiographic abnormality (SCIWORA) --> requires consultation to spine surgery. - SCIWORA is used to describe patients with neurologic findings suggestive of spinal cord injury without anatomic misalignment or bony abnormalities seen on plain radiographs or CT scans. - SCIWORA more commonly occurs in the pediatric population, especially in patients under 8 years of age. - Pediatric patients have spinal ligaments and capsules that are more elastic than adults. Their head size to body ratio is also larger, and the diameter of the spinal canal relative to the diameter of the spinal cord is larger, which results in increased mobility of the spinal cord within the canal. P - Patients may also present with transient neurologic symptoms, which may be resolved by the time they present to the emergency department. - A portion of patients may also have a delayed onset of symptoms up to four days after injury. - Any injury with a neurologic deficit requires emergency consultation with spine surgery to guide supportive care and definitive management of the injury. - If available, an MRI should also be obtained as it is the best imaging modality to assess for spinal cord injury.

The severe acute respiratory syndrome (SARS) outbreak in the early 2000s was caused by what virus?

coronavirus - They cause up to 15% of common colds and are implicated as causes of croup, asthma exacerbations, and lower respiratory tract infections, including bronchiolitis and pneumonia. - In addition, there is evidence that coronaviruses may be causes of enteritis or colitis in neonates and infants and may be underappreciated as agents of meningitis or encephalitis. - The discovery that severe acute respiratory syndrome (SARS) is caused by a novel human coronavirus (SARS-CoV) has led to increased surveillance and the recognition of additional human coronaviruses, revealing that new coronaviruses enter human populations from zoonotic vectors such as bats.

A 3-week-old boy presents with his first febrile urinary tract infection and is started on intravenous antibiotics. He is noted to have elevated serum creatinine and a potassium of 5.6 mg/dL. He is found to have hydroureter on renal ultrasound and posterior urethral valves on a follow-up voiding cystourethrogram. What is the next best step in management?

correction of serum potassium neonate presents with his first febrile urinary tract infection, hydroureter, elevated serum creatinine, and elevated serum potassium. - all of these findings are likely secondary to his posterior urethral valves, which result in distal urinary obstruction. - amongst children with posterior urethral valves, both vesicoureteral reflux and chronic kidney disease are common. Chronic kidney disease is thought to result from both renal dysplasia and injury from chronic urinary obstruction. - Chronic kidney disease may result in a decreased glomerular filtration rate, as manifest by this infant's elevated creatinine. - It may also result in other electrolyte derangements, such as hyperkalemia. - The first step in management of infants with posterior urethral valves is correction of serum electrolytes. This should occur prior to any operative intervention. Once electrolyte derangements have been corrected, the neonate can undergo ablation of the posterior urethral valves.

A male newborn infant is delivered by emergency cesarean section for severe fetal distress and placental abruption. Resuscitation is required in the delivery room. The infant develops gross hematuria and oliguric acute renal failure with a peak serum creatinine level of 8.0 mg/dL at 8 days of age. The most likely cause of the infant's acute renal failure is:

cortical necrosis - In newborns, cortical necrosis is most commonly associated with hypoxic or ischemic insults caused by perinatal asphyxia, placental abruption, and twin-twin or fetal-maternal transfusion - Urine output is diminished and gross and/or microscopic hematuria may be present - Hypertension is common, and thrombocytopenia may be present as a result of renal microvascular injury - Laboratory results are consistent with acute renal failure: an elevated blood urea nitrogen (BUN) and creatinine, hyperkalemia, and metabolic acidosis. - Anemia and thrombocytopenia are common. - Urinalysis reveals hematuria and proteinuria.

Types of CGM (continuous glucose monitors)

dexcom libre - give pts a constant BG reading - check the BG of interstitial fluid every 5 minutes (= 5 minutes behind blood glucose number) - pts change CGM every 10 days

An 8 yr old girl presents with high fever of 5 days' duration, cool extremities, a rapid and weak pulse, and a petechial rash. Pulse pressure is less than 20 mm Hg, and a small effusion is detected in the right pleural space. The child lived in Thailand for 2 yr prior to immigrating to the United States and has just returned from Thailand after visiting to see her grandparents. The most likely diagnosis is:

dengue hemorrhagic fever - now endemic in tropical Asia, the South Pacific Islands, northern Australia, tropical Africa, the Caribbean, and Central and South America - occurs frequently among travelers to these areas - pathogenesis of dengue hemorrhagic fever is incompletely understood, but epidemiologic studies suggest that it is usually associated with 2nd infections with dengue types 1-4 The World Health Organization criteria for dengue hemorrhagic fever are - fever (2-7 days in duration or biphasic) - minor or major hemorrhagic manifestations - thrombocytopenia (≤100,000/mm3) - objective evidence of increased capillary permeability (hematocrit increased by ≥20%) - pleural effusion or ascites (by chest radiography or ultrasonography) - hypoalbuminemia Dengue shock syndrome criteria include those for dengue hemorrhagic fever as well as hypotension, tachycardia, narrow pulse pressure (≤20 mm Hg), and signs of poor perfusion (cold extremities).

A preschool-aged child's response to the death of a parent often is characterized by:

denial and magical wishing - Preschool children are in the preoperational cognitive stage, in which communication takes place through play and fantasy. - They do not show well-established cause-and-effect reasoning. - They believe that death is reversible, analogous to someone going away. - In attempts to master the finality and permanence of death, preschoolers frequently ask unrelenting, repeated questions about when the person who died will be returning. - Preschool children typically express magical explanations of death events, sometimes resulting in guilt and self-blame ("He died because I wouldn't play with him." "She died because I was mad at her.").

An 8 yr old with nighttime enuresis is invited to a sleepover next weekend. What treatment is most likely to keep him dry for the party?

desmopressin acetate - fast action - role for special occasions (e.g., sleepovers), when rapid control of bedwetting is desired - relapse rate is high when desmopressin is discontinued - associated with rare side effects of hyponatremia and water intoxication, with resulting seizures

A 16 yr old boy is suspected of having legionellosis. Which of the following is the most useful test for prompt diagnosis of Legionella pneumonia?

detection of antigen in urine - The urinary antigen assay that detects L. pneumophila serogroup I has revolutionized the diagnosis of Legionella infection and has 80% sensitivity and 99% specificity.

The preferred agent for treatment of Lyme disease in a 13 yr old child is:

doxycycline - Young children are generally treated with amoxicillin. - Doxycycline has the advantages of good CNS penetration and is active against Anaplasma phagocytophilum, which may be transmitted at the same time as B. burgdorferi in certain geographic areas. - In general, children < 8 yr of age should not be treated with doxycycline because of the risk of permanent staining of the teeth (although courses of ≤ 2 wk are usually safe in this regard).

A 3-month-old male was recently diagnosed with a metabolic disorder, following a workup for failure to thrive associated with difficulty feeding and episodes of respiratory distress. He is alert and smiling during your exam but is diffusely hypotonic, and his liver margin is palpable 5 centimeters below the costal margin. You note macroglossia on exam as well. A chest X-ray obtained revealed cardiomegaly. An electrocardiogram reveals shortened PR interval and giant QRS complexes. Which is the following is shown to be the mainstay of treatment for the most likely diagnosis?

enzyme replacement therapy infantile form of lysosomal acid alpha-glucosidase deficiency, also known as Pompe disease - glycogen storage disease type II. - 1 in 138,000 in the infantile form, and 1 in 57,000 for the juvenile and adult forms. - occurs most frequently in African American, Chinese, and Taiwanese populations, and the juvenile form occurs more frequently in the Netherlands. I - autosomal recessive manner and is due to mutations on chromosome 17 - deficiency in acid alpha-1,4-glucosidase, or lysosomal acid maltase, which is responsible for breaking down glycogen in lysosomal vacuoles. - excess storage of glycogen in the lysosomes of body tissues. T - issues relating to concentric hypertrophic cardiomyopathy, as well as skeletal muscle weakness. - start within the first few months of life, presenting as hypotonia, feeding difficulties, failure to thrive, and respiratory distress due to cardiac insufficiency. - Macroglossia may be present. Hepatomegaly may also be present, secondary to congestive heart failure. Electrocardiogram changes relating to the biventricular hypertrophy reveal a high-voltage QRS complex and a shortened PR interval. - Without treatment, those affected die within one to two years of life due to cardiorespiratory failure. - elevated creatinine kinase, aminotransferases, and lactate dehydrogenase. - Gene sequencing is used to confirm diagnosis. Enzyme replacement therapy with recombinant human alglucosidase alfa is available for treatment, given as 20 mg/kg intravenously every two weeks. - Enzyme replacement therapy has been shown to reduce risk of death by 95% and reduce need for mechanical ventilation by 90% over a three-year study period. - A high-protein diet may be used in the juvenile and adult form. Ventilatory support at nighttime may help to support respiratory function.

A 14-year-old adolescent boy is brought to the emergency department for evaluation of difficult and painful swallowing. His symptoms began 5 hours ago after he ate ramen noodles and chicken sausage. He noticed a feeling that something "was stuck" in his chest; he has been retching and vomiting since then. He has a 2-year history of dysphagia but has never experienced symptoms to this degree. He has a history of multiple environmental allergies, for which he receives immunotherapy, and a history of anaphylaxis to eggs. On physical examination, the boy is uncomfortable appearing and frequently retching and spitting clear liquid into the emesis basin near his bed. His examination findings are otherwise normal. Chest radiograph is normal. Of the following, the BEST next management step for this adolescent is

endoscopy - Esophageal obstruction presents clinically with chest pain, dysphagia, and the inability to swallow fluids or oral secretions. - Emergent consultation with a pediatric gastroenterologist and a therapeutic endoscopy are indicated for esophageal obstruction. - Older children with esophageal food impaction are likely to have eosinophilic esophagitis.

A 9 yr old boy develops local pain and swelling of the left lateral aspect of the skull. Physical examination reveals an afebrile child with marked tenderness and warmth over the affected area. Plain film shows a well-defined radiolucent skull lesion. The skeletal survey is otherwise normal. The most likely diagnosis is:

eosinophilic granuloma - monostotic or polyostotic disease with no extraskeletal involvement. this latter finding distinguishes eosinophilic granuloma from the other forms of Langerhans cell histiocytosis (Hand-Schüller-Christian or Letterer-Siwe variants), which can have a less favorable prognosis - usually occurs during the 1st 3 decades of life and is most common in boys 5-10 yr of age - skull is most commonly affected, but any bone may be involved. - usually present with local pain and swelling - marked tenderness and warmth often are present in the area of the involved bone - spinal lesions can cause pain, stiffness, and occasional neurologic symptoms - radiographic appearance of the skeletal lesions is similar in all forms of Langerhans cell histiocytosis but is variable enough to mimic many other benign and malignant lesions of bone - radiolucent lesions have well defined or irregular margins with expansion of the involved bone and periosteal new bone formation - skeletal survey is warranted because polyostotic involvement and the typical skull lesions strongly suggest the diagnosis of eosinophilic granuloma

A 6-year-old boy presents with the acute onset of fever, respiratory distress, and drooling that began this evening. He is otherwise healthy and had no preceding symptoms. On examination, he is leaning forward on his hands and holding his neck in extension. He appears toxic, is in severe respiratory distress, and demonstrates inspiratory stridor. What is the most likely diagnosis?

epiglottitis - most commonly affected children are now ages 6 to 12 years. E - upper airway obstruction manifests as stridor. In addition, the inflammation may preclude swallowing of secretions, resulting in drooling. - may assume the tripod and sniffing positions to maximize airway patency. T - rapid recognition and treatment of epiglottitis is critical, as inflammation may rapidly progress to obstruct the entire airway. - initial treatment of epiglottitis includes broad-spectrum antibiotics and airway management. - Endotracheal intubation is recommended for patients with more than 50% occlusion of the laryngeal airway.

A 10-month-old infant boy is brought to the emergency department. Your initial assessment reveals a lethargic, pale infant with slow respirations and slow, weak central pulses. One team member begins ventilation with a BVM with 100% O2. A second team member attaches the monitor/defibrillator and obtains vital signs while a third team member attempts to establish IV/IO access. The patient's HR is 38/min with the rhythm (bradycardia) on the monitor. The infant's BP is 58/38 mmHg, and capillary refill is 4 seconds. His central pulses remain weak, and distal pulses cannot be palpated. Chest compressions are started and IO access is obtained. Which medication do you anticipate will be given next?

epinephrine 0.01 mg/kg IV/IO

Most common form of esophageal atresia

esophageal atresia with distal TE fistula

Hymenectomy is facilitated by tissue that has been exposed to stimulation by which hormone?

estrogen

A 35-week-gestation female neonate is delivered at home precipitously. Her mother had routine prenatal care. The father drives the mother and neonate to the nearest hospital, with the mother holding the neonate in a wet receiving blanket. On arrival at the emergency department, the neonate has a temperature of 35.6°C, heart rate of 92 beats/min, respiratory rate of 15 breaths/min, blood pressure of 55/37 mm Hg, and oxygen saturation on pulse oximetry of 87% in room air. Her extremities are cool. Of the following, the MOST likely mechanism for the neonate's heat loss is

evaporative - The neonate in the vignette is losing heat by evaporation. - The wet blanket wrapped around the neonate allows continuous evaporative heat loss. - For term neonates, immediate skin-to-skin care is an effective means of preventing hypothermia. - Neonates should be placed prone on their mother's abdomen with a dry blanket over them to prevent evaporative and convective heat loss.

A previously healthy 10 mo old female infant is found unresponsive in her crib. In the emergency department, she is noted to be well developed and well nourished with normal blood pressure and appearance of the genitalia but with increased pigmentation of her skin. Blood glucose level is 30 mg/dL. The most likely diagnosis is:

familial glucocorticoid deficiency - form of chronic adrenal insufficiency characterized by isolated deficiency of glucocorticoids, elevated levels of ACTH, and generally normal aldosterone production, although salt-losing manifestations as are present in most other forms of adrenal insufficiency occasionally occur - mainly have hypoglycemia, seizures, and increased pigmentation during the 1st decade of life - disorder affects both sexes equally and is inherited in an autosomal recessive manner - marked adrenocortical atrophy with relative sparing of the zona glomerulosa

Patients with Turner syndrome should undergo careful analysis of their chromosomes for Y chromosome material because they may:

experience gonadoblastoma - Patients with 45,X/46,XY mosaicism can have Turner syndrome. This variant is estimated to represent approximately 6% of patients with mosaic Turner syndrome. - Some of the patients with Turner syndrome phenotype and a Y cell line exhibit masculinization. - Phenotypic females with 45,X/46,XY mosaicism have a 15-30% risk of developing gonadoblastoma. - The risk for the patients with a male phenotype and external testes is not so high, but tumor surveillance is nevertheless recommended. - The AAP has recommended the use of FISH analysis to look for Y-chromosome mosaicism in all 45,X patients. If Y chromosome material is identified, laparoscopic gonadectomy is recommended.

What during embryology leads to the constellation of defects observed in pts with meningomyelocele (MMC)?

failure of closure of neural tube during third week of gestation

A 10 yr old boy is found to have an elevated low-density lipoprotein cholesterol (LDL-C) level. His sister has an elevated triglyceride level. The father has elevated LDL-C and triglyceride levels. The paternal grandmother has a high cholesterol level and had a heart attack at the age of 48 yr. (Note: In this case, elevated levels refer to levels greater than 95th percentile.) The most likely diagnosis for this child is:

familial combined hyperlipidemia - autosomal dominant condition characterized by moderate elevation in plasma LDL cholesterol and triglycerides and reduced plasma HDL cholesterol - most common primary lipid disorder, occurring in approximately 1/200 people - damily history of premature heart disease is typically positive Formal diagnosis requires that at least 2 first-degree relatives have evidence of one of three variants of dyslipidemia: (1) >90th percentile plasma LDL cholesterol; (2) >90th percentile LDL cholesterol and triglycerides (3) >90th percentile triglycerides

Any 35+ baby born to a mom with blood type O or Rh negative status will ...

have their blood type and Coombs status checked. - If the baby is Coombs positive, they will get a 6 hour bili, CBC and retic. Checking these levels is very important on call - there is less guidance on treatment levels for infants younger than 35 weeks

Infant has hyponatremic dehydration because of an excessive loss of sodium from gastroenteritis and attempted rehydration with diluted formula. The result is low serum osmolality, which in turn leads to

fluids shifting from the extracellular fluid (ECF) compartment to the intracellular fluid (ICF) compartment - Treatment of hyponatremia should be gradual, and the target rise in sodium should not be more than 0.5 mmol/L/h or 10 to 15 mmol/L in 24 hours. - A rapid correction of hyponatremia leads to osmotic shift of water from the brain cells into the ECF causing central pontine myelinolysis. - Rapid onset of hypernatremia, rather than hyponatremia, may lead to subarachnoid hemorrhage.

Recommended screening in children and adolescents with a BMI at the 85th percentile or higher includes

glycemic indicators (fasting glucose, hemoglobin A1c, or oral glucose tolerance test) fasting lipids liver transaminases as well as - polycystic ovary syndrome (in females) - obstructive sleep apnea - psychiatric comorbidities

Boy has Prader-Willi syndrome (PWS). When the child is aged 4 to 6 years, hypothalamic hyperphagia occurs, primarily owing to decreased satiety, which can lead to significant obesity if food intake is not strictly controlled. Facial features include almond-shaped eyes, a thin upper lip, and small hands and feet. Hypogonadism is a prominent feature in both males and females. In male infants, the most common manifestation is cryptorchidism, which occurs in nearly all males with PWS. Endocrine abnormalities result from hypothalamic-pituitary dysfunction and include

growth hormone deficiency - considered universal - and variable central adrenal insufficiency and central hypothyroidism

A 3-month-old infant is being evaluated for a hoarse cry of 2 weeks' duration. His mother is concerned about noisy breathing and progressive difficulty feeding. On physical examination, his vital signs are normal and he has an oxygen saturation of 98% on pulse oximetry. He has mild inspiratory stridor with increased work of breathing and intercostal retractions. His breath sounds are clear. Cardiovascular examination findings are normal. No hepatosplenomegaly is noted. Of the following, the finding that would be MOST helpful in making a diagnosis is

hemangioma of the scalp and lower lip - A head or facial hemangioma in the presence of respiratory symptoms suggests the possibility of a hemangioma in the airway. - Vascular rings and slings may present with wheezing or cough and dysphagia. - Magnetic resonance imaging is the modality of choice for the evaluation of central vascular anomalies.

Where to look for fluid in FAST exam...

hepato-renal spleno-renal pelvic pericardial - >200 ml fluid - 4 views = RUQ, LUQ, pelvic, subxiphoid

A 15 yr old girl wishes to play soccer in school and needs medical clearance. On physical examination, her liver edge is palpable and seems minimally enlarged. Ultrasonography of the liver shows one cyst approximately 3 cm in diameter. Her menstruation is normal. Results on serologic testing for Echinococcus and hepatitis are negative, and liver enzyme values are normal. She has lived her whole life in Salt Lake City, Utah, and has never traveled abroad. The most important factor in allowing her to play this sport is:

her travel history - Echinococcosis (hydatid disease or hydatidosis) is the most widespread, serious human cestode infection in the world. - In areas endemic for E. granulosus, the parasite is often acquired in childhood, but liver cysts require many years to become large enough to detect or cause symptoms. - In the liver, many cysts never become symptomatic and regress spontaneously or produce relatively nonspecific symptoms. Symptomatic cysts can cause increased abdominal girth, hepatomegaly, a palpable mass, vomiting, or abdominal pain. However, the more serious complications result from compression of adjacent structures, spillage of cyst contents, and location of cysts in sensitive areas, such as the reproductive tract, brain, and bone. - Anaphylaxis can occur with cyst rupture or spontaneous spillage, due to trauma or intraoperatively. Spillage can also be catastrophic long term, because each protoscolex can form a new cyst. - Serologic studies may be useful in confirming a diagnosis of cystic echinococcosis, but the false-negative rate may be > 50%.

One of the first labs to check on with sepsis...

lactate - anaerobic metabolism blood gas - metabolic acidosis +/- lactate

Typically, the treatment of hypertension begins with a nonpharmacologic approach, including diet and lifestyle modifications. Pediatric data mirroring those in adults demonstrate that diet and exercise have a favorable effect on blood pressure. The Dietary Approaches to Stop Hypertension (DASH) diet, in particular, is recommended, which includes a diet...

high in fruits, vegetables, low-fat milk products, whole grains, fish, poultry, nuts, and lean red meats - sugar and sweets are limited

A 5-day-old male neonate is seen for his first health supervision visit. He was delivered at 39 weeks of gestation by a 28-year-old primiparous woman with an uncomplicated pregnancy. His postnatal course was complicated by breastfeeding difficulties and excessive weight loss. He did not pass the newborn hearing screen in either ear. The neonate's weight is 7% below his birth weight. His vital signs are normal for age. He has a flat midface, anteverted nares, micrognathia, and a bifid uvula. The remainder of his examination findings are normal. Of the following, the MOST likely associated congenital anomaly is

high myopia Stickler syndrome - connective tissue disorder characterized by ocular abnormalities (myopia, cataracts, and/or retinal detachment), midface abnormalities (flattened midface, depressed nasal bridge, short nose, anteverted nares, micrognathia, and cleft palate), hearing loss, and joint hypermobility - bifid uvula may be a marker of an underlying submucosal cleft palate - submucosal cleft palates can cause breastfeeding difficulties and poor weight gain in neonates and velopharyngeal insufficiency and associated speech abnormalities in older children and adults

Give kids what food en route to ED if ingested battery?

honey - look for halo sign in battery https://www.poison.org/battery/guideline

Maneuver for slipping rib syndrome

hooking maneuver - curl fingers under anterior costal margin and gently pull rib cage anteriorly

A 9-month-old infant is being evaluated for a rash. He was febrile earlier in the week with temperatures as high as 39.4°C, but has been afebrile for the past 24 hours. This morning, his mother noted a red rash on his neck and trunk; the rash has spread and is now present on his arms and legs as well. He is fussy and not eating normally but is drinking and has appropriate urine output. He has an erythematous, blanching, maculopapular rash confluent on his neck, chest, and upper back, but involving his whole trunk and proximal extremities. His conjunctiva and oropharynx appear normal. Of the following, the virus MOST likely causing this infant's symptoms is

human herpesvirus type 6 - Roseola is characterized by 3 to 5 days of high fever (typically >39.5°C) which resolves suddenly, at which time a rash appears. The blanching, maculopapular rash can last for hours to days, typically starting on the neck and trunk before spreading to the face and limbs (Item C120). Approximately 10% to 15% of children with primary HHV-6 infection have an associated febrile seizure. Nearly all children acquire HHV-6 infection before age 2 years. - Like other viruses in the herpesvirus family, HHV-6 remains latent in the body after primary infection and may reactivate, particularly in immunocompromised patients. Reactivation of HHV-6 is a common cause of encephalitis in patients after hematopoietic stem cell or solid organ transplantation.

Initial impression of a 2-year old girl shows her to be alert with mild breathing difficulty during inspiration and pale skin color. On primary assessment, she makes high pitched inspiratory sound (mild stridor) when agitated; otherwise, intercoastal retractions. Lung auscultation reveals transmitted upper airway sounds with adequate distal breath sounds bilaterally. Which is the most appropriate initial intervention for this child?

humidified oxygen as tolerated

Pityrisasis Rosea is treated with...

hydroxyzine - oval, scaling, thin plaques. The long axes of plaques are oriented parallel to lines of skin stress - scale is located on the trailing edge of lesions rather than at the leading edge (border) - On the back, the arrangement of pityriasis rosea lesions along lines of skin stress (and the ribs) may mimic the appearance of the boughs of a fir tree (ie, the "Christmas-tree" appearance) - In persons of color, pityriasis rosea may have an "inverse" distribution (with lesions concentrated on the extremities and relative sparing of the trunk); alternatively, lesions may be papules with fewer plaques

A 1-day-old neonate is admitted to the neonatal intensive care unit for persistent hypoglycemia. He was born at 40 weeks' gestation by cesarean delivery because of failure of labor progression. The pregnancy was otherwise uncomplicated. His birth weight was 3400 g. Jitteriness noted at 8 hours after birth prompted measurement of a plasma glucose level, which was 28 mg/dL (1.6 mmol/L). Despite treatment with oral feedings of standard infant formula, the neonate was unable to maintain a plasma glucose level above 50 mg/dL (2.8 mmol/L). He is requiring a glucose infusion rate of 8 mg/kg/min to maintain normal glucose levels. He is afebrile and has normal vital signs for age. Physical examination findings are normal except for the genital examination. His right testicle is not palpable, and his left testicle is palpable high in the scrotum. The stretched phallic length is 1.6 cm. Of the following, the MOST likely etiology of this neonate's hypoglycemia is

hypopituitarism - Hypoglycemia is a common presenting symptom of congenital hypopituitarism. - Gonadotropin deficiency is high on the differential diagnosis of micropenis and can be isolated or associated with other pituitary hormone deficiencies. - Neonates with micropenis should undergo evaluation in the immediate newborn period to detect and treat hypoglycemia, adrenal crisis, and other consequences of hypopituitarism.

A 2 week old formula-fed infant presents with poor feeding and somnolence. Examination is notable for microphallus, jaundice, and mild dehydration. Laboratory studies are significant for Na 145 mEq/L, K 3.4 mEq/L, and glucose 25 mg/dL. Total bilirubin is 13 mg/dL with conjugated fraction of 4. The most likely diagnosis is:

hypopituitarism - In panhypopituitarism, isolated adrenocorticotropic hormone (ACTH) or growth hormone deficiency, or combined ACTH deficiency plus growth hormone deficiency, the incidence of hypoglycemia is as high as 20% - In the newborn period, hypoglycemia may be the presenting feature of hypopituitarism; in males, a microphallus may provide a clue to a coexistent deficiency of gonadotropin. - Newborn infants with hypopituitarism often have a form of "hepatitis" associated with cholestatic jaundice and hypoglycemia. - The combination of hypoglycemia and cholestatic jaundice requires exclusion of hypopituitarism as a cause, because the jaundice resolves with replacement treatment of growth hormone, cortisol, and thyroid as required. This constellation is often associated with the syndrome of septo-optic dysplasia.

A 1 yr old child presents with a history of recurrent fevers for several weeks. On physical examination you find that the pupils fail to constrict. This finding is associated with:

hypothalamic dysfunction - Fever of unknown origin is sometimes caused by hypothalamic dysfunction. - A clue to this disorder is failure of pupillary constriction due to absence of the sphincter constrictor muscle of the eye. This muscle develops embryologically when hypothalamic structure and function also are undergoing differentiation.

A 6 wk old infant has gained no weight since birth. Her skin appears mottled, and an indirect bilirubin level is measured at 24 mg/dL. Her extremities are cold, and her temperature is 35oC. The most likely diagnosis is:

hypothyroidism - birthweight and length are normal, but head size may be slightly increased because of myxedema of the brain - prolongation of physiologic jaundice, caused by delayed maturation of glucuronide conjugation, may be the earliest sign - feeding difficulties, especially sluggishness, lack of interest, somnolence, and choking spells during nursing, are often present during the 1st mo of life - respiratory difficulties, due in part to the large tongue, include apneic episodes, noisy respirations, and nasal obstruction - affected infants cry little, sleep much, have poor appetites, and are generally sluggish - there may be constipation that does not usually respond to treatment - abdomen is large, and an umbilical hernia is usually present - temperature is subnormal, often <35°C (95°F), and the skin, particularly that of the extremities, may be cold and mottled - edema of the genitals and extremities may be present. The pulse is slow, and heart murmurs, cardiomegaly, and asymptomatic pericardial effusion are common - macrocytic anemia is often present and is refractory to treatment with hematinics - because symptoms appear gradually, the clinical diagnosis is often delayed

A 13-year-old girl is being evaluated for recurrent abdominal pain. She reports crampy pain localized to her lower abdomen that is 10 out of 10 in severity. She has associated nausea, but has not had any vomiting, diarrhea, or fever. The pain does not improve with acetaminophen. She has experienced similar cramping approximately once a month over the past 3 months, but her current pain is much more severe. She is premenarchal and has never been sexually active. On physical examination, she has a sexual maturity rating of 4 for breast and pubic hair. There is a palpable mass in the suprapubic region. The girl is nervous and uncooperative with an external genital examination. Of the following, the MOST likely diagnosis for this girl is

imperforate hymen - should be considered in any adolescent with cyclical abdominal pain and primary amenorrhea - one of the most common obstructive anomalies of the female genital tract - can be diagnosed in the neonatal period if a bulge is noticed at the introitus, which is due to vaginal secretions occupying the uterus, stimulated by maternal estrogen levels - if unrecognized, the mucus will be reabsorbed and the child will be asymptomatic until menarche - Can be made on physical examination, and can be confirmed with ultrasonography (translabial or transabdominal)

A 5-year-old previously healthy boy is being evaluated for rapidly progressive pain, redness, and swelling of his left lower leg. A few days earlier, he was bitten by a mosquito. On physical examination, the boy appears quiet and tired. He has a temperature of 39.1°C, pulse of 130 beats/min, respiratory rate of 30 breaths/min, and blood pressure of 80/47 mm Hg. On his left lower leg, there is a 5 × 5-cm area of induration underlying a 0.5 × 0.5-cm papule with central punctum at the site of the insect bite. There is a 6 × 6-cm area of surrounding erythema and duskiness. The area is exquisitely tender to palpation. A complete blood cell count shows a peripheral white blood cell count of 28 × 103/μL (28 × 109/L) with a differential of 85% neutrophils, 5% band forms, 8% lymphocytes, and 2% monocytes; C-reactive protein is significantly elevated. Parenteral antibiotic therapy is started. Of the following, the MOST appropriate next step in management is

incision and drainage in the operating room - The boy in the vignette has acute necrotizing fasciitis (ANF) from invasive group A β-hemolytic Streptococcus (GAS), otherwise known as Streptococcus pyogenes, an aerobic gram-positive β-hemolytic organism. - Acute necrotizing fasciitis requires urgent treatment. - Management of necrotizing fasciitis requires a combination of parenteral antibiotic therapy and aggressive, extensive surgical debridement in the operating room to halt the progression of tissue necrosis. - Treatment should not be delayed for further diagnostic studies if clinical suspicion for ANF is high. - Often only localized tissue swelling is visualized rather than the classic finding of gas.

reticulocyte count in ABO incompatibility

increased retic count

Hypertrophic cardiomyopathy may produce a murmur because of turbulent flow through a relatively obstructed outflow tract. The degree of obstruction is dynamic. As cardiac preload increases, the obstruction is reduced, and the murmur decreases in intensity. Therefore, the murmur of hypertrophic cardiomyopathy

increases with the Valsalva maneuver decreases when supine as compared to sitting or standing - These effects are opposite to those seen with most benign cardiac murmurs

Age range for roseola

infancy - typical course = defervescence and emergence of rash

A 3 mo old boy is brought in by his parents because he appears to be very light sensitive. On examination, he is tearing, and his corneas appear large and cloudy. The most likely diagnosis is:

infantile glaucoma - classic triad of epiphora (tearing), photophobia (sensitivity to light), and blepharospasm (eyelid squeezing). Each can be attributed to corneal irritation. - signs of glaucoma include corneal edema, corneal and ocular enlargement, and conjunctival injection - corneal edema leads to tearing and photophobia - glaucoma should be considered in a child in whom a nasolacrimal duct obstruction is suspected if any of these other signs or symptoms are present

Children with burns over less than 10% of their total body surface area (TBSA) have REE that is nearly normal; however, those with burns that affect more than 40% TBSA have REE that may be as high as 1.5 times the normal REE. In children with significant TBSA burns, adequate nutrition is required, and the current recommendations include

initiation of enteral nutrition as soon as possible - to ensure positive nitrogen balance and reduce gastrointestinal bacterial translocation - in rare circumstances in which enteral nutrition cannot be administered owing to severe malabsorption or feeding intolerance, parenteral nutrition can be used, but it should still provide the appropriate calories to meet the increase in REE - nutritional supplementation with vitamins C and A and zinc sulfate will ensure adequate wound healing and should be prescribed

A 2-year-old boy presents to clinic with constipation. His parents report that for the past three months, since they have started toilet training, his frequency of stooling has decreased and he seems to be withholding. He is now stooling every three to four days, and his stools are hard. He cries when stooling and sometimes has blood on the outside of his stools. Which of the following interventions is the most appropriate next step?

initiation of polyethylene glycol - Prevention of constipation in this age group can be achieved by increasing fiber intake (with a goal of 7-15 grams of fiber daily) and limiting cow's milk intake to 24 ounces per day. - Additionally, using a "relaxed" approach to toilet training and waiting until the child shows interest may prevent constipation and withholding behaviors. - In children who have already started withholding, especially in the setting of rectal bleeding as this boy has, laxatives are recommended to make the stools soft and pain-free. - Polyethylene glycol (0.4 g per kg per day) is recommended as first-line treatment. - Lactulose is considered second-line treatment if polyethylene glycol is not available. - After stools have been soft and painless for six to eight weeks, the laxative can be weaned.

T2D diabetes management approach if: no symptoms, blood glucose >250, negative ketones =

insulin +/- metformin +lifestyle changes

The most common cause of recurrent DKA is

insulin omission - Adolescent girls are a higher risk group for recurrent DKA. - Often, recurrent DKA is a symptom of an underlying psychosocial problem. Examples include intentional insulin omission for weight loss ("diabulimia"), depression, poor coping skills, or a mechanism of escape from an undesirable social situation. Thus, recurrent DKA should prompt a behavioral health evaluation. - An interdisciplinary approach to the management of recurrent DKA includes diabetes education, psychosocial evaluation, medical treatment, and appropriate adult supervision.

umbilical arteries are extensions of the...

internal iliac (hypogastric) arteries - umbilical arteries extend from pelvis to umbilicus in anterior abdominal wall deep to rectus muscle as fascia

Bilious emesis in a neonate or infant should prompt consideration for

intestinal obstruction

An 8-year-old boy presents to the clinic, for concerns of congestion, rhinorrhea, frequent sneezing, and itchy throat for the last three weeks. On examination, his turbinates are swollen and pale, and there is darkening of the skin below both eyelids. What is the best treatment for his condition?

intranasal fluticasone allergic rhinitis. - characterized by upper respiratory congestion, sneezing, and rhinorrhea, along with ocular itching, post nasal drip, and coughing. - Physical findings include infraorbital darkening, nasal crease, and crease of the lower eyelids. - The first line of treatment for allergic rhinitis is intranasal corticosteroids such as intranasal fluticasone. - Numerous studies show superiority of intranasal corticosteroids over oral antihistamines in the control of nasal symptoms. - There are first- and second-generation intranasal corticosteroids, and both are equally effective and work by decreasing the inflammatory response. - Common side effects are dryness or burning of the nasal mucosa, and epistaxis.

Expiratory wheezing suggests...

intrathoracic obstruction - asthma - bronchiolitis

Treatment of acute symptomatic neonatal or infant hypoglycemia includes ...

intravenous administration of 2 mL/kg of D10W --> followed by a continuous infusion of glucose at 6-8 mg/kg/min - adjusting the rate to maintain blood glucose levels in the normal range

Maternal immune thrombocytopenic purpura results from the passive transplacental transmission of a maternally derived antibody to a surface antigen found on both maternal and neonatal platelets, resulting in thrombocytopenia in both the mother and the neonate. Maternal immune thrombocytopenic purpura can be temporized through the administration of

intravenous immune globulin

BUN:Cr ratio of <20:1 suggests an ...

intrinsic renal injury

When to introduce cups to infants...

introduce cup as soon as infant can sit unsupported --> eliminate bottle by 1 yo

For highest risk infants (with severe eczema and/or egg allergy), what is recommended regarding solid food introductions?

introduction to peanut as early as 4-6 months of age + allergy testing strongly advised prior to peanut introduction for this group (SPT or Serum IgE)

The girl in the vignette exhibits a severe microcytic anemia with a complete absence of hemoglobin A on electrophoresis. This presentation is diagnostic of β thalassemia major. Thalassemia and thalassemia trait have an increased incidence among Middle Eastern and southeast Asian populations. Children with β thalassemia major are dependent on red cell transfusions, and the only cures are an allogeneic hematopoietic stem cell transplant or gene therapy. The chronic red cell transfusions needed to sustain these children deliver significant parenteral iron. There is no mechanism for the body to eliminate excess iron, and only a small amount of iron is lost through sloughing of the intestinal mucosa, or through menstrual bleeding in postmenarcheal girls. Iron absorption is tightly regulated through the intestine. As a consequence, children with β thalassemia major (transfusion-dependent thalassemia) will inevitably develop

iron overload - The iron overload can be managed with aggressive chelation, but it remains a significant driver of morbidity and mortality in this population.

Common warts appear as rough-surfaced (ie, verrucous) papules that may have punctate dark specks (thrombosed wart capillaries). Although a variety of treatments for common warts are available, for most children what is the preferred initial approach?

keratolytic therapy using topical salicylic acid

A 4-year-old boy is seen in the clinic for evaluation of cough and eye redness. He has had nasal congestion, mild cough, and bilateral itchy, red eyes for the past week. He has been afebrile and otherwise well, with a normal appetite and energy. He has normal vital signs. He has mild bilateral periorbital edema and erythema. His bulbar and palpebral conjunctiva are erythematous but without discharge. His tympanic membranes are normal, and he has normal findings from cardiac and respiratory examinations. Of the following, the MOST appropriate treatment for the boy's ocular symptoms is

ketotifen eye drops - The boy in the vignette has nasal congestion, mild cough, and bilateral itchy, red eyes. Although his symptoms could be consistent with either a viral or allergic conjunctivitis, the presence of pruritus and the absence of physical examination findings consistent with upper respiratory tract infection make allergic conjunctivitis more likely. - In addition to removal of the allergen, treatment for allergic conjunctivitis can be with supportive measures (eg, eye lubricant, cool compresses), a short course of vasoconstrictor/antihistamine combination drops (eg, naphazoline and pheniramine), antihistamine drops (eg, olopatadine, ketotifen), or mast cell-stabilizing drops (eg, cromolyn sodium).

A 2-year-old girl with mild global developmental delay and refractory epilepsy is placed on a ketogenic diet for management of her seizures. She experiences improved seizure control and is followed closely by a pediatric epileptologist and dietician. Her mother brings her for her health supervision visit and states that overall the girl is tolerating the diet well with mild constipation and is taking her multivitamin with calcium and vitamin D supplements daily. Her mother states that she understands that the diet can have other side effects and asks if the girl should be monitored for additional complications. Of the following, the girl is MOST at risk for

kidney stones Ketogenic diet - can be effective in the management of children with medically refractory epilepsy - with initiation of the diet, half of children show a 50% or more reduction in seizure burden independent of epilepsy type or electroencephalographic findings - common adverse events of the ketogenic diet include gastrointestinal complaints, hyperlipidemia, and kidney stones - Carbohydrate-free multivitamin, calcium, and vitamin D supplementation is recommended to prevent vitamin and mineral deficiencies, and the development of osteopenia

exam of maternal blood for fetal erythrocytes....

kleihauer-betke test

Delayed puberty

lack of breast development by age 13 in girls lack of secondary sexual development by age 14 in boys

A child in the 3rd grade has problems with spelling and reading. She appears very quiet and confused in class. Her teacher has noticed that this girl has trouble following directions. Her mind seems to wander whenever the teacher tells a story or explains something complicated. She is skilled in art and so far has performed well in arithmetic. Which of the following diagnostic procedures is most likely to yield useful findings in this child?

language evaluation - A child's language and communication skills also must be interpreted within the context of his or her overall cognitive and physical abilities. - No child is too young for a speech and language or hearing evaluation. - A referral for evaluation is appropriate whenever there is suspicion of language impairment.

Which lung lobe contains two lobes?

left - includes lingula

S2 heard best at...

left upper sternal border

Cardiogenic pulmonary edema (CPE) results from

left ventricular failure or obstruction to pulmonary venous return at proximal or mitral valve

A karyotype of 45,X, is consistent with Turner syndrome (TS). Patients with TS are known to have an increased incidence of what cardiac lesions?

left-sided cardiac lesions = bicuspid aortic valve and coarctation of the aorta - Other vascular anomalies that have been noted in TS include anomalous pulmonary venous connection and persistent left superior vena cava. - Dilation of the aorta in general, as well as dilation of other vessels such as the brachial and carotid arteries, may also be seen. - Affected patients can have resting sinus tachycardia and prolonged QT syndrome. - At risk of experiencing aortic dissection. Bicuspid aortic valve, coarctation of the aorta, and hypertension are risk factors for the development of aortic dissection. An aortic dissection is very rare but is typically fatal. The average age of dissection is 30 years, but dissection has also been seen in early childhood. Patients and their parents should be educated regarding the signs and symptoms of aortic dissection, should follow suggestions regarding imaging screening, and should undergo aggressive treatment for risk factors such as hypertension. - At the time of diagnosis, patients should be referred to pediatric cardiology for an evaluation that includes echocardiography and electrocardiography. - A magnetic resonance imaging scan should also be used in the surveillance process, once a patient is old enough to have it without sedation, unless there is reason to obtain one before that time. Interval timing of repeat imaging studies will be dictated on the basis of screening study findings

Chest radiograph showing cardiomegaly and pulm vasc congestion in infant with resp distress and normal saturations in all 4 extremities is indicative of...

left-to-right shunting process - newborn = obligate shunt (ex. AV malformation) - 2- to 8-wk old infant = dependent shunt (ex. VSD)

Risk of early-onset sepsis due to group B Streptococcus is higher among neonates born at what GA?

less than 36 weeks' or greater than 41 weeks' gestation

Should be considered in patients > 3 yr old who present with intussusception.

lymphoma - most common GI malignancy in the pediatric population - 30% of children with non-Hodgkin lymphoma (NHL) present with abdominal tumors - predisposing conditions include HIV/AIDS, agammaglobulinemia, long-standing celiac disease, and bone marrow or solid organ transplantation - can occur anywhere in the GI tract, but it most commonly occurs in the ileocecal region and small bowel

A previously healthy 2 wk old now has progressive lethargy. Physical examination reveals muscle rigidity, opisthotonos posture, periods of hypertonicity, and flaccidity. Laboratory data reveal hypoglycemia, metabolic acidosis, and cerebral edema. Plasma levels of leucine, isoleucine, and valine are elevated. The most likely diagnosis is:

maple syrup urine disease - infants who are normal at birth develop poor feeding and vomiting in the 1st wk of life, but lethargy and coma may ensue within a few days. - Physical examination reveals hypertonicity and muscular rigidity with severe opisthotonos. - Periods of hypertonicity may alternate with bouts of flaccidity. - Neurologic findings are often mistaken for generalized sepsis and meningitis. - Cerebral edema may be present; convulsions occur in most infants, and hypoglycemia is common. - In contrast to most hypoglycemic states, correction of the blood glucose concentration does not improve the clinical condition. - The disorder is usually confirmed by amino acid analysis showing marked elevations in plasma levels of leucine, isoleucine, valine, and alloisoleucine (a stereoisomer of isoleucine not normally found in blood) and depression of alanine

The approach to laboratory workup for juvenile dermatomyositis should include

markers of serum muscle enzymes including creatine phosphokinase, aspartate transaminase, alanine aminotransferase, and lactic acid dehydrogenase concentrations

The most common complication of mumps in prepubescent children is:

meningitis - The most common complications of mumps are meningitis, with or without encephalitis, and gonadal involvement. - Mumps virus is neurotropic and is thought to enter the CNS via the choroid plexus and infect the choroidal epithelium and ependymal cells, both of which can be found in CSF along with mononuclear leukocytes. - The meningoencephalitis may occur before, along with, or following the parotitis. It most commonly manifests 5 days after the parotitis. - In adolescent and adult males, orchitis is 2nd only to parotitis as a common finding in mumps. - Involvement in prepubescent boys is extremely rare, but after puberty orchitis occurs in 30-40% of males.

Infants receiving iNO therapy should have what measured daily?

metHgb - methemoglobinemia (metHgb >5%) occurs in appx 10% of newborns treated with iNO and resolves with decreasing the iNO dose

A 4 yr old girl has experienced progressive loss of ambulation over a 2-yr period. On examination, the child is apathetic and uninterested in her surroundings. She has horizontal nystagmus and optic atrophy. Her voice is dysarthric. She is hypotonic, and her deep tendon reflexes are absent. A sibling died at the age of 6 yr with a similar history. The motor nerve conduction velocities show marked slowing, and computed tomography (CT) of the head shows diffuse symmetric attenuation of the cerebral and cerebellar white matter. The most likely diagnosis is:

metachromatic leukodystrophy - Late infantile MLD begins with insidious onset of gait disturbances between 1 and 2 yr of age. - The child initially appears awkward and frequently falls, but locomotion is gradually impaired significantly and support is required in order to walk. The extremities are hypotonic, and the deep tendon reflexes are absent or diminished. Within the next several months, the child can no longer stand, and deterioration in intellectual function becomes apparent. The speech is slurred and dysarthric, and the child appears dull and apathetic. Visual fixation is diminished, nystagmus is present, and examination of the retina shows optic atrophy. - Within 1 yr from the onset of the disease, the child is unable to sit unsupported, and progressive decorticate postures develop. - Patients ultimately become stuporous and die of aspiration or bronchopneumonia by age 5-6 yr. - Neurophysiologic evaluation shows slowing of peripheral nerve conduction velocities (NCVs) and progressive changes in the VEPs, ABRs, and somatosensory- evoked potentials (SSEPs). - CT and MRI of the brain indicate diffuse symmetric attenuation of the cerebellar and cerebral white matter, and examination of the CSF shows an elevated protein content. - Bone marrow transplantation is a promising experimental therapy for the management of late infantile MLD.

Diagnosis of PWS is made by

methylation analysis - imprinting disorder caused by an absence of expression of paternally derived genes in the PWS/Angelman syndrome region of chromosome 15q11.2-q13 - recurrence risk, the risk that parents will have another child with PWS, is very low for the first two mechanisms at <1% (the same as in the general population), whereas it is 50% if the underlying etiology is an imprinting center defect

strabismus is defined as...

misalignment of the eyes

Class to start with for ADHD when looking at stimulant medications

methylphenidate class - better tolerated - inhibits the reuptake of dopamine and norepinephrine, increased dopaminergic and noradrenergic activity in the prefrontal cortex may explain its efficacy in ADHD

Hemolytic uremic syndrome is characterized by the triad of ...

microangiopathic hemolytic anemia thrombocytopenia kidney injury

Stickler syndrome, a connective tissue disorder, is associated with ...

midfacial hypoplasia cleft palate Pierre Robin sequence hearing loss eye abnormalities (including high-grade myopia, cataracts, and increased risk for retinal detachment) - Pierre Robin sequence is the clinical constellation of micrognathia, cleft palate, and glossoptosis that can lead to life-threatening obstructive apnea and feeding difficulties in the neonatal period.

A 24-hour-old term neonate is being evaluated. He was born at 38 weeks' gestation by cesarean delivery after failed induction for maternal preeclampsia; his birthweight was 3.6 kg. Since birth, he has breastfed 8 times. His nurse reports a good latch. He has had 1 meconium stool and 2 wet diapers. His weight is 3.2 kg and his physical examination findings are unremarkable. Of the following, the clinical information that SUPPORTS the decision not to supplement with formula is

mode of delivery - For full-term neonates, loss of 7% to 10% of birthweight is acceptable and not unexpected over the first week after birth. - Neonates born via cesarean section may lose more weight in the first 2 days after birth than those delivered vaginally. - In an exclusively breastfed neonate, weight loss of greater than 10% in the first 24 to 48 hours after birth should alert clinicians to the possibility of problems with breastfeeding.

Sedation characterized by purposeful response to verbal commands with or without gentle stimulation, maintenance of cardiovascular function, spontaneous ventilation, and no required intervention for airway maintenance.

moderation sedation

With elective or semi-elective intubation and if baby is hemodynamically stable, give....

morphine (0.1 mg/kg) and/or lorazepam (0.05 mg/kg) IV to facilitate intubation, to prevent increases in intracranial pressure, and decrease stress responses d/t intubation - monitor BP; even these low drug doses can produce hypotension if infant is hypovolemic

Phenotypic features of multiple endocrine neoplasia type 2B include

mucosal neuromas tall, thin body habitus full lips joint laxity alacrima - Gastrointestinal tract ganglioneuromas may cause intermittent constipation and diarrhea. - Multiple endocrine neoplasia type 2B is associated with early-onset and aggressive medullary thyroid carcinoma, in addition to pheochromocytoma and the physical phenotype.

Which viral illnesses are known to cause vestibular neuronitis and can cause vertigo?

mumps measles EBV

A previously healthy 12 yr old boy presents with upper respiratory symptoms of 8 days' duration, with worsening cough and fever. The season is autumn. Examination reveals a temperature of 39°C, a respiratory rate of 24 breaths/min, and inspiratory crackles in both lung fields. Oxygen saturation is 93-94%. Chest film shows scattered infiltrates in multiple lung fields, more focal consolidation in the right lower lobe, and blunting of the right costophrenic angle. White blood cell count is 11,000/mm3 with a normal differential. Which of the following is the most appropriate next step in diagnosis?

mycoplasma pneumoniae PCR - In this age group, atypical pneumonia is most likely. - Atypical pneumonia due to C. pneumoniae or M. pneumoniae is difficult to distinguish from pneumococcal pneumonia on the basis of radiographic and laboratory findings - Acute infection caused by M. pneumoniae can be diagnosed on the basis of a positive polymerase chain reaction (PCR) test result or seroconversion in an IgG assay

The most common cause of death from Kawasaki disease (KD) in the early phase of disease is:

myocarditis - Cardiac involvement is the most important manifestation of KD. - Myocarditis occurs in most patients with acute KD and manifests as tachycardia out of proportion to fever along with diminished left ventricular systolic function.

Common side effects of tacrolimus

n/v/d HT hypomagnesemia hyperkalemia Also: DM, tremor, HA, renal dysfunction

A 2 yr old girl had an upper respiratory tract infection 5-7 days ago. She had a low-grade fever, cough, and rhinorrhea. She did not require any medical intervention. She appeared to be improving; now, however, she has a high fever and brassy cough. On physical examination she appears toxic and has a fever of 39.8°C. She can lie flat; she does not drool and has no dysphagia but does have some evidence of respiratory distress with increased work of breathing and retractions. On auscultation her lungs are clear bilaterally. Which of the following is the most appropriate antibiotic for this condition?

nafcillin + vancomycin Bacterial tracheitis - acute bacterial infection of the upper airway that is potentially life threatening - Staphylococcus aureus is the most commonly isolated pathogen. Moraxella catarrhalis, nontypable H. influenzae, and anaerobic organisms have also been implicated. - The mean age is between 5 and 7 yr. - Often follows a viral respiratory infection (especially laryngotracheitis), so it may be considered a bacterial complication of a viral disease, rather than a primary bacterial illness. - Typically, the child has a brassy cough, apparently as part of a viral laryngotracheobronchitis. - High fever and "toxicity" with respiratory distress can occur immediately or after a few days of apparent improvement. - The patient can lie flat, does not drool, and does not have the dysphagia associated with epiglottitis. - Appropriate antimicrobial therapy, which usually includes antistaphylococcal agents, should be instituted in any patient whose course suggests bacterial tracheitis. - Current empirical therapy recommendations for life-threatening infections such as bacterial tracheitis include vancomycin and a β-lactamase-resistant β-lactam antimicrobial agent (e.g., naficillin or oxacillin). - When bacterial tracheitis is diagnosed by direct laryngoscopy or is strongly suspected on clinical grounds, an artificial airway should be strongly considered. - Supplemental oxygen is usually necessary.

Patient has right flank pain radiating to the suprapubic region, gross hematuria with clots, and a urinalysis showing minimal pyuria; these findings favor a diagnosis of an acute episode of

nephrolithiasis or renal stone - The diagnosis of nephrolithiasis is confirmed by abdominal imaging. Renal ultrasonography is the initial test, but it may not detect small stones or ureteral stones. A plain abdominal radiograph helps in detecting radiopaque stones. Noncontrast helical computed tomography is the most sensitive test for detection of nephrolithiasis in children. - The acute management of nephrolithiasis includes pain control, aggressive fluid administration, and facilitating passage or removal of stone. - The long-term management includes evaluation of the type of nephrolithiasis and the risk factors for stone recurrence. Accordingly, dietary interventions and medications are recommended to prevent recurrent nephrolithiasis.

A 4 yr old child presents with a hard, fixed abdominal mass that causes discomfort. Physical examination also shows hypertension. The most likely etiology is:

neuroblastoma - most common extracranial solid tumor in children and the most commonly diagnosed malignancy in infants - can mimic many other disorders and may be difficult to diagnose - metastatic disease can cause a variety of signs and symptoms, including fever, irritability, failure to thrive, bone pain, cytopenias, bluish subcutaneous nodules, orbital proptosis, and periorbital ecchymoses - can also be associated with a paraneoplastic syndrome of autoimmune origin, termed opsoclonus-myoclonus-ataxia syndrome - some tumors produce catecholamines that can cause increased sweating and hypertension, and some release vasoactive intestinal peptide, causing a profound secretory diarrhea

Temperatures in excess of 41°C are most often associated with ...

noninfectious cause - causes for very high temperatures (>41°C) include central fever (resulting from central nervous system (CNS) dysfunction involving the hypothalamus), malignant hyperthermia, malignant neuroleptic syndrome, drug fever, or heatstroke

Drug-induced acute interstitial nephritis is commonly caused by

nonsteroidal anti-inflammatory drugs, such as ibuprofen - The mainstay of initial treatment for drug-induced acute interstitial nephritis is discontinuation of the offending agent. - The majority of patients improve after the offending medication is withdrawn. - Supportive interventions for acute interstitial nephritis may include fluid and electrolyte management, adequate hydration, and avoidance of nephrotoxic drugs.

A 12-year-old, right-handed boy is seen for evaluation of right medial elbow pain. He has played baseball for the past 6 years and is considered the best pitcher on his travel team. This past year he has been noticing increasing pain over the medial aspect of his elbow. He has been icing and using ibuprofen after every practice with some improvement but continues to have pain with throwing. He reports no traumatic injury, but one week ago he had increased pain while pitching and noted medial elbow swelling. He feels better after resting the elbow and using ice and a compression wrap over the past several days. He is otherwise feeling well and has no other musculoskeletal concerns. There is a small amount of swelling and tenderness to palpation over the medial epicondyle and a minimal flexion contracture at the right elbow as compared to the left. Strength testing is normal in the right arm and elicits no pain. His physical examination findings are otherwise unremarkable. Of the following, the BEST next step in management is to

obtain bilateral elbow radiographs - Overhead throwing places significant stress across the medial elbow, and the boy in this vignette has sustained an avulsion injury of the medial epicondyle. - Radiographs are indicated to assess for possible displacement of the epicondyle, and bilateral images are often needed in skeletally immature athletes to assess for subtle changes in growth plate alignment. - Growth plate injuries are more common than tendon overuse syndromes in the pediatric elbow. - Bilateral elbow radiographs are often needed for detection of physeal injuries in the elbow.

Treatment of poison ivy

oral prednisone - 14-21 days

A 2-week-old female infant is seen during rounds in the neonatal intensive care unit. The infant was delivered at 32 weeks' gestation because of maternal preeclampsia; she was small for gestational age with a birthweight of 1,200 g. Her neonatal course was complicated by mild respiratory distress requiring nasal continuous positive airway pressure for the first 4 days after birth and unconjugated hyperbilirubinemia for which she received 3 days of phototherapy in the first week after birth. She tolerated enteral feeding well, and was receiving fortified breast milk. However, her mother's milk supply has decreased and the infant was recently switched to a cow milk-based premature formula. She is growing well and takes a combination of oral and nasogastric feedings without difficulty. Her parents ask about using a cow milk-based formula designed for term infants. Of the following, the condition this neonate would be MOST at risk for, with the use of this suggested formula, is

osteopenia of prematurity - Preterm neonates have increased calcium and phosphorus requirements compared with those delivered at term. - Use of fortified breast milk or enriched preterm formula with increased calcium and phosphorus can decrease the likelihood of the development of osteopenia of prematurity.

Toddlers who consume how much cow milk daily are at high risk of iron deficiency?

over 32 oz

The measurement which best assesses an infant's oxygenation status is ...

oxygen saturation

A 3-month-old male infant is seen for concerns of frequent regurgitation and irritability, which began at 1 month of age. He is exclusively breastfed and has normal growth and development. He feeds about every 3 hours and does not have any difficulty swallowing. Shortly after feedings, he begins to cry and is difficult to settle. He will often spit up or vomit during this time. About 1 month ago, his mother eliminated dairy and soy from her diet, but she has not noted improvement in his symptoms. He has a daily yellow, seedy stool without blood. He was born at 42 weeks' gestation via cesarean delivery for fetal distress but had no complications. He has a weight at the 95th percentile for age, length at the 98th percentile for age, and weight-for-length at the 95th percentile for age. He is smiling and in no apparent distress. No abnormalities are observed during physical examination. His mother is concerned about his irritability and frequent regurgitation and would like to discuss potential treatments. Of the following, the BEST next step for this infant is

parental education and support - The infant in this vignette has gastroesophageal reflux and fussiness with excellent weight gain and development. Parental education, support, and reassurance are advised as the most appropriate next step.

A 6 mo old presents with tachycardia, tachypnea, and poor feeding for 3 mo. Physical examination reveals a continuous machinery murmur and a wide pulse pressure with a prominent apical impulse. The most likely diagnosis is:

patent ductus arteriosus - large PDA will result in heart failure similar to that encountered in infants with a large VSD - small PDA is associated with normal peripheral pulses, and a large PDA results in bounding peripheral arterial pulses and a wide pulse pressure, due to runoff of blood into the pulmonary artery during diastole - thrill, maximal in the 2nd left interspace, is often present and may radiate toward the left clavicle, down the left sternal border, or toward the apex - usually systolic but may also be palpated throughout the cardiac cycle - classic continuous murmur is described as being like machinery in quality

Formula on onc in which chemo is likely to cause side effects GI-wise

peptamen/Kate farms

Intraperitoneal calcifications visualized in newborn suggestive of...

perforation with meconium ileus

A 20 mo old female infant presents with disproportionately short limbs, long trunk, and large head. An examination shows midface hypoplasia, mild hypotonia of the lower extremities, and exaggerated lumbar lordosis. Which of the following is the most appropriate diagnostic test for this patient?

plain films of the skeleton - Radiographic evaluation for a chondrodysplasia should include plain films of the entire skeleton. - Efforts should be made to identify which bones and which parts of bones (epiphyses, metaphyses, diaphyses) are most affected. - If possible, films taken at different ages should be examined because the radiographic changes evolve with time. -p Films taken before puberty are generally more informative because pubertal closure of the epiphyses obliterates many of the signs needed for a radiographic diagnosis.

Rare complication of peritoneal dialysis (PD) which can progress quickly to cause acute respiratory distress

pleural effusion

A 5 yr old girl with cirrhosis and portal hypertension experiences increasing abdominal distention and fever. Shifting dullness and a puddle sign, as well as abdominal tenderness, are noted on physical examination. Paracentesis reveals cloudy fluid. Culture of the ascitic fluid is most likely to reveal:

pneumococci Primary peritonitis - bacterial infection of the peritoneal cavity without a demonstrable intra-abdominal source - most cases occur in children with ascites resulting from nephrotic syndrome or cirrhosis - infection can result from translocation of gut bacteria as well as immune dysfunction - rarely, primary peritonitis occurs in previously healthy children - pneumococci (most common), group A streptococci, enterococci, staphylococci, and gram-negative enteric bacteria, especially Escherichia coli and Klebsiella pneumoniae, are also commonly found

Standard order for chest radiograph is

posteroanterior (PA) and lateral views - taken at full inspiration --> diaphragm at level of 8th-10th posterior rib or 5th-6th anterior rib

A female neonate was born at 26 weeks of gestation via cesarean delivery. The mother received a dose of betamethasone 2 hours before the delivery. Cord clamping was performed 30 seconds after birth. The neonate was resuscitated in the delivery room using continuous positive airway pressure with a positive end-expiratory pressure of +5 mm Hg and fraction of inspired oxygen equal to 30%. On day 1 after birth, she underwent intubation due to poor respiratory effort, and developed a pneumothorax that required chest tube placement. Ultrasonography of the head performed 1 week after birth shows a right grade 3 intraventricular hemorrhage. Her mother asks what caused this bleeding. Of the following, the risk factor MOST likely to be associated with this complication is

pneumothorax - Pneumothorax requiring chest tube placement is associated with increased risk of intraventricular hemorrhage (IVH) in premature neonates. - Intraventricular hemorrhage is primarily a disease of premature neonates born before 32 weeks of gestation, and occurs due to a combination of developmental factors and postnatal exposures. - After birth, premature neonates have impaired autoregulation of their cerebral vasculature, unlike term neonates, whose cerebral perfusion is maintained at a constant value as systemic blood pressure varies. Therefore, in premature neonates, fluctuations in systemic blood pressure can cause acute increases and decreases in cerebral perfusion that increase the risk of bleeding into the vascularized germinal matrix, resulting in IVH. - The risk of IVH is highest in the first few hours after birth. Clinically, most neonates with IVH are asymptomatic. Rarely, a premature neonate will present with an acute drop in blood pressure, decrease in activity, and associated increased fullness of the anterior fontanelle. - Screening head ultrasonography at 1 week of age will identify approximately 90% of cases of IVH.

A 16-year-old adolescent girl is seen for evaluation of excessive daytime sleepiness for the past year. Despite sleeping an average of 8 to 10 hours each night, she states that she is constantly falling asleep during the school day and sometimes feels drowsy when out with friends on the weekends. Her academic performance has been declining over the course of this school year. For the past 3 months, she has noted weakness in her neck and face with strong emotions, especially laughing with her friends. She recalls one episode of waking and being unable to move her arms and legs for several minutes, which self-resolved. Her general physical and neurological examination findings are normal. Diagnostic test?

polysomnography with multiple sleep latency test - Polysomnography with multiple sleep latency tests is the gold standard for confirmatory diagnostic testing for narcolepsy. - Management of narcolepsy is aimed at supportive care targeted at the clinical features experienced by the patient with recognition and management of common medical and neuropsychiatric comorbidities.

A 13-year-old adolescent girl is brought to the office by her mother. The mother is concerned because her daughter's period has lasted for 2 weeks. She is using 5 to 6 overnight pads per day and occasionally soaks through her pads at school. She had menarche at age 12 years and had 2 regular periods followed by no bleeding for 5 months. She reports no fatigue, headaches, palpitations, or dizziness. She has no other significant medical history except for nosebleeds associated with seasonal allergies during the spring and summer. She has a temperature of 37.1°C, a heart rate of 96 beats/min, and a blood pressure of 110/76 mm Hg. She is at the 50th percentile for height and weight. Her physical examination findings are significant for conjunctival pallor. Her lungs are clear bilaterally. Her heart examination is significant for tachycardia but no murmurs or gallops. She has no hepatosplenomegaly, petechiae, or lesions on her skin. Of the following, the MOST appropriate initial test in determining the etiology of this patient's symptoms is

pregnancy test - In adolescents with abnormal uterine bleeding, a pregnancy test must be done as the initial step in evaluation.

OCP for birth control you can use

prempro (conj estro-medroxyprogestace) - lowest dose, like 3-0.02 mg per tablet another for = yaz, gianvi (drospierenone-ethinyl estradiol) - 3-0.02 mg per tablet

What constitutes irregular cycle, particularly in years following menarche?

primary amenorrhea at age 15 or >3 years post thelarche

A 14-year-old girl is being evaluated for severe abdominal pain. For each of the past 3 months, she has missed 1 to 2 days of school at the start of her menses because of cramping, nausea, and vomiting. She only experiences these symptoms during menstruation. Her mother has been treating the pain with 400 mg of ibuprofen orally every 8 hours, with minimal relief. The girl had menarche at age 12 years. Her periods occur regularly every 28 days, and last for 5 days. She had never experienced menstrual cramping before the previous 3 months. Her mother has a history of fibroids, and her maternal aunt has endometriosis. The girl has a blood pressure of 108/70 mm Hg, heart rate of 80 beats/min, respiratory rate of 18 breaths/min, and temperature of 37°C. There is mild diffuse tenderness of her lower abdomen. There is no guarding, rebound tenderness, palpable masses, or costovertebral angle tenderness. The remainder of the examination findings are unremarkable. Of the following, the girl's MOST likely diagnosis is

primary dysmenorrhea - most common gynecologic condition in adolescent girls, and one of the most common reasons for adolescent girls to miss school - painful menses without pelvic pathology - usually associated with crampy, lower abdominal pain that may present a few days before the onset of bleeding and can last for a few days or throughout the entire period - symptoms tend to be most severe during the first few days of bleeding - thought to be related to increased levels of uterine leukotrienes and cyclooxygenase which are converted to prostacyclins and prostaglandins. prostaglandin F2α mediates smooth muscle contraction and stimulates pain sensation, and prostaglandin E2 causes vasodilation in the endometrium and platelet disaggregation

Attacks of FMF can be prevented by ...

prophylactic colchicine - 0.02-0.03 mg/kg/day; maximum 2 mg/day: in 1 to 2 divided doses - initial dose should be 0.5 mg/day for children < 5 yr of age, 1 mg/day for children 5-10 yr, and 1.5 mg/day for those > 10 yr - reduces the frequency of acute attacks and also greatly decreases the probability of development of amyloidosis - may produce partial regression of existing amyloidosis

A neonate is being evaluated 8 hours after birth for decreased perfusion. He was born at 38 weeks' gestation. His mother is a 33-year-old woman with a history of poorly controlled gestational diabetes. Her laboratory testing is significant for group B Streptococcus-negative status and hemoglobin A1c of 9.3% at 36 weeks' gestation. The neonate has breastfed twice. His vital signs show a heart rate of 205 beats/min, respiratory rate of 35 breaths/min, blood pressure of 45/23 mm Hg, and temperature of 37.2°C. On physical examination, he appears plethoric, has a 1/6 systolic murmur at the left lower sternal border, and +1 peripheral pulses. Echocardiography reveals asymmetric septal hypertrophy. Of the following, the pharmacologic therapy MOST appropriate for this neonate is

propranolol - Neonates born to mothers with poorly controlled diabetes are at risk for cardiomegaly, asymmetric septal hypertrophy, and heart failure. - Neonates with asymmetric septal hypertrophy may develop dynamic outlet obstruction with decreased cardiac output.

Neonates with critical congenital heart disease can exhibit cyanosis, which requires urgent initiation of

prostaglandin infusion to reopen the ductus arteriosus - Neonates with congenital heart disease lesions associated with cyanosis have decreased pulmonary blood flow or decreased mixing of oxygenated and deoxygenated blood at the atrial level, as seen in transposition of the great arteries. - Prognosis in neonates with cyanotic heart disease depends on the severity of the cardiac lesion, surgical outcome, and the presence of genetic syndromes and comorbidities.

An 8-year-old boy is brought to the office with concerns about a mass in his groin. He was diagnosed with a buttock abscess 3 days ago that was successfully incised and drained. He has been otherwise well, afebrile, and with no pain. His vital signs are normal. The boy's physical examination reveals a healing incision on his left buttock and a 2-cm subcutaneous, nontender, nonerythematous, mobile, well-circumscribed nodule in his left groin. Of the following, the BEST next step in management is to

provide parental reassurance - Reactive lymphadenopathy is one of the most common causes of inguinal masses in older children and adolescents, and requires limited evaluation.

A woman brings her 12-year-old son to the emergency department after finding bats in the room in which they were sleeping. The family is on vacation and staying in a log cabin in a heavily wooded area. They spent the previous day hiking and the boy had fallen, sustaining abrasions to his arms and legs. When returning to the cabin she cleaned his wounds with soap and water. In the middle of the night she was awoken by scratching noises and saw 2 bats when she turned on the lights. She swatted at them with a broom, and they flew out of an open window. The boy reports that he feels fine and does not think he was bitten by a bat, although he is not sure. His physical examination findings are normal aside from large areas of abrasions to his knees, shins, palms, and forearms. There are no definitive bite marks. He has no chronic medical conditions. His mother has his vaccine card, which shows a Tdap vaccination 6 months ago. Of the following, the BEST postexposure prophylaxis for this boy is

rabies vaccine and rabies immunoglobulin - The boy in this vignette was sleeping in a room where bats were later found. The child is unsure if he has been bitten in part due to the abrasions on his arms and legs. However, as bats are extremely high risk for infection with rabies and bat bites often go unnoticed even without an overlying wound, it is recommended that he receive rabies postexposure prophylaxis. Because there is a history of close contact with a bat, whether or not a bite is visualized, both the rabies vaccine and rabies immunoglobulin should be administered for rabies postexposure prophylaxis as soon as possible. - Appropriate rabies postexposure prophylaxis of an immunocompetent patient consists of both the rabies vaccine and rabies immunoglobulin on the day of the bite (day 0). Rabies immunoglobulin is administered at a dose of 20 U/kg with as much of the immunoglobulin as possible injected directly into the wound and the excess administered into a deltoid muscle. The rabies vaccine is administered into the contralateral deltoid muscle during the initial visit and also administered on days 3, 7, and 14 after the initial dose. Rabies immunoglobulin is not administered at subsequent visits. Additional management of an animal bite consists of thorough cleaning, debridement of devascularized tissue, repair of facial and neck wounds, and consideration for antibiotic prophylaxis and tetanus postexposure prophylaxis.

A 6-year-old previously healthy girl presents to the rural emergency department where you work, after she was bitten by an unknown dog during a family camping trip. Approximately 90 minutes ago, the girl and her siblings were playing close to their family's campsite when a stray dog appeared, barking and growling at them. The children ran back toward the family campsite; however, the dog ran after them and attacked the girl, biting her multiple times in her right axillary region, upper back, shoulder, and upper arm before running away into the woods. The girl's parents immediately took her to the park ranger who applied direct pressure to her bites with gauze and called emergency medical services. The ranger attempted but was unable to locate the dog. On arrival to the emergency department, the girl is alert and oriented, but crying in pain. Her temperature is 36.7°C, heart rate is 120 beats/min, respiratory rate is 16 breaths/min, and blood pressure is 110/70 mm Hg. On physical examination, she has no respiratory distress, and her extremities are well perfused. You note multiple (>20) puncture wounds over her right shoulder and upper arm, a 3 × 4-cm gaping wound in her right axillary region, and a 6 × 8-cm deep wound to her right upper back with exposed muscle. There is a slight bleeding from the deep wound on the girl's back. The girl's medical and surgical history are not significant, she takes no medications, has no known drug allergies, and is up to date on all recommended immunizations. A nurse places a peripheral intravenous catheter, and you order an analgesic for her pain. Because of the number and complexity of the girl's wounds, you request an emergent surgical consultation for evaluation and management of the girl's wounds. Of the following, the MOST appropriate treatment plan for this girl includes

rabies vaccine, rabies immunoglobulin, and intravenous ampicillin-sulbactam - bacterial organisms most commonly involved in wound infections arising from dog bites are Staphylococcus aureus and Pasteurella species - amoxicillin-clavulanic acid is the recommended first-line oral agent for children requiring antibiotic therapy after sustaining dog bite wounds. - for children requiring intravenous antibiotic therapy after sustaining dog bite wounds, ampicillin-sulbactam is the recommended first-line antibiotic agent. an extended-spectrum cephalosporin or trimethoprim-sulfamethoxazole plus clindamycin is the recommended alternative regimen for children who are allergic to penicillin. - any dog, cat, or ferret that has bitten a human and is suspected of having rabies should be captured, confined, euthanized, and tested for the virus or should be observed by a veterinarian for period of at least 10 days. - for patients bitten by dogs that appear healthy and can be observed for 10 days, rabies postexposure prophylaxis is indicated only if the dog develops signs of rabies during the observation period. Patients bitten by dogs that are known or suspected to be rabid should receive postexposure prophylaxis as soon as possible.

What is the most common finding in children under the age of 5 years that present to the emergency department with acute appendicitis?

rebound tenderness Appendicitis - Nearly half of young children with appendicitis do not have localized tenderness, migration of pain, or anorexia. - Rebound tenderness and diffuse abdominal pain are frequent findings and may be due to the high prevalence of perforation in this age group.

Immunoglobulin A nephritis characteristically presents with

recurrent episodes of gross hematuria triggered by an upper respiratory tract or gastrointestinal illness - the synpharyngitic (concomitant with an upper respiratory illness) presentation of IgA nephritis differentiates it from acute poststreptococcal glomerulonephritis, in which a prior history of sore throat is present - the gross hematuria usually resolves within a few days to a week - the laboratory evaluation of IgA nephritis may show features of AGN (azotemia, proteinuria, and hematuria) and a normal serum level of C3 and C4 - the serum IgA level can be high or normal and is not diagnostic of IgA nephritis - treated by using different regimens based on severity of presentation and findings on the renal biopsy - children may need intravenous methylprednisolone pulses, oral prednisone, other immunosuppressive medications, or angiotensin-converting enzyme inhibitors - poor prognostic markers are proteinuria, hypertension, acute kidney injury at presentation, and severe histologic changes on the renal biopsy

Accidental liquid dishwater detergent in 2 year old boy + drooling profusely + vomiting + ulcer on lower lip and tongue --> next step in eval and management of the boy?

refer boy for urgent endoscopy - within 6-24 hours - ulcers = point towards potential esophageal burns

A 14-year-old basketball player is being evaluated for a recurrent left ankle injury. Several days ago he sustained his third inversion injury to this ankle over the past several months. These injuries result in mild swelling over the lateral ankle for several days, which he treats with a compression wrap, ice, and several doses of ibuprofen. He was able to return to full basketball participation within 7 to 10 days after each injury. However, with the current injury, the ankle feels unstable and he tapes it before practice. The patient is otherwise healthy. On physical examination, the adolescent's left ankle is slightly swollen and minimally tender inferior to the lateral malleolus. He has full and pain-free strength and range of motion in both ankles. There is increased laxity with anterior drawer testing on the left compared with the right. His gait is normal and he moves comfortably throughout the evaluation. The adolescent has some difficulty with single leg hop and balance testing on the left leg compared with the right. Of the following, the BEST next step in management for this patient is

referral to a physical therapist - The boy in the vignette does not exhibit any of the aforementioned findings, and the location of his tenderness just distal to the fibula most strongly suggests a ligamentous injury. Therefore, radiography is not indicated. - Physical therapy will be most likely to speed his recovery.

What to do when you have a kiddo with nevus sebaceous ...

remove before adolescence bc it can undergo malignant degeneration...

Potter's syndrome =

renal agenesis + pulmonary hypoplasia - fetal - flat nose, low set ears, receding chin, arthrogryposis, bell-shaped chest - prolonged oligohydramnios

After identification and removal of lead sources, children with mild lead toxicity (5-44 ug/dL) should have...

repeat venous blood lead level within 1 month - to confirm that value is not increasing

You get a sweat test in infant that is 30 ... next step?

repeated and worked up

What major changes in infant circulation occurs following birth in the systemic circulation?

resistance increased with removal of placenta

Results from an alveolar space-filling lesion such as pneumonia. It is especially common in patients with neuromuscular weakness who lack the ability to reinflate the collapsed area of lung or in patients with chest wall pain who limit their lung expansion and coughing.

resorptive atelectasis

Cardinal sign in babies with congenital diaphragmatic hernia (CDH)

respiratory distress

A 9 yr old previously healthy white girl manifests progressive painless proptosis, periorbital edema, and decreased visual acuity of the left eye during a 2-mo period. The most likely diagnosis is:

rhabdomyosarcoma - most common presenting feature of rhabdomyosarcoma is a mass that may or may not be painful - symptoms are caused by displacement or obstruction of normal structures - orbital primary tumors are usually diagnosed early in their course because of associated proptosis, periorbital edema, ptosis, change in visual acuity, and local pain - most common soft tissue sarcoma in children - may occur at virtually any anatomic site but are usually found in the head and neck (25%), orbit (9%), genitourinary tract (24%), and extremities (19%); retroperitoneal and other sites account for the remainder of primary sites

What is the most common germ cell tumor of childhood?

sacrococcygeal teratoma - account for 40% of germ cell tumors. - can have glandular tissue, teeth, hair, and bones. - located in the distal part of the spine and may be detected in utero on prenatal ultrasound. - When available, fetal MRI is recommended to detail the characteristics of the mass. - Serial ultrasounds are done to monitor the size of the mass. - A high-output cardiac state can occur, especially if the teratoma is highly vascular, which can lead to hydrops fetalis. - Treatment in the postnatal period is complete surgical resection of the teratoma. - Bowel and bladder incontinence is a common complication in spite of complete removal of the mass. - For patients with malignant components, adjuvant chemotherapy is required.

the fracture line extends from the articular surface through the epiphysis, across the physis all the way into the metaphysis

salter harris IV - T (fracture Through physis)

A 3 yr old boy is missing from his mother's house approximately 1 mo after a divorce. The most likely explanation is:

searching for his father - More long-term separations occur due to divorce, placement in foster care, or adoption, whereas permanent separation may occur due to death. - Some children may repeatedly ask for the absent parent and question when he or she will return. - The child may go to the window or door or out into the neighborhood to look for the absent parent; a few may even leave home or their place of temporary placement to search for their parents. Other children may not refer to the parental absence at all.

In a pediatric emergency, a focused history and physical examination occurs in which assessment?

secondary assessment - The components of a secondary assessment include a focused history and focused physical examination. - The history should be targeted to information that could explain cardiorespiratory or neurologic dysfunction and should take the form of a SAMPLE history (Signs/symptoms, Allergies, Medications, Past medical history, timing of Last meal, and Events leading to this situation). - Medical personnel not engaged in resuscitative efforts can be dispatched to elicit history from witnesses or relatives. - The physical examination during the secondary assessment is a thorough head-to-toe examination, although the severity of the child's illness or injury could necessitate curtailing portions of the examination or postponing nonessential elements until a later time.

The fear of dying in young children is best described as fear of:

separation - Children's fears of death are often centered on the concrete fear of being separated from parents and other loved ones and what will happen to their parents rather than themselves. - This can be true for teens and young adults as well. - This fear may be responded to in different ways: some families may give reassurance that loving relatives will be waiting, whereas others use religious figures to refer to an eternal spiritual connection.

A 2-year-old girl is having a health supervision visit. She has been healthy and is on track with her development. Her parents are getting a divorce. They are planning on living in the same neighborhood so that they can spend as much time as possible with their daughter. They would like to know how their daughter might respond to the upcoming transition in their lives. Of the following, the MOST likely response is

separation anxiety - Toddlers commonly exhibit separation anxiety as a response to the divorce of their parents. - Children adjust best to divorce when custody arrangements allow them to maintain a meaningful relationship with each parent and to have stability in schedules and caregivers.

critical sample labs for hypoglycemia...

serum glucose (BMP) BHB insulin C-peptide free fatty acids cortisol and growth hormone ammonia and lactate urine organic acids (next voided urine) +/- if concerns for metabolic disorder - uric acid - free and total carnitine - acylcarnitine profile

Young girl has moderate acute, postprocedural pain; what would be the most appropriate medication for her?

short-acting opiate like morphine (can do IV morphine) - short-acting opiates are generally preferred for opiate-naive children with acute pain, whereas long-acting opiates like methadone are beneficial in situations of chronic, severe pain

A 7-year-old boy presents to the ED with sudden paralysis of his left upper and lower extremity and slurring of his speech. During evaluation, an MRI of the brain on diffuse weighted imaging shows restricted diffusion in the arterial territory. Magnetic resonance angiography is also performed and shows stenosis of the distal internal carotid artery and collateral vessels of the basal ganglia. What condition predisposes him to the above-described imaging findings?

sickle cell disease stroke. - Vasculitic conditions include moyamoya disease. Moyamoya means "puff of smoke" in Japanese, which is used to describe the radiological findings found on magnetic resonance angiography of either stenosis or occlusion of the distal internal carotid artery and circle of Willis and presence of collateral vessels of the basal ganglia. - The etiology of moyamoya is unknown, but it is common in people of Asian descent. Those patients with sickle cell disease have a higher risk of moyamoya disease and associated complications of transient ischemic attacks and stroke. - Generally, aspirin is initiated. If a patient has cardiac thrombotic stroke, unfractionated heparin or low molecular heparin is used. In a patient with sickle cell disease, aggressive hydration and blood transfusion is performed.

Boy in clinic has significant short stature, a normal body mass index, a concordant bone age, and a high upper-to-lower body segment ratio (U/L ratio), indicating relatively longer trunk and shorter legs. These findings are most consistent with

skeletal dysplasia - The U/L ratio can be used to assess body proportions. - The lower segment is measured from the pubic symphysis to the floor while standing. - The upper segment is calculated as height minus the lower segment. Infants have a relatively high U/L ratio (about 1.7 at birth), which gradually decreases to 1 around age 10 years, reaches a nadir during early puberty, and returns to about 0.9-1 by adulthood. - A high U/L ratio indicates a relatively longer trunk and shorter legs, and it can be seen in skeletal dysplasias and precocious puberty. - A low U/L ratio indicates a relatively shorter trunk and longer legs, and it can be seen in Klinefelter syndrome and delayed puberty. - There are many types of skeletal dysplasias. More common skeletal dysplasias include achondroplasia, hypochondroplasia, and short stature homeobox-containing gene (SHOX) deficiency, either associated with Turner syndrome or isolated.

A 3-year-old boy is receiving a health supervision evaluation. He is an only child with no significant medical history. His teacher at the preschool he has attended for 9 months mentioned that the boy rarely interacts with other children in the classroom and only engages in solo play. He becomes upset when the teacher attempts to involve him in circle time or other group activities. The boy's mother describes him as "shy." He speaks in 3- to 4-word phrases but not all of his words can be understood by a stranger. He follows complex verbal commands, can fill his water glass from a small pitcher, and draws rough circles. He cannot yet draw a figure, and when he dresses himself, he sometimes puts his shirt on backwards or his shoes on the wrong foot. He jumps, climbs, and can walk on tiptoe. He is not yet fully toilet trained. Of the following, the MOST accurate assessment of this boy's development is

social-emotional delay - The 3-year-old boy in the vignette rarely interacts with other children and only engages in solo play; his social-emotional development is delayed. Other realms of his development are age-appropriate. - Typically developing 3-year-old children can pedal a tricycle, walk on tiptoe, walk up stairs with alternating feet, catch a ball, use a fork and spoon, pour water from a small pitcher into a cup, imitate a circle, speak in 3+ word sentences with ¾ of their language understood by a stranger, follow complex commands, and engage in make-believe play and turn taking.

A 3-year-old girl is at a health supervision visit. The girl was diagnosed with bilateral grade IV vesicoureteral reflux after a urinary tract infection 6 months ago. The girl has been otherwise healthy, with normal growth parameters and development. She has been toilet trained since age 2 years. Her physical examination findings are normal. Urinalysis shows a specific gravity of 1.020, pH of 6.0, and no protein, blood, leukocyte esterase, or nitrites. Of the following, it is MOST likely that in this disorder:

spontaneous resolution is unlikely - Low grade of reflux, unilateral reflux, prenatal hydronephrosis, and diagnosis before age 1 year have been favorably associated with spontaneous resolution of vesicoureteral reflux. - Antibiotic prophylaxis is recommended for children with high-grade reflux, recurrent urinary tract infection, bladder or bowel dysfunction, or obstructive uropathy. - Bladder or bowel dysfunction leading to dysfunctional elimination is frequently seen in patients with vesicoureteral reflux and has been associated with recurrent infections, increased time until spontaneous resolution of reflux, and reduced success of endoscopic surgery.

A 12-year-old girl is seen for evaluation of possible scoliosis after her mother noticed asymmetry of the back when bending forward. The mother underwent brace treatment for scoliosis when she was an adolescent. The girl is otherwise healthy and is premenarchal. She reports no pain or other back-related symptoms. Initial inspection of the back shows slight elevation of the left shoulder. She has full and pain-free range of motion through the hips and spine, but scoliometer measurement with Adams forward bend reveals 8° elevation of the right hemithorax. Of the following, the BEST next step in management is

standing radiographs of the spine - The girl in this vignette has a curve greater than 7° on scoliometer assessment, and therefore needs radiographs for further evaluation. - Girls should be screened at age 10 years and again at 12 years. - Boys should be screened at age 13 or 14 years. - Office assessment should be performed using the Adams forward bend test to obtain scoliometer readings --> scoliometer measurement of greater than 5° to 7° is considered a positive screening result and should be evaluated further with radiographs - Bracing is generally considered for patients with scoliosis who are still growing and with curves approaching 25°

Which antifungal is first-line treatment for invasive aspergillosis?

voriconazole

What gross motor milestones do you expect in an 18-month-old?

walk unassisted help undress himself drink from a cup eat with a spoon

A 5-year-old boy who is otherwise healthy is brought to the emergency department after 7 days of fever and fatigue. He has not had cough, congestion, rhinorrhea, vomiting, or diarrhea. He appears ill but is not in distress. He has a temperature of 39°C, a heart rate of 140 beats/min, a blood pressure of 90/60 mm Hg, a respiratory rate of 40 breaths/min, and oxygen saturation of 95%. He has clear bilateral breath sounds. His heart rate and rhythm are regular. He has a grade 2/4 diastolic murmur that is not indicated in prior clinic notes. He has petechiae on his distal extremities. Blood cultures and an echocardiogram are obtained. A vegetation is noted on his mitral valve. Of the following, the pathogen that is MOST likely to be the cause of this child's infection is

staphylococcus aureus - The most common pathogens associated with infective endocarditis are streptococcus viridans group and Staphylococcus aureus. - The clinical presentation of infective endocarditis can be subacute or acute and is often nonspecific. A high index of suspicion is needed. - The modified Duke criteria can aid in the diagnosis of infective endocarditis. - Treatment initially includes broad-spectrum antibiotics that can then be tailored to the clinical situation.

A 16-year-old girl is admitted to the inpatient ward for dehydration secondary to profuse watery diarrhea. She is having over 20 stools per day, and each stool is approximately 100 mL. She is having significant abdominal cramps related to stooling. She has been afebrile and denies weight loss.The cause is unknown, and her stool culture came back negative for common bacterial and viral pathogens. Her complete blood count is normal. She has had four prior admissions in the past six months for the same reason. She has missed almost two months of school due to these admissions. What other testing should be performed in evaluation of this patient?

stool laxative screen Factitious disorder - affects single females more commonly, with higher incidence in health care workers. - Diarrhea is a common symptom that can be falsified using laxatives. - Patients will present with chronic profuse watery diarrhea with large-volume stool output and crampy abdominal pain. - Due to large water loss, patients will have symptoms of dehydration such as dizziness, lightheadedness, and orthostatic hypotension. - When suspecting factitious diarrhea, evaluation should include stool osmolality, serum electrolytes, and stool laxative screen.

Girl has Williams syndrome resulting from a contiguous gene deletion in 7q11.23 that encompasses the elastin gene (ELN). Penetrance is 100% while expression is variable. The most common cardiovascular finding in Williams syndrome is

supravalvar aortic stenosis - Other cardiovascular manifestations include elastin arteriopathy, peripheral pulmonary stenosis, and hypertension. - Distinctive facies include bitemporal narrowing, periorbital fullness, stellate irides, long philtrum, upper/lower lips with a thickened vermilion, wide mouth, small jaw, large ear lobes, and a broad forehead. - Individuals commonly possess a specific neurocognitive profile with overfriendliness, generalized anxiety, attention-deficit/hyperactivity disorder, strengths in verbal short-term memory and language, and weakness in visual-spatial construction. Most have intellectual disability, typically mild. - Growth abnormalities are characterized by prenatal growth deficiency and slow weight gain and linear growth in the first 4 years, and a brief pubertal growth spurt resulting in short stature. - Endocrine abnormalities can include idiopathic hypercalciuria and hypothyroidism. - The diagnosis of Williams syndrome is confirmed via chromosomal microarray or targeted deletion analysis using fluorescence in situ hybridization.

Neonate experiencing narrow complex, regular tachycardia and has poor perfusion. Therefore, the treatment of choice is

synchronized cardioversion

What term describes the emerging model of health that examines the synergistic manner by which socio-political environments contribute to disease clustering within individuals and groups?

syndemics

A 2-year-old boy is admitted to the hospital for the evaluation and treatment of suspected septic arthritis of his right knee. A blood culture specimen is obtained, synovial fluid sample is collected by joint aspiration, and he is started on appropriate empiric antibiotic therapy. His clinical symptoms improve. The clinician would like to narrow the empiric antimicrobial treatment, but the synovial fluid culture has no bacterial growth. Of the following, the test MOST likely to confirm the etiology of this boy's infection is

synovial fluid pathogen-specific polymerase chain reaction - The toddler in the vignette has septic arthritis of the knee with negative synovial fluid culture findings. In this scenario, Kingella kingae must be considered as a likely pathogen. K kingae is difficult to isolate on typical culture media, but polymerase chain reaction assays can improve the diagnostic yield. If PCR is unavailable, coverage for Kingella should be considered in cases of culture-negative septic arthritis in children younger than 4 years. - Kingella kingae is a gram-negative organism of the Neisseriaceae family that may colonize the posterior pharynx of healthy children and be transmitted through close contact. Infections most commonly occur in infants and toddlers, and typically present as arthritis, osteomyelitis, or bacteremia. K kingae arthritis most commonly occurs in the knee, hip, or ankle. - Ampicillin-sulbactam or a first-, second-, or third-generation cephalosporin can be used to treat K kingae bone or joint infections.

minute ventilation

tidal volume x respiratory rate

UAC courses from...

umbilicus --> down right or left umbilical artery --> to ipsilateral iliac artery --> up to aorta - acceptable positions = high in distal thoracic or upper abdominal aorta above T12 but below T4 (great vessel takeoff) or low below renal arteries - ideal position = between T5 and T8

BXO is a complication of

unrepaired hypospadias -sclerosing - affects foreskin and glans of penis - breakdown of skin --> plaques coalesce into sclerosing mass = urethral strictures, changes in urinary stream, sexual dysfunction

Superior mesenteric artery syndrome is diagnosed with

upper gastrointestinal series - demonstrating an obstruction in the third portion of the duodenum with contrast "to and fro" flow present

A 6-year-old boy with ear drainage is brought to the otolaryngology clinic. Four weeks ago he underwent bilateral tympanostomy tube placement for recurrent otitis media. For the last 2 weeks, he has had persistent foul-smelling drainage from the right ear. He has a temperature of 37.2°C, blood pressure of 100/60 mm Hg, heart rate of 90 beats/min, and respiratory rate of 22 breaths/min. There is purulent drainage in the right ear canal, and the right tympanic membrane cannot be visualized. A patent tympanostomy tube is seen in the left tympanic membrane. Of the following, the BEST management of this boy's infection is

topical ofloxacin - Certain pathogens, including Pseudomonas aeruginosa and Staphylococcus aureus, must be considered as the causative agents of tympanostomy tube otorrhea in older children when water penetration could have contributed to developing otorrhea. Thus, empiric therapy should have broad-spectrum activity. - For tympanostomy tube otorrhea, topical therapy is more efficacious than systemic therapy. Therefore, of the agents and routes presented as therapeutic options, topical ofloxacin is preferred.

Most common cyanotic heart dz manifesting in first wk after birth

transposition of great arteries - 2nd most common cyanotic CHD - present with cyanosis within first 12 hrs after birth & are not responsive to oxygen or mechanical ventilation - presence of VSD may delay presentation

What type of formula may be implemented in age-appropriate preterm infants with signs of severe reflux?

trial of extensively hydrolyzed protein-based - hypoallergenic formulas with extensively hydrolyzed protein are effective for the treatment of milk protein allergy and the prevention of atopic disease in high-risk infants

A 10-year-old boy is seen for evaluation of left heel pain. He was previously healthy until the onset of pain several months ago, shortly after the beginning of his soccer season. The pain initially occurred during and after running, but it is now present throughout the day and is associated with a limp. The boy and his mother report no traumatic injury, and there has been no associated swelling, bruising, or other musculoskeletal concerns. The pain improved significantly during a 1-week break from soccer, but it recurred within several days of return to practice. He has focal tenderness over the medial and lateral aspects of his left heel and prefers to walk on his toes. The remainder of his physical examination findings are unremarkable. Of the following, the BEST next step in his care is

trial of heel cups or cushions Sever apophysitis - Initial treatment should focus on protecting the injured calcaneal growth plate with a trial of heel cushions. - Application of ice for 10 to 15 minutes several times per day and use of over-the-counter analgesics as needed for several days can also be helpful. - Stress reaction through the cartilaginous calcaneal apophysis. It can occur in any child who is skeletally immature but is most common in pre-adolescent boys participating in running sports. The calcaneal apophysis is subject to multiple stressors in young athletes - During Achilles assessment, growth patterns should be queried as traction from the Achilles tendon often increases during periods of rapid linear growth. - The patient should be able to achieve at least 10° of foot dorsiflexion, and it is not uncommon for males who are growing rapidly to lack this degree of dorsiflexion.

A 13 yr old girl presents with a 1-wk history of fever, myalgias, and periorbital edema. She emigrated with her family from a rural area of China to the United States 4 wk ago. A communal meal that included a locally butchered pig was part of the family's farewell party. Her 16 yr old brother had similar symptoms, which have now resolved. The most likely cause of the patient's condition is:

trichinosis - caused by consumption of undercooked meat containing encysted larvae of Trichinella spiralis, a tissue-dwelling nematode with a worldwide distribution - development of symptoms depends on the number of viable larvae ingested - watery diarrhea is the most common symptom corresponding to maturation of the adult worms in the gastrointestinal tract, which occurs during the 1st 1-2 wk after ingestion - patients may also complain of abdominal discomfort and vomiting - fulminant enteritis may develop in individuals with extremely high worm burdens - the classic symptoms of facial and periorbital edema, fever, weakness, malaise, and myalgia peak about 2-3 wk after the infected meat is ingested as the larvae migrate and then encyst in the muscle

A sexually active adolescent girl presents with copious malodorous gray vaginal discharge with vulvovaginal irritation. The most likely etiologic agent is:

trichomonas vaginalis - Common signs and symptoms of trichomoniasis include a copious malodorous gray, frothy vaginal discharge, vulvovaginal irritation, dysuria, and dyspareunia. - Physical examination may reveal a frothy discharge with vaginal erythema and cervical hemorrhages ("strawberry cervix"). - The discharge usually has a pH of > 4.5. Abdominal discomfort is unusual and should prompt evaluation for pelvic inflammatory disease.

A child who has recently returned from a visit to a developing country experiences a 2-wk illness characterized by gradually increasing fever with temperature that eventually reaches 104°F, associated with headache, malaise, cough, and abdominal pain. The most likely diagnosis is:

typhoid fever - The incubation period of typhoid fever is usually 7-14 days but depends on the infecting dose and ranges between 3 and 30 days. - Typhoid fever usually manifests as high-grade fever with a wide variety of associated features, such as generalized myalgia, abdominal pain, hepatosplenomegaly, abdominal pain, and anorexia. - The fever may rise gradually, but the classic stepladder rise of fever is relatively rare. - In about 25% of cases, a macular or maculopapular rash (rose spots) may be visible around the 7th-10th day of the illness, and lesions may appear in crops of 10-15 on the lower chest and abdomen and last 2-3 days.

A neonate with a murmur is being evaluated in the newborn nursery. The neonate appears to be in no distress. Her heart rate is 150 beats/min, respiratory rate is 40 breaths/min, blood pressure is 75/45 mm Hg, and oxygen saturation is 95% in room air. She is breathing comfortably, aerating well, and is warm and well-perfused. Her liver is palpable 3 cm below the right costal margin. There is a normal S1 and S2 with a 3/6 harsh systolic murmur heard best at the left upper sternal border. Echocardiography demonstrates severe pulmonary stenosis. Of the following, the BEST next step in management is

urgent referral to cardiology for cardiac catheterization - Neonates with severe pulmonary stenosis should urgently undergo balloon valvuloplasty, and therefore be transferred as soon as possible to a center that performs such procedures regularly. - With severe pulmonary stenosis, the right ventricle can ultimately fail because of the increased work needed for pulmonary blood flow in the context of a high pressure gradient. - This neonate's oxygen saturations are normal, which is not consistent with critical pulmonary stenosis.

An 8-month-old girl is being evaluated for a droopy left eyelid. In addition, her left pupil has become noticeably smaller than the right. Her parents are unsure of when these symptoms began. She has no history of fever, poor feeding, or recent illness. She was born via an uncomplicated vaginal delivery at term and has been otherwise healthy. The girl has been growing and developing normally. Physical examination findings are significant for left ptosis and pupillary anisocoria, with left miosis and preservation of response to light. The remainder of her physical examination findings are normal. Of the following, the MOST likely laboratory abnormality(ies) in this infant is(are)

urinary homovanillic and vanillylmandelic acid levels - The infant has acquired left ptosis in the setting of Horner syndrome (unilateral ptosis, miosis, and anhidrosis) because of neuroblastoma. The tumor arises from primitive sympathetic ganglion cells that have the capacity to synthesize and secrete catecholamines. - Neuroblastoma may be diagnosed by measuring urinary homovanillic (HVA) and vanillylmandelic acid (VMA) levels, which are catecholamine metabolites secreted by neuroblastoma cells; these are elevated in 90% to 95% of affected children. - Neuroblastomas that involve the cervical paravertebral sympathetic chain and inferior cervical (stellate) ganglion may result in Horner syndrome, though anhidrosis may be absent or unnoticeable. - Horner syndrome is caused by a disruption in the 3-neuron oculosympathetic chain. - Young children with isolated Horner syndrome should undergo careful examination for cervical and abdominal masses, measurement of urinary HVA and VMA levels, and radiologic imaging of the head, neck, and chest.

A 14-year-old girl is being evaluated for concern about evolving seizures. She has a history of partial complex seizure disorder, which has been well-controlled with medication. She recently had a prolonged episode of abnormal motor movements that included backward arching of her head, neck, and back, and pelvic thrusting. Her eyes were closed tightly during the episode, but she became alert quickly when the movements subsided. The adolescent also has an anxiety disorder. She has been diligent about taking her antiepileptic and antianxiety medications as prescribed. She has otherwise been well. She has a family history of epilepsy, hypertension, anxiety, and diabetes, and a cousin who recently died of meningitis. On physical examination, her vital signs are within normal limits. The remainder of her physical and neurologic examination findings are normal. Of the following, the test or study MOST likely to reveal this girl's diagnosis is

vEEG - The girl in the vignette is demonstrating behaviors consistent with psychogenic nonepileptic seizure (PNES). - Video electroencephalography (EEG) is the gold standard test for this diagnosis and will have normal findings at the time of the apparent seizure behavior. - Forward pelvic thrusting, side-to-side head or body movements, closed eyes resistant to opening, and a lack of postictal confusion are findings suggestive of psychogenic nonepileptic seizures rather than epileptic seizures. - Neurologic disorder (eg, epilepsy), panic disorder, and dissociative disorder may co-occur with conversion disorder. Patients with epileptic seizures are more likely to have psychogenic nonepileptic seizures.

A 16 yr old female high school student is seen for pretravel counseling. Departing next month with a group of classmates, she will spend 7 days on a bird-watching excursion in the amazonian portion of Peru. Your recommendation with regard to travel immunization is:

vaccination against yellow fever - Yellow fever 17D is a live-attenuated vaccine with a long record of safety and efficacy. - It is administered as a single 0.5-mL sub cutaneous injection at least 10 days before arrival in a yellow fever-endemic area. - Residence in or travel to areas of known or anticipated yellow fever activity (e.g., forested areas in the Amazon basin), which puts an individual at high risk, warrants immunization.

VACTERL association, formerly known as VATER association, stands for

vertebral defects (V) anal atresia (A) cardiac defects (C) tracheoesophageal fistula (TE) renal defects (R) limb defects (L) - Diagnosis of VACTERL is established when at least three of these features are present. - VACTERL association is a diagnosis of exclusion

You are reviewing the medical records of a patient who is entering your practice. This 2-week-old male newborn was born at term to a 20-year-old, gravida 1 para 1 mother who did not receive prenatal care. At delivery, the baby was found to have micrognathia, glossoptosis, and a cleft palate consistent with Pierre Robin sequence. He was cared for in the neonatal intensive care unit, and at the time of discharge he was being fed breast milk through a nasogastric tube. Notes report that he is able to breathe comfortably and maintain adequate oxygen saturation when he is positioned prone or on his side. Genetics consultation and testing during his hospital stay confirm Stickler syndrome. Of the following, the condition MOST commonly associated with this syndrome, other than those conditions already noted, is

vision impairment Stickler syndrome - group of genetic conditions characterized by the Pierre Robin sequence (cleft palate, glossoptosis, and micrognathia or retrognathia) and severe myopia or other ocular abnormalities. - Many patients with Stickler syndrome also have sensorineural hearing loss or skeletal abnormalities including hypermobility, scoliosis, or early arthritis.

A 14-year-old girl presents to your clinic for a pre-sports participation evaluation. She is planning on playing on her high school's junior varsity soccer team this autumn and will be having four one-hour practices per week with the soccer team. You note that she has an elevated body mass index on her growth charts. Of the following oral hydration regimens, which should you recommend for her during each practice period to prevent dehydration?

water - To offset insensible fluid loss, it is recommended that 9 to 12-year-olds consume about 3 to 8 ounces of fluid every 20 minutes and that adolescents consume about 34 to 50 ounces every hour before, during, and after physical activities.

The risk of reherniation in congenital diaphragmatic hernia varies from 30% to 50%. Having a large diaphragmatic defect repaired with a prosthetic patch is associated with increased rates of recurrent herniation. Affected children should be screened how?

with chest radiography annually until age 16 years - Infants whose congenital diaphragmatic hernia was repaired with a patch are at increased risk for recurrent intestinal herniation. - Left-sided CDH is more common than right. - Although most cases of CDH are isolated, it has been associated with genetic syndromes such as trisomies 21, 18, and 13, and Fryns, Pallister-Killian, and Turner syndromes. - Most neonates will undergo surgical repair within 5 to 10 days after birth once pulmonary vascular resistance has decreased. - Extracorporeal membrane oxygenation (ECMO) may be needed before or after surgical repair. - Growth failure is common for infants and children after CDH repair, particularly in the first 3 years of life. Possible causes include delayed introduction of enteral feedings and persistent lung disease. These children often do not feed well by mouth. Approximately one-third of infants who have undergone CDH repair will be discharged from the hospital with nasogastric or gastrostomy tube feedings.

An adolescent in rural New Mexico living in a household with domestic cats that roam freely presents with fever and chills of sudden onset and extraordinarily painful lymphadenopathy. He appears toxic. The most likely etiology is:

yersinia pestis infection - Plague is uncommon in the USA (0-40 reported cases/yr); most of these cases occur west of a line from east Texas to east Montana, with 80% of cases in New Mexico, Arizona, and Colorado. - Bubonic plague is the most common form and accounts for 80-90% of cases in the USA. - From 2-8 days after a flea bite, lymphadenitis develops in lymph nodes closest to the inoculation site, including the inguinal (most common), axillary, or cervical region. - These buboes are remarkable for tenderness. - Fever, chills, weakness, prostration, headache, and the development of septicemia are common.