RX pediatrics

when can a kid tie shoes?

5 yo - copy triangle, print name. A 4-year-old child is able to hop, skip, draw crosses and squares, count to ten, and sing songs or recite poems from memory.

Rule of 2's in Meckel's Diverticulum

2 ft from ileocecal valve, 2 cm long, 2 types mucosa (gastric/ pancreatitc), 2% population, usually presents at 2 yo.. use technetium pertechnetate to dx.

congenital hypothryoidism

constipation, umbilical hernia, prolonged jaundice, FTT, OPEN (large) fontanelle's.. 2x more common in females. - can lead to severe mental retardation, neurological issue sand growth failure..

criteria for physiologic jaundice

(1) the bilirubin is <14 mg/dL; (2) the bilirubin increases by <5 mg/dL per day; (3) during the first 3 days the bilirubin is <5 mg/dL (the level at which jaundice is first apparent); (4) at 7 days in a full-term infant and by 14 days in a preterm infant, the jaundice has resolved, and the bilirubin is <2 mg/dL; and (5) the conjugated bilirubin is always normal.

CT before lumbar puncture is indicated to exclude a mass lesion or increased intracranial pressure if a patient has one or more of the following risk factor... need to do LP eventually if you think it is meningitis.

- Immunocompromised state (eg, HIV or immunosuppressive therapy)- Hx of CNS disease (eg, stroke, masses)- New onset seizure- Papilledema- Altered level of consciousness- FND If LP is delayed, blood cultures should be obtained and antibiotics administered. In the clinical vignette, the child has papilledema. Therefore, a CT scan should be performed prior to LP

infections in CF patient that cause pneumonia

0-mid 20's (young): S. Aureus is most common Mid 20's and older: Pseudomonas is most common

21 hydroxylase deficiency is the most common CAH!!- no aldosterone or cortisol is made shunts everything to DHEA and T and DHT and E2 production- so would expect hypoTN, hyperK+ - in XX: would notice because would have really high T levels!! Virilized genitalia (good warns you something is wrong) - in XY: might not notice may have precocious puberty.. Boy's get sent home aldosterone deficiency -> really dehydrated -> ill (severe vomiting)- the low cortisol -> lot of ACTH secreted (from pituitary)

11 B-hydroxylase deficiency- no aldosterone and no cortisol produced - but the precursor to those = those 11-deoxycorticosone things are mineralocorticoids - HTN, and hypOK+ (mineralocorticoid excess)- virialized xx- precocious puberty in XY- still high T - still high ACTH 17 alpha-hydroxylase deficiency- really high aldosterone!!- note that 17 alpha hydroxylase is involved in both cortisol and androgen synthesis (but not aldosterone! #$%#$% IMPOTANT) - will have low cortisol- low DHEA, T, and E2- high ACTH (low cortisol) - would have HTN and hypoK+ - this will end up w/ boys w/ ambiguous genitalia (low T and DHEA and stuff) - girls only have sexual side effects of 17 alpha hydroxylase deficiency at puberty may not be able to get enough E2 to -> secondary sex characteristics

The young patient presents with acne refractory to treatment, hirsutism, and elevated 17-hydroxyprogesterone. This suggests a diagnosis of late-onset congenital adrenal hyperplasia, an autosomal recessive condition characterized by impaired synthesis of cortisol.

21-hydroxylase deficiency CAH can present in two ways. For this patient, a late-onset form manifests after the neonatal period with signs of hyperandrogenism (medication-refractory cystic acne, premature pubarche, early fusion of epiphyseal plates, irregular menses), without adrenal insufficiency. This distinction is important because the clinical presentations are different. A serum 17-hydroxyprogesterone greater than 200 ng/dL (6 nmol/L) strongly suggests the diagnosis of late-onset 21-hydroxylase deficiency. This patient does not have virilizing features, nor are her blood pressure and potassium level consistent with this diagnosis.

Simple febrile seizures are defined as generalized seizures that last less than 15 minutes and do not recur in a 24-hour period.

30% of kids w/ simple febrile seizures will have subsequent febrile seizures

when to wave bye bye and 2-3 word vocab of like mama

9-10 mo.. walk using exam table to supper himself 12 mo kid has a bigger vocab than mama.. independent steps

hemophilia A vs B

A = factor 8 which is way more common 1/5,000 compared to B = factor 9 deficiency which is 1/30,000 - both x-linked

With the Valsalva maneuver, there is a DECREASE in VENOUS RETURN, right and left ventricular volumes, stroke volume, and a decrease in arterial and pulse pressure. As a result, all flow murmurs decrease (aortic stenosis, pulmonary stenosis, mitral regurgitation, tricuspid regurgitation, aortic regurgitation, pulmonary regurgitation, mitral stenosis, and tricuspid stenosis). The only murmur that increases in intensity is the murmur of hypertrophic cardiomyopathy.

A change in position from supine or squatting to a standing position causes a DECREASE in VENOUS return to the heart. As a result, there is a decrease in right and left ventricular diastolic volumes, decrease in stroke volume, decrease in arterial pressure, and reflex increase in heart rate. This decreases the murmurs of pulmonary stenosis, aortic stenosis, mitral regurgitation, tricuspid regurgitation, and ventricular septal defect. The only murmur that increases in intensity with standing is the systolic ejection murmur of hypertrophic obstructive cardiomyopathy (HOCM). The murmur of HOCM increases as a result of increased obstruction to the aortic outflow tract. This is often a useful technique for diagnosing HOCM.

when do we use skeletal survey? (xray overview of entire skeleton)

A skeletal survey is mandatory for all patients <2 years old in whom physical abuse is suspected. A skeletal survey would also detect femoral fractures, making a skeletal survey a better answer than just imaging of the femurs.

Mineralcorticoids = aldosterone Glucocorticoids = cortisol Adrenal steroids and CAH (congenital adrneal hyperplasia) 17-alpha hydroxylase is needed to create DHEA, T, and E2 2 enzymes to get to aldosterone and cortisol for both - 21-hydroxylase - 11B-hydroxylase The stuff between 21 and 11 (11-deoxycorticosterone, 11-deoxycorticosterone stuff that sounds like cortisol a little bit does not act like cortisol but acts like mineralcoritcods = acts like ALDOSTERONE a little bit)

ACTH more responsible for cortisol synthesis and RAAS (angiotensin 2) is more responsible for aldosterone synthesis CAH involves partial deficiency in one of the steps used to make cortisol.. Low cortisol -> high ACTH!! Aldosterone increases BP and decreases K+ Layers G F R: - glomerulosa: aldosterone made - Fasciculata: cortisol made - Reticularis: T and E2 made Aromatase inhibitors prevent T -> E2 (-anastrozole and exemestane) - tamoxifen and raloxifene block the ER - these prevent breast cancer! 5 alpha reductase inhibitor can prevent DHT formation = treat BPH - but for prostate cancer use flutamide = AR blocker! #$%#$% (androgen receptor) Ketakonazole: inhibits ergosterol in fungal cell wall: inhibits other steps in pathway especially steps related to androgen synthesis.. Can lead to loss of T levels in men! #$%#$%

urine: - RBC 2-5 hpf, no RBC cast, protein 1+ isn't enough for like PSGN nephritis needs RBC cast and higher RBC and higher protein in urine..

Acute interstitial nephritis = allergic inflammatory rx w/in kidney interstitium -> acute kidney injury.. can be caused by drugs, infections, autoimmune/ systemic diseases Cr elevated and urine WBC elevated! #$%% This 10-year-old has fever and morbilliform rash on her chest and back after being treated with antibiotics (amoxicillin) for streptococcal pharyngitis. Her creatinine level is elevated and her urine shows the presence of WBCs. These findings are typical of drug-induced acute interstitial nephritis (AIN). AIN is a renal lesion that typically causes a decline in renal function and is characterized by an inflammatory infiltrate in the kidney interstitium. The classic clinical presentation of patients with drug-induced AIN is a triad of fever, rash, and flank pain along with acute kidney injury. The presence of eosinophils in the urine and increased numbers in circulation are classic findings in such patients, although they are not always present in cases of AIN. In terms of etiology, medications cause the majority of AIN cases, with antibiotics (especially β-lactams) responsible for 30%-50% of the incidences. Nonsteroidal anti-inflammatory drugs (NSAIDs), diuretics, allopurinol, proton pump inhibitors (PPIs), indinavir, and 5-aminosalicylates are also associated with AIN. The two other main causes of AIN include infections (many organisms have been linked with AIN) and autoimmune and systemic disorders, especially systemic erythematosus lupus (SLE), sarcoidosis, and Sjogren syndrome. The main causes of AIN can be remembered using the mnemonic AIN: Autoimmune and systemic diseases; Infections; NSAIDs, antibiotics, and other drugs. Management usually involves addressing the underlying cause such as immediately stopping the offending drug or treating the infection. If AIN still persists or is particularly severe, systemic glucocorticoids may be used for immunosuppression and resolution of inflammation.

when to use Acyclovir in chicken pox?

Acyclovir is recommended in individuals with varicella >13 years old, children >12 months old with complicating conditions, and patients with severe or disseminated infection. Supportive therapy only would be appropriate in uncomplicated cases of this infection in young children. However, this patient's age > 13 years puts her at a higher risk for complications and therefore necessitates treatment in addition to supportive care.

The child in question has the classic triad of West syndrome: - infantile spasms, regression of psychomotor development, and hypsarrhythmia on ECG

Although her spasms will eventually regress, she is likely to be neurologically impaired - They are often followed by the appearance of other seizure disorders and/or neurologic impairment.

A Dandy-Walker malformation is a cystic expansion of the fourth ventricle along with hydrocephalus. Patients often have other associated cerebral anomalies. This is a congenital anomaly that would have been apparent at birth. Patient present with developmental delays, poor muscle tone, poor balance and coordination, visual impairments, and sometimes seizures.

An Arnold-Chiari malformation is a congenital herniation of the cerebellar tonsils (and possibly other parts of the brain) into the cervical canal. It is much less common than primary brain tumor and would present with a different clinical picture. Type I Arnold-Chiari malformations are often asymptomatic until adulthood but may be associated with HA with coughing and hx of LOC when looking upward. The severity and type of symptoms present depend on the degree of tonsillar herniation. Herniations >12 mm are almost always symptomatic.

Apgar

Apgar scores are based on five factors: Appearance (2 = all pink, 1 = pink body/blue extremities, 0 = blue/pale) Pulse (2 = >100/min, 1 = <100/min, 0 = absent), Grimace (2 = sneeze/cough to stimulation, 1 = grimace to stimulation, 0 = absent) Activity (2 = active and spontaneous movement, 1 = some extremity flexion, 0 = limp) Respiration (2 = normal rate and effort, 1 = slow and irregular, 0 = absent)

_________ is associated w/ the following; hemihyperplasia (enlargement of one side of body), organomegaly, perinatal growth acceleration, macroglossia, linear ear creases, and transient neonatal hypoglycemia

Beckwith-Wiedenmann syndrome.. - ass w/ increased risk of WILM'S tumor! #$%

patients who begin on a normal growth curve and subsequently fall off have attenuated growth = pathologic.. what is initial test to see what etilogy of delayed growth =?

Bone age scan.. help tell between familial and acquired cases. Causes of attenuated growth include renal disease, hypothyroidism, Crohn disease, cancer, glucocorticoid therapy, and growth hormone deficiency (GHD). The initial work-up for attenuated growth is to determine bone age, with a bone age scan. Specifically, a basic evaluation consisting of a bone age determination is appropriate for children with short stature, normal growth rate (eg, height velocity [HV] at least 5 cm/year between 4 and 6 years of age, and at least 4 cm/year between 6 years of age and puberty), and no other symptoms. Broader testing may be warranted if the child is severely short (eg, height ≤-2.5 standard deviations [SD, 0.6th percentile]), has growth failure (eg, height-for-age curve crossing two major percentile lines, or HV <5 cm/year between 4 and 6 years of age, and <4 cm/year between 6 years and puberty), or if the history or physical examination raises suspicion for a specific systemic, endocrine, or genetic disorder.

if someone has bronchiolitis what do you do?

Bronchiolitis is a clinical diagnosis and is generally a self-limiting illness, requiring only supportive treatments - A respiratory viral PCR panel can be useful in establishing the diagnosis of lower respiratory tract infections, including influenza A and B, parainfluenza, and RSV (can also cause pneumonia). It is not needed to establish the diagnosis and will not help to change management in this case. Bronchiolitis is a clinical diagnosis. Bronchiolitis is typically caused by a viral infection. While it varies depending on the season and year, respiratory syncytial virus (RSV) is the most common cause, accounting for about 50% of cases. Less common causes include parainfluenza virus, influenza virus, adenovirus, and coronavirus. - Further imaging, such as a neck x-ray or CT scan, and additional tests, such as a nasopharyngeal swab or viral PCR panel, are NOT NOT NOT needed to make the diagnosis.

Jaw facial bone mass young kid

Burkitt lymphoma is a high grade, B-cell neoplasm that is more common in children and young adults. It has a ″starry sky″ appearance on histology and is associated with translocations involving the ^~€#% c-myc gene on chromosome 8. %{^~!{ The endemic form, most prevalent in African children, is generally associated with an initial finding of jaw or facial bone masses, while the sporadic form most often has an abdominal mass or masses as an initial finding. There are three separate forms: endemic (African), sporadic, and immunodeficiency-associated subtypes (HIV or post-transplantation) Endemic form ass w/ EBV Burkitt lymphoma is also associated with the t(8:14) translocation of the c-myc gene.

presentation of congenital adrenal hyperplasia (steroidogenic pathway defective) compared to like CF?

CAH presents w/ in first days-wks after birth.. CF can present later in like 5 mo. CF is an autosomal recessive disease caused by a defect in the CFTR gene, resulting in abnormalities in the CAMP-regulated chloride transport across epithelial cells on mucosal surface - most men w/ CF have absent vas deferens = infertile, absence of sperm in ejaculate.. - CF; childhood FTT (LOW WEIGHT), bulky foul smelling stools (Pancreatic insufficiency), GI symptoms mostly when infant and then more pulmonary symptoms later in life predominant... so when 5 mo lungs = clear to auscultation! #$%#$% - uncle w/ chronic cough who has always been thin = CF! #$%

CEA vs CA 19-9

CEA used for colon CA follow up not dx (monitor recurrence) CA 19-9: pancreatic and biliary tract CA. (monitor recurrence)

immunodeficiency w/ recurrent abscesses

CGD

What immunodeficiencies do we use BMT for (bone marrow transplant) and what ones can we not do that for?

Can use BMT for: WAS (wiskott aldrich syndrome) and CGD (chronic granulomatous disease) Can't use BMT for: X-linked agammaglobulinemia (Bruton's).. for this one instead just give IVIG,.. - this has mutation in BTK gene on X-chromosome so pre-B cells can't mature into mature B lymphocytes, plasma cells and produce Ab. - Bruton agammaglobulinemia is an X-linked inherited immune deficiency characterized by repeated pyogenic infections, low levels of immunoglobulins, and an absence of B lymphocytes. It usually occurs in infants at the age of 6 to 9 months, and treatment consists of regular administration of intravenous immunoglobulin and prophylactic antibiotics.

Tuberous sclerosis is a multisystem disease commonly presenting with skin lesions, malar angiofibromas, hypopigmented areas, and benign tumors of the CNS leading to seizures and mental retardation. what happens to heart and kidneys?

Cardiac rhabdomyomas and the angiomyolipomas of the kidney, liver, or adrenal glands are also common with proliferation of spindle cells, epithelioid cells, and adipocytic cells in concert with many thick-walled blood vessels. kidney angiomyolipomas = epithelioid cells, adipocytes, blood vessels.

This patient presents with a 4-day history of rhinorrhea and fever progressing to a loud cough and difficulty breathing. On physical exam she is tachypneic and has increased work of breathing as evidenced by intercostal retractions. Stridor is heard on auscultation and the x-ray demonstrates significant narrowing of the hypopharynx with extensive soft tissue fullness in the prevertebral/pharyngeal mucosal soft tissues.

Croup is an acute inflammatory disease of the larynx, most commonly caused by parainfluenza type 1 infection. Patients are typically 6 months to 3 years old and experience a prodrome of upper respiratory infection symptoms followed by low-grade fever, mild dyspnea, inspiratory stridor, and a characteristic barking cough. Though not necessary for the diagnosis, an anteroposterior film of the neck would demonstrate subglottic narrowing (ie, "steeple" sign), as seen in this patient. While complications from croup are rare, one of the possible complications is a bacterial superinfection causing a tracheitis or a bacterial pneumonia. - Staphylococcus aureusis the most common pathogen associated with secondary infection following croup.

flat facial profile, prominent epicanthal folds, upslanting palpebral fissure, clinodactyly (curved finger/s toes), simian crease, and congenital heart disease

Down's syndrome: Tripple screen done 15-18 wks old - Low levels of E2, low maternal serum AFP, high β-hCG levels could indicate Down syndrome in a fetus. - increased nuchal translucency - decreased PAPP-A Quad screen: 3 markers + inhibin A.. inhibin A is elevated in Down's syndrome and unchanged in 13 and 18

This patient has excessive gingival bleeding after losing his first tooth and also has new onset of bruises on his body. This presentation is consistent with von Willebrand disease. Von Willebrand disease is the most common inherited bleeding disorder. It is inherited in an autosomal dominant fashion and affects 1% of the population. Patients present with easy bruising, bleeding from mucosal surfaces (eg, the oropharynx, uterus, and gastrointestinal tract), and post-incisional bleeding.

Elevated PTT and bleeding time

presents w/ renal failure

Fabry's disease

what are you worried about in exclusively cow's milk fed patient?

Fe deficiency (there is little iron in cow's milk) can lead to IDA

Absence seizures appear as staring spells lasting 5-10 seconds. On an electroencephalogram, they show classic 3-per-second spike-and-wave discharges. Ethosuximide is not an appropriate therapy for this patient's condition. 8yo girl She first complained of stomach pain and soon afterward (within 30 seconds), her lips started smacking and her left eye started twitching. This whole episode lasted for about 4 minutes. During that time, the girl was unresponsive but did not lose consciousness. There is no history of head trauma or seizures. Her mother had seizures that resolved in puberty and is distraught about her daughter's seizure. The patient's temperature is 37.8°C (100.0°F), pulse is 70/min, respiratory rate is 22/min, and blood pressure is 90/60 mm Hg. She is drowsy but is able to be aroused. Her neck is supple, and the remainder of the neurologic examination shows no focal findings. A complete metabolic panel (complete blood cell count and liver function tests) reveals no abnormalities except for a slightly elevated prolactin level. A CT scan of the head with contrast shows no abnormalities. An electroencephalogram shows slow waves throughout, with sharp waves over the left temporal region.

Febrile seizures are unlikely to occur in children >6 years old. In a pediatric patient whose neurologic exam shows no focal findings and who has no evidence of meningitis, a LP is NOT indicated, antibiotics are not warranted, and anticonvulsant therapy may be initiated. &($(&&; So tx w/ carbamazepine, not ethosuximide this is not absence seizure

person w/ ESRD has highK+ what do we do to treat?

First thing to do is to give Calcoium gluconate!! (antagonizes the membrane actions of hyperK+) then can do hemodyalisis if need to later Hemodialysis is one of the definitive ways of removing potassium from the body. However, because it takes significantly longer to initiate than intravenous calcium, hemodialysis is not the preferred next step in management.

how to treat Lyme's disease in normal people, those under 8 yo and those who are pregnant?

First-line treatment for Lyme disease is doxycycline, except in pregnant women and children younger than 8 years old, who should be treated with amoxicillin.

What is a genetic cause of hypertrophic cardiomyopathy?

Friedreich ataxia is an AR disorder thought to result in mitochondrial iron deposition. It typically causes damage in the dorsal column of the spinal cord, the heart, and the pancreas, resulting in ataxia, hypertrophic cardiomyopathy, and diabetes. There is no known effective treatment and life expectancy is between 30 and 40 years.

what is most common cause of meningitis 0-3 mo?

GBS.. E coli = #2 and Listeria = #3 strep pneumo is MC in 18-60 yo

what leads to elevated AFP??

HCC and various non-seminomatous testicular germ cell cancer = teratomas, yolk sac tumors, embryonal cell tumors..

HLA-B27 is strongly associated with a number of inflammatory conditions including ankylosing spondylitis, IBD, enteropathic arthritis, psoriatic arthritis, and reactive arthritis. PAIR HLA-B51 is associated with Behçet syndrome, a rare autoimmune inflammatory disease that may manifest as sores anywhere along the alimentary canal or genitals, eye swelling, and joint pain. HLA-DR2 is associated with Goodpasture syndrome, in which autoantibodies attack basement membranes found in the kidneys and lungs. HLA-DR2 is also associated with MS and SLE HLA-DR5 is associated with Hashimoto disease and pernicious anemia. Hashimoto disease is manifested by fatigue, weight gain, and irregular menstruation, among other signs and symptoms. Pernicious anemia may cause a multitude of signs and symptoms including macrocytic anemia, fatigue, pallor, neuropathic pain, diarrhea, angular cheilitis, and glossitis. what is type I DM?

HLA-DR3 - also has SLE and Graves disease

11B-hydroxylase deficiency

HTN and hypoK+, has high T so ambiguous genitals for female! #$%

can cause FTT, mental retardation, osteoporosis, megaloblastic anemia, lens dislocation, and most importantly, thromboembolic events (DVT, pulmonary emboli, and cerebrovascular accidents).

Homocystinuria results from a defect in cystathionine or methionine synthesis, leading to accumulation of homocysteine - Homocystinuria can lead to failure to thrive, mental retardation, osteoporosis, megaloblastic anemia, lens dislocation, and thromboembolic events. Other heritable causes of hypercoagulable states include factor V Leiden mutation, protein C deficiency, protein S deficiency, antithrombin III deficiency, and defects in fibrinolysis.

All the hydroxylase deficiencies - what causes hyper K+? what causes high aldosterone?

HyperK+ 21 Aldosterone high 17 (cortisol and T and E involved w/ 17 synthesis) Low cortisol and high ACTH all of them! Low T and E - 17 Remember 11 -> deoxycorticosol (acts more like aldosterone than cortisol)

talk about TE fistula Xray babies present with excessive secretions that cause frothing at the mouth and nose, drooling, choking, cyanosis, and respiratory distress immediately after birth.

If the trachea is connected to the distal esophagus, gastric distention can occur. The symptoms of respiratory distress are exacerbated by feeding. In the presence of EA, a naso- or orogastric tube can be passed only 10-15 cm down the airway and cannot be passed into the stomach. With a distal TEF, a distended gas-filled stomach and duodenum are seen on x-ray of the chest, and the TEF may be seen on a lateral view. Fluoroscopy can confirm the diagnosis of EA.

when do we need to use IV acyclovir?

If you have varicella pneumonia (complication of varicella) need to treat w/ IV acyclovir - males, smokers, pregnant women, very young/ old, and immunocompromised at increased risk of getting pneumonia as complication of varicella for shingles (uncomplicated); just use PO acyclovir.. only need IV acyclovir when it is complicated (like in herpes zoster opthalmicus or host is immunocompromised)

what is this image?

Impetigo starts as papules that progress to vesicles with surrounding erythema, which become pustules that break and then form a thick, adherent crust with the characteristic golden appearance. Treatment with an antistaphylococcal and antistreptococcal agent is warranted to prevent post-streptococcal sequelae. (can prevent heart stuff can't prevent PSGN) treat w/ Dicloxacillin

ITP appearing after a viral infection presents with petechiae, ecchymoses, purpura, and/or mucosal bleeding. how to treat?

In children, this condition usually resolves spontaneously within 6 months and is managed with observation alone in most cases (especially in patients with a platelet count > 30,000/mm³ and who have only mild cutaneous bleeding). This patient has a platelet count of 38,000/mm³ and mild symptomatology; therefore, intravenous immunoglobulin is an inappropriate next step. IVIG is generally reserved for pediatric patients with platelet counts less than 10,000/mm³ or less than 30,000/mm³ depending on the severity of bleeding, or for adult patients with severe thrombocytopenia and bleeding.

LP w/ papilledema that you have to worry about risk of brain stem herniation

In patients with brain tumors and increased intracranial pressure (ICP), brain stem herniation is a risk of lumbar puncture. One must always rule out elevated ICP before performing lumbar puncture, by obtaining imaging of the head such as a CT scan or MRI. A CT scan or MRI is always indicated prior to a lumbar puncture (LP) in a patient with concerns for increased ICP (eg, focal deficits, AMS, papilledema, etc.). - (imaging is especially important in children with closed anterior fontanelle) Tumors of neuroepithelial tissue account for almost 3/4 of all primary childhood CNS tumors, which include pilocytic astrocytoma, a low-grade astrocytoma that is the most common brain tumor, and medulloblastoma, the most common malignant CNS tumor of childhood.

The American dog tick (Dermacentor variabilis) is one of the main vectors for Rocky Mountain spotted fever (RMSF) in the eastern and south central United States.

Infected patients become symptomatic after 2 to 14 days and clinical manifestations include fever, headache, malaise, mayalgias, arthralgias, and rash. Infected patients become symptomatic after 2 to 14 days and clinical manifestations include fever, headache, malaise, mayalgias, arthralgias, and rash. The hallmark of RMSF is a blanching erythematous rash with macules (1 to 4 mm) that become petechial over time. Approximately 90% of patients develop this rash between the third and fifth day; it starts on the ankles and wrists and spreads to the trunk. The rash on palms and soles usually occurs in later-stage disease. Therapy should be initiated within five days of symptom onset; clinicians should not wait for the skin rash to develop. - will have THROMBOCYTOPENIA, mild elevation of LFT's, mild hypoNa+... - tx = PO or IV doxycyclien non-pregnant..

when to use IV betamethasone?

Intravenous betamethasone is used to treat women who are preterm (<34 weeks' gestation) and likely to deliver within the next 7 days; the compound increases the baby's surfactant production and reduces the risk of neonatal respiratory distress syndrome (NRDS). note use caffeine to tx central apena of prematurity in newborns... - Apnea is much more common in preterm infants (<37 weeks' gestation) and in newborns with a low birth weight (<2500 g). Apnea usually begins 2 to 3 days after birth and only rarely on the first day. It presents with notable hypoxemia, central cyanosis, and bradycardia (<80/min).

The source of genital injury in children is not always sexual abuse

It is important to discuss with parents toilet training techniques as well as appropriate cleaning of the genital area to avoid irritation. Noninvasive techniques such as warm baths and cleaning the area with soap and water are appropriate management.

high spiking fevers, arthritis, and a salmon-colored rash

JIA - persistent inflammation in one or more joints for >6 weeks in a patient who is <16 years old

Varicella develops in approximately 90% of susceptible household contacts with significant exposure... what do you do for the sibling that is 16 yo and has not been vaccinated nor has had chickenpox yet?

Just give varicella vaccine to household contacts (don't have to give IVIG or acyclovir to family) The vaccine, if given in the first 3 days post-exposure, has been shown to prevent or modify the course of illness in susceptible individuals.

Niemann-Pick disease (NPD) results from the deficiency of sphingomyelinase, leading to a build-up of sphingomyelin and cholesterol. Patients with NPD usually have a cherry-red macula on ophthalmologic examination but also other features that are absent in this patient. Type A presents with hepatosplenomegaly, failure to thrive, and rapidly progressive neurodevelopmental regression leading to spasticity and rigidity in later stages. Type B has a more variable course and pulmonary involvement. NPD type A is more common in persons of Ashkenazi Jewish descent.

Krabbe disease results from the absence of β-galactosidase and leads to accumulation of galactocerebroside. This disease presents during the first year of life with optic atrophy, seizures, and spasticity. Opisthotonos, or abnormal posturing characterized by rigidity and arching of the back with the neck hyperextended, occurs and the child usually dies by age 3 years. Presence of phenylketones in the urine is diagnostic for phenylketonuria (PKU). PKU presents with fair skin, eczema, and musty body odor. As the child ages, mental retardation, hyperactivity with purposeless movements, rhythmic rocking, and athetosis will become evident if left untreated. Deficiency of β-glucocerebrosidase leads to accumulation of glucocerebroside in the reticuloendothelial system. Gaucher disease is diagnosed by the presence of Gaucher cells in the bone marrow. Gaucher cells have a characteristic "crinkled paper" appearance. Patients typically present in adolescence with bone pain, hepatosplenomegaly, and anemia. Thrombocytopenia often causes easy bleeding and bruising. On x-ray, an Erlenmeyer flask deformity of the distal femur is commonly noted.

optic atrophy, seizures, and spasticity

Krabbe's disease results from absence of B-galactosidase -> accumulation of galactocerebroside

Brugada syndrome is a primary arrhythmia syndrome caused by a mutation in the SCN5A gene that encodes the cardiac sodium channel. Hypertrophic cardiomyopathy is associated with Pompe disease, which can be associated with weak muscles in the arms and legs in the setting of facial hypotonia, macroglossia, and tongue weakness

Long QT syndrome is an inherited cardiac condition that results in a prolonged electrical recovery phase or QT interval; it is typically diagnosed after a cardiac event or the sudden death of a family member. note dilated cardiomyopathy is ass w/ DMD

Premie He does well for a few days but then begins to have abdominal distension and bleeding in his NG tube.

NEC is a disease seen primarily in premature neonates ( w/ < 1500g) in which the intestinal wall dies and begins to slough off. BREASTFEEDING has been shown to decrease the risk of developing NEC.

Lisch nodules are raised pigmented hamartomas of the iris and are a relatively specific finding for

NF-1. They are useful in establishing this diagnosis in children and in determining whether parents are affected since they are seen in >90% of adults with NF-1. There is no association with the disorder that this patient has.

Duodenal atresia is complete obstruction of the lumen of the intestine that typically presents with bilious emesis on the first day of life. how to treat? - has double bubble

NG tube suction then IVF

preductal coarctation of the aorta

Need to give PDEi keep PDA open because need some of the blood to go through it to get a little more to legs! Because the obstruction is proximal to the entry of the ductus arteriosus, blood flow to the abdomen and lower extremities has been supplied primarily by the ductus arteriosus and collateral flow through the intercostal arteries. Eventually fix with surgery but start w/ keeping PDA open

A 2-year-old boy is brought to the pediatrician's office by his mother because of strange brown spots on his body. These coffee-colored lesions are 5 mm in diameter and located over the child's torso. The mother reports that her son is slightly developmentally delayed. On physical exam, freckles are noted in the axilla. On ophthalomgic exam, brown dome-shaped elevations over the surface of the iris are seen. A mutation on which of the following chromosome accounts for this disease?

Neurofibromatosis type 1 is characterized by café-au-lait spots, axillary or inguinal freckling, and Lisch nodules in addition to scoliosis and optic gliomas. The gene responsible is located on chromosome 17.

The infantile form presents with hepatosplenomegaly, failure to thrive, and rapidly progressive neurodevelopmental regression

Niemann-Pick disease results from deficiecny of sphingomyelinase.

Food poisoning . 6-72 hrs vs 1-6 hrs onset

Nontyphoidal Salmonella infection presents, 6-72 hours after ingestion of poultry or related products, with acute onset of nausea, vomiting, and abdominal cramping, followed by watery and bloody diarrhea. Antibiotics are contraindicated; treatment consists of aggressive hydration. Staphylococcus aureus is a gram-positive coccus that is associated with ingestion of a preformed exotoxin that causes symptoms of nausea, vomiting, and abdominal cramps within 1-6 hours. Fever and diarrhea occur in a minority of patients, and duration of symptoms is usually around 1 day. It is classically associated with custards and mayonnaise. The longer incubation period, presence of fever, and bloody diarrhea experienced by this patient are not consistent with food poisoning caused by S. aureus

what vaccines are live vaccines and are contraindicated in patients w/ immunodeficiencies?

PO Polio, MMR, varicella (MMRV)

8yo Three weeks ago, he had a rash around his mouth that resolved after treatment with mupirocin ointment. Urinalysis shows 3+ blood and 2+ protein. Laboratory work shows a low serum C3 level.

PSGN generally presents 1-3 weeks (even up to 12 weeks) after a recent Group A β-hemolytic streptococcal infection, with a positive antistreptolysin titer. Patients may have oliguria, hematuria, hypertension, and smoky-brown urine. Spike-and-dome $&%#$&% appearance on electron microscopy. IgA nephropathy: presents with recurrent episodes of gross hematuria 1-2 days after an URI or GI infection, with recovery that is often rapid between attacks (PSGN follows pharyngitis by 1 to 2 weeks) 1-2 days = iGA nephropthy... 1-3 wks = PSGN

Chediak Higashi syndrome: what are they at risk for getting in future?

Patients with Chédiak-Higashi syndrome are at risk for developing an ″accelerated phase″ or hemophagocytic lymphohistocytosis (HLH) after viral infection, which is characterized by pancytopenia, high fever, and lymphohistiocytic infiltration of the liver, spleen, and LN. HLH is the most frequent cause of death in patients with Chédiak-Higashi syndrome and presumably arises from an inability to eradicate virus-infected cells, allowing for an uncontrollable immune response.

fair skin, eczema, and musty body odor.

Phenylketonuria (PKU) results from an enzyme or cofactor deficiency that prevents phenylalanine from being converted to tyrosine. - if untreated will -> mental retardation

physiologic jaundice

Physiologic jaundice affects most newborns; it results in a mild unconjugated hyperbilirubinemia after day 3 of life and resolves by 1 week in full-term neonates and 2 weeks in preterm neonates. Notably in physiologic jaundice, the conjugated bilirubin is always normal, and total bilirubin is always <14 mg/dL.

what is the MC pediatric CNS tumor?

Pilocytic astrocytoma = MC BRAIN TUMOR in kids; is in cerebellar hemisphere.. best prognosis of kid brain tumors.. cystic rosenthal fibers (eosinophilic corkscrew-fibers).. 5 yr survival > 90%, surgical resection! #$% medulloblastoma is MC MALIGNANT brain tumor in kids.. found in posterior fossa... arises from cerebellar vermis or 4th ventricle... - they -> ICP... small, blue, poorly differentiated homer-wright rosettes.. highly malingnat has high propensity to metastasize to CSF -> spread to bone.. all patients > 4 yo tx w/ surgery/ radiation.. 60% survival. - med school is for kids Ependymomas only 10% peds tumors posterior fossa, from primitive glial cells... usually 4th ventricle.. cystic w/ calcification and hemorrhage.. perivascular rosettes = diagnostic.. 50% 5 yr survival. 3rd most common = brain stem gliomas; CN invasion and personality changes usually.

what is elevated in sepsis that is a blood product

Plasminogen activator inhibitor-1 is a procoagulant molecule and its levels are elevated in sepsis (both adult and neonatal). This is because the delicate procoagulant-anticoagulant balance is tipped towards the procoagulation side in sepsis, resulting in widespread coagulation and microvascular thrombi, which compromise oxygen delivery to end organs...

This patient exhibits symptoms of elevated intracranial pressure (ICP). what is cushing triad for ICP??

Possible physical examination findings include papilledema (as shown in the image); a dilated ipsilateral pupil; CN palsies of the third (most common), fourth, and sixth nerves; and NUCHAL RIGIDITY (not just for meningitis). Hemiparesis, hypertonia, hyperreflexia, or Cushing triad (systemic 1. HTN, 2. Bradycardia, and respiratory depression) are late signs 1. HTN 2. lower HR 3. lower RR

Angelman's syndrome vs Prader-Willi syndrome

Prader-Willi (obese) = paternally inherited gene deletion Angelman's syndrome = happy puppet = maternally inherited chromsome deletion. both chromosome 15

NRDS (neonatal respiratory distress syndrome)

Presents w/in first 24 hrs

painless abdominal mass, hypertension, respiratory distress, Horner syndrome, or cord compression

Primary neuroblastoma; however, neuroblastoma is not diagnosed until after it has metastasized - Tumor infiltration of the bone marrow causes pancytopenia. - Tumor infiltration of the periorbital bones causes periorbital ecchymosis and proptosis, an appearance often referred to as ″raccoon eyes.″ - Low-grade fever, fatigue, and FTT occur w/ metastatic disease - Urinary catecholamines are elevated in neuroblastoma, but definitive diagnosis requires biopsy. Treatment involves chemotherapy and surgical excision of local disease. - younger you are better diagnosis!

Children with this condition walk on their toes and have a scissor gait.

Pyramidal cerebral palsy (the most common variant) is nonprogressive and characterized by spasticity, hyperreflexia, slow effortful voluntary movements, and impaired fine motor function - Mental retardation is present in 90% of cases of pyramidal CP. - risk factors include prematurity, perinatal asphyxia, intrauterine growth retardation, infection, trauma, brain malformation, and hemorrhage.

erythema toxicum neonatorum

Red papular rash on babys torso which is benign and disappears after a few days. - can be on face

patient was developmentally normal until like 6 mo then they regress.. language and coordination are most commonly affected functions - Her parents note that she has developed a peculiar behavior of wringing her hands for long periods.

Rett syndrome is a genetic neurodegenerative disease found almost exclusively in females. The MECP2 gene responsible for this syndrome is located on the X chromosome - Rett syndrome is a genetic neurodegenerative disease found almost exclusively in girls.

ABO incompatibility can cause a generally milder neonatal jaundice that is triggered almost exclusively in type A or B neonates born to type O mothers. IgG antibodies (anti-A and anti-B) can cross the placenta and cause hemolysis in the fetus. This will cause a weakly positive direct Coombs test. Disease from ABO incompatibility is generally much less severe than with Rh disease. Hemolytic disease only occurs in about 15% of type A or B neonates born to type O mothers and severe disease only occurs in about 1% of that population.

Rh incompatibility between mother and fetus can result in the production of antibodies that can cross the placenta. This can result in unconjugated hyperbilirubinemia, anemia, and increased hematopoiesis in a fetus with Rh-positive blood. Diagnosis is supported by a positive Coombs test.

failure to thrive and recurrent infections

SCID mutation in the gene encoding the interleukin 2 receptor gamma, - X-linked recessive tx = HCT always fatal w/in first 1 yr of life w/out tx... - long term survival after SCID HCT = 90%

7 mo girl recurrent infections Her CBC with diff shows a lymphocyte count of 1200 cells/µL. An immunologist is consulted, and T-receptor excision circle (TREC) test results show T-cell lymphopenia.

SCID. Give bactrim tmpsmx for PCP ppx... Then HCST eventually stem cell transplant

most common cause of osteomyelitis in sickle cell disease

Salmonella is the most common cause of osteomyelitis in the United States and Europe, while S. aureus is the most common cause in Africa and the Middle East. so salmonella

"Strawberry tongue"

Scarlet fever (strep pyogenes GAS), Kawasaki disease If person has Kawasaki's; - complication = coronary aneurysms happen in 25% of untreated patients.. - MI is a consequence of developing a coronary aneurysm. Patients with Kawasaki disease are at risk for the development of coronary aneurysm because of the underlying associated vasculitis, and the MI is only a further complication because of the presence of the aneurysm itself.

18 mo kid comes into office... was good at birth, but since then poor feeding during neonatal period (right after birth).. now delayed motor milestones, poor head control.. has difficulty walking, knees often crossing and need to walk on tiptoes, muscles = hypertonic/ spastic

Sissor gait - knees cross each other as they are brought forward, cerebral palsy this is Cerebral Palsy; - mostly idiopathic can be ass w/ following - Perinatal ASPHYXIA, prematurity, IUGR, early infection/ trauma, brain malformation, neonatal cerebral hemorrhage.

X-linked agammaglobulinemia (Bruton's) as a result of a B-lymphocyte-specific tyrosine kinase defect. #$%#$% BTK

Suspect this diagnosis in males with a positive family history, history of recurrent bacterial infections, normal T-lymphocyte levels, and low to absent B-lymphocyte levels treat the ear infection w/ amozacillin.. but then do regular IVIG don't do BMT in this disease... - BMT is needed in like SCID and DiGeorge Syndrome.

The image shows the "cherry-red" retinal lesions characteristically seen in infantile Tay-Sachs disease. Absence of hexosaminidase A in WBCs is diagnostic for Tay-Sachs disease.

Tay-Sachs has AR inheritance. It is most prevalent among Ashkenazi Jews (1:25-30 are carriers). Infantile Tay-Sachs presents at 4-5 months of age with loss of motor skills, increased startle reaction to noise, and retinal cherry-red spots. Seizures develop by 2 years of age, and children typically do not survive past 4-5 years of age.

At 3 months of age, the child should be able roll on side, reach for parent's face, visually follow a person who is moving across the room, and vocalize when spoken to. 6 months old. This means she should be able to sit propped on hands, roll over, demonstrate stranger anxiety (recognize familiar versus unfamiliar people), smile/vocalize to mirror, and transfer objects from hand to hand.

The child should be able to crawl with all four limbs, pull to a stand, use a pincer grasp, wave, and say "mama/dada" (nonspecific) at 9 months. The child would also demonstrate separation anxiety (anxiety provoked in a young child by separation or the threat of separation from their mother/primary caregiver) at 9 months.

This child's presentation is concerning for bacterial meningitis, a subtle and potentially devastating illness. Its presentation is notoriously protean and nonspecific in neonates, but its overall mortality rate is relatively high, making it a critical diagnosis. The clinical presentation of neonatal meningitis is typically indistinguishable from that of neonatal sepsis without meningitis, and the most commonly reported clinical signs are Temperature instability (can be low), irritability or lethargy, and poor feeding or vomiting.

The full fontanelle is a more common finding than a bulging one in bacterial meningitis, and should make one think of increased intracranial pressure; nuchal rigidity is appreciated in only 15% of neonatal bacterial meningitis. Finally, this patient has low-normal blood pressure and high-normal heart rate, which suggest that she might be trending towards sepsis. Any newborn with possible sepsis requires a full work-up including a lumbar puncture (LP), CBC with differential, blood culture, and urine culture (if >6 days of age). When signs of sepsis are present, an LP should be performed, ideally before antibiotic therapy is initiated. If clinical instability precludes performing an LP at the time of initial diagnostic evaluation, then antimicrobials should be provided in doses sufficient for the treatment of meningitis until such time that the infant's condition permits evaluation of the cerebrospinal fluid (CSF). Patients are at greater risk of bacterial meningitis during their first month of life than they are at any other point in life. It is caused by GBS in 50% of cases and E coli in 25% of cases.

Trisomy w/ midline defects

Trisomy 13, or Patau's syndrome. The major midline dysmorphic features of trisomy 13 are due to a defect in the fusion of the midline precordial mesoderm in the first 3 weeks of gestation, and may include mental and motor retardation, POLYDACTYL, microcephaly, low-set ears,, HOLOPROSENCHEPHALY heart defects, microphthalmia, midline cleft lip and/or palate, and OMPHALCELE. Most children affected with trisomy 13 die within the first 6 month

kid had acute otitis media following URI.. sign of middle ear inflammation (otalgia) and sign of middle ear effusion (dishcarge)... untreated AOM can have complication of mastoiditis..

The risk of untreated acute otitis media includes mastoiditis, in which the area behind the ear is painful, swollen, and erythematous. - puss filled mastoid cells and infections can spread -> meningitis because mastoid is close to brain.. tx w/ IV Abx and maybe surgery.

A newborn with bilious emesis, abdominal distention, and failure to pass meconium likely has cystic fibrosis (CF), an autosomal recessive defect. Meconium ileus is nearly pathognomonic for CF, and a diagnosis of CF should be considered until proven otherwise; however, it can also be caused—although less commonly—by volvulus, intestinal pseudo-obstruction, or other rarer causes of pancreatic insufficiency Physical examination reveals a severely distended abdomen that elicits pain on palpation. An x-ray of the abdomen reveals a small bowel obstruction with numerous air-filled loops of bowel. The patient is successfully treated with a diatrizoate meglumine enema. Which of the following is the most likely mechanism of this infant's acute intestinal problem?

This causes meconium to become mucilaginous and viscid

HAMARTOMASS" can be used for tuberous sclerosis: Hamartoma; Angiofibromas (previously called adenoma sebaceum); Mitral regurgitation; Ash-leaf spots; Rhabdomyoma; (Tuberous sclerosis); dOminant inheritance; Mental retardation; Angiomyolipoma (of kidney); Seizures/Shagreen patches. IRIS HAMAROMA's

This patient has tuberous sclerosis, and the image shows characteristic facial angiofibromas (previously called adenoma sebaceum). Ophthalmic findings in tuberous sclerosis include both retinal and nonretinal abnormalities and can be useful in making the initial diagnosis. Retinal lesions are hamartomas, also known as phakomas, which can be flat and translucent or can be multilobular lesions that, if calcified, have a classic mulberry appearance. These lesions rarely affect vision and thus do not require any specific treatment. Nonretinal lesions include angiofibromas of the eyelids, strabismus, colobomas, and iris depigmentation.

This patient has a recent history of a sore throat, no significant family history, and is demonstrating spasmodic limb movements with emotional lability. This clinical presentation of Sydenham chorea (St. Vitus dance) and polyarthritis is consistent with acute rheumatic fever. Diagnosis is made based on Jones criteria by satisfying either two major criteria or one major criterion plus two minor criteria. The major criteria are polyarthritis, carditis, subcutaneous nodules, erythema marginatum, and Sydenham chorea (St. Vitus dance). Treatment of acute rheumatic fever involves what? rheumatic fever = group A strep = strep pyogenes

Treatment of acute rheumatic fever involves long-term antibiotic therapy and supportive therapy for relief of symptoms. Treatment of Sydenham chorea consists of steroids or anticonvulsants to reduce the severity and duration of symptoms. answer for this question = prednisone and long term Abx steroids for sydenham chorea for the S in JONES

what is the MC cyanotic congenital heart defect in newborns?

Transposition of the great vessels. - incompatible w/ life unless PDA - most often present immediately after birth. In addition to tachypnea, cyanosis, and hypoxemia, some patients have signs of congestive heart failure (CHF). The radiographic findings described (a narrow heart base and absence of the main pulmonary artery segment, resulting in an egg-shaped silhouette, and increased vascular markings) are present at birth - if you can't keep PDA open make an ASD before surgical correction

Cerebral palsy is the most common motor disability in childhood. Cerebral palsy affects one's ability to coordinate movements - delayed developmental milestones, persistence of infantile reflexes (babinski - should be gone after 2 yo), contractures, weakness or development of affected limbs.. how to treat spasticity?

Treat spasticity with muscle relaxants (diazepam) and PT and OT

Scarlet fever is caused by group A β-hemolytic streptococci and can occur if streptococcal pharyngitis is left untreated. It is characterized by findings such as strawberry tongue and a sandpaper-like rash on the trunk that desquamates (scales/ flakes) after a few days.

Treatment is with penicillin to prevent rheumatic fever and its complications, which can include irreversible damage to the heart and its valves. note strawberry tongue

Prominent occiput, micrognathia (strawberry chin), horseshoe kidneys, malrotation

Trisomy 18, or Edwards' syndrome, is the second most common trisomy observed in live births. Clinical manifestations include feeding difficulties, hypotonia eventually leading to hypertonia, prominent occiput, micrognathia, horseshoe kidneys, flexed fingers with overlapping fingers, congenital heart defects, and malrotation of the gastrointestinal system.

A 5-year-old boy with a known history of developmental delay and seizure disorder presents to the emergency department presenting with an episode of generalized tonic-clonic seizures that lasted for 15 minutes. The boy's seizures were previously controlled on valproic acid. On examination, his temperature is 36.8°C (98.4°F), blood pressure is 120/76 mm Hg, heart rate is 88/min, and respiratory rate is 14/min. He appears confused and drowsy but there is currently no seizure activity; cardiovascular and respiratory examinations are normal. Abdominal examination reveals a lesion similar to the one shown.

Tuberous sclerosis is a neurocutaneous disorder characterized by a spectrum of mental retardation, seizures caused by cortical tubers, Shagreen (leathery) patches, ash-leaf spots (in image = hypopigmented macule), ungal/periungal fibromas, retinal hamartomas, cardiac rhabdomyomas, subependymal nodules, giant cell astrocytomas, and renal angiomyolipomas (AML). small solitary angiomyolipomas don't need follow up but if they have a lot of them might monitor for growth.. - renal angiomyolipomas = MC renal manifestation of Tuberous sclerosis -> 1-2% -> RCC... periodic US for screening.

A postmature infant who is small for gestational age has evidence of meconium-stained amniotic fluid and developed respiratory distress shortly after birth. This is most suggestive of meconium aspiration syndrome (MAS). &,!:$:!: Radiographic features are irregular LINEAR infiltrates with HYPEREXPANSION and PNEUMOTHORAX. ^~€]>~!|*{!{!| The pathophysiology of MAS involves INTRAUTERINE PASSAGE of meconium, aspiration, and pulmonary disease, which results in hypoxemia and acidosis. Aspirated meconium can interfere with normal breathing by several mechanisms which include airway obstruction, chemical irritation and inflammation, infection, and surfactant inactivation. MAS is a risk factor for persistent PULMONARY HTN. Infants at highest risk for MAS are those born POST-TERM or who are SGA. %#+%+]

Tx: PPV via Cpap

Wilms tumor, also known as nephroblastoma, is the most common primary malignant neoplasm of the kidney in children, typically presenting as a PAINLESS abdominal or flank mass.

Tx: nephrectomy and chemo

US vs Xray for DDH

US - for < 6 mo (ossificaiton of femoral head has not occured yet) Xray for those > 6 mo before 6 mo use pavlik harness after 6 mo use spina casting.. if you fail closed reductions or in those > 2 yo use surgery! < 6 mo = US and harness > 6 mo = Xray and cast

turner syndrome what other than wide set nipples 45% have coarctation of aorta (give PGE1 to keep open)

WEBBED neck, and cubitus valgus (wide carrying angle)

complex skull fracture

admit patient for observation contact CPS (child abuse) if child is crying (vocalizing) and not vomiting = stable condition don't need to intubate subdural hemorrhage is NOT an indication for mannitol.. - use manitol if they had evidence of increased ICP = drowsy, vomiting, bulging fontanelle, separated skull sutures

eczema, elevated IgE and IgA levels, decreased IgM levels, and thrombocytopenia - high AE and low M

Wiscott Aldrich syndrome.. treat w/ bone marrow tranpslant if not.. use splenectomy and IVIG

when to use different confirmatory tests potassium hydroxide preperation vs wood's lamp for fungus stuff like tinea

Wood's lamp; for tinea capitus, erythrasma, porphyria cutanea tarda... Potassium hydroxide preperation show branching hyphae; tinea pedis (athlete's foot) is how to diagnose that!

Wiscott aldrich syndrome

X-linked; atopic dermatitis, thrombocytopenia, increased susceptibility to infection... - persistent WAS prone to VIRUS related MALIGNANCIES such as LYMPHOMA related to EBV! #$%#$%

NRDS (neonatal respiratory distress syndrome)... what is X-ray? presents within minutes to hours of birth

X-ray of the chest shows lung parenchyma with a reticulogranular ground-glass appearance and air bronchograms. Treatment is with artificial surfactant, and ventilation and oxygenation as needed

WAGR syndrom

anirida (absence of iris) and renal anomoly at birth Wilms tumor, Aniridia, GU anomolies, mental Retardation/ Intelectual disability.. - it is from chromosme microdelation of 11p13..

APGAR scores

appearance pulse grimace activity respiration 0-3 = immediate resuscitation (bag mask ventilation/ intubation)... 4-7 possible resuscitation (blow-by o2).. - 8-10 = good.

Vaccine schedule Birth 1 month 2, 4, 6, 9, 12, 15 months

at birth only vaccine you get is hep B.. it is 3 doses start influenza at 6 mo start MMR, Variccella, and hep A at 1 yo like all the vaccines PCV13, DTaP, rotavirus, IPV, HiB start at 2 mo.

treating spasticity in Cerebral palsy?

baclofen - GABA agonist! muscle relaxant

RSV vs parinfluenza virus

both are paramyzoviruses RSV = MC of bronchioliits (also can cause pneumonia though) parainfluenza virus = steeple sign = croup (inspiratory stridor, laryngotracheobronchiolitis), lose voice more.. also can cause brocnhiolitis Croup is an upper respiratory illness with inflammation in the larynx causing inspiratory stridor and a harsh, barky cough. Bronchiolitis is a lower respiratory illness with inflammation in the bronchioles causing expiratory wheezing and a wet cough croup vs bronchiolitis = just diff in location.

bacterial tracheitis.. is acute bacterial infection of upper airway -> fatal airway obstruction.. < 3 yo in setting of URI..

brassy cough, high fever, respiratory distress - tx aimed at croup (mist and racemic epi) don't work for this - MC = S. Aureus.

A 5-year-old boy presents to the emergency department with 4 days of nonproductive cough and shortness of breath. His mother reports that he has had multiple episodes of upper respiratory infections in the past, and recently a few classmates at preschool were ill. She says he has had no fever or rhinorrhea, no gastrointestinal symptoms, and no ear pain. There is a family history of eczema and a cousin has cystic fibrosis. His blood pressure is 115/80 mm Hg, temperature is 37.4°C (99.4°F), heart rate is 89/min, respirations are 30/min, and oxygen saturation is 94% on room air. On examination, there is end-expiratory wheezing bilaterally. There are also some costal retractions and nasal flaring. X-ray of the chest shows mild hyperinflation. Which of the following is a likely cause of this patient's symptoms?

bronchial hyperractivity = bronchospasm this patient has asthma (wheezing, cough, and dyspnea) = acute ashtma exacerbation.. fam hx of atopy.. - CXR = HYPERINFLATED lungs.. urban environmenet.. eczema/ atopy, family hx of asthma.. trigger can abe smoke or allergen from pet. if it was CF = GI issues and FTT.. usually has significant cough w/ mucous production.

4 yo

can count to 4.. speak 100% intelligibility.

type I distal RTA

causes hypoK+ w/ normal anion gap metabolic acidosis.. - is ass w/ Collagen vascular disease (EDS), autoimmune disorders (sjogren's and SLE), amphotericin B use

Juvenile idiopathic arthritis is a collagen vascular disease defined by persistent inflammation in one or more joints for >6 weeks in a patient who is <16 years old.. what are some complications long term? quotidian fever that eventually subsides, morning stiffness and joint pain, and visual changes.

chronic uveitis -> blindness in young children if left undiagnosed. note Urethritis is ass w/ like gonarrhe and other infections can be ass w/ joint issues but not w/ JIA

systolic ejection murmur at the left upper sternal border radiating to the interscapular region

coarctation of aorta know by delayed femoral pulses relative to upper extremity pulses and HTN of upper extremities.

what do we use racemic epi for?

croup = laryngotracheobronchitis (parainfluenza virus).. inspiratory stridor

Renal pathologies decreased C3, normal C4, Antistreptolysin Ab +, ANA -, ANCA - Decreased C3 and C4, antistreptolysin Ab -, ANA +, ANCA - Decreased C3 levels and subendothelial immune complex deposition in the kidney Normal C3 and C4, antistreptolysin Ab-, ANA -, ANCA + crescent formation on light misroscopy and linear dpeosition of immunofluorescence

decreased C3, normal C4, Antistreptolysin Ab +, ANA -, ANCA - - PSGN (10-21 days after strep pharyngitis infection) Decreased C3 and C4, antistreptolysin Ab -, ANA +, ANCA - - SLE Decreased C3 levels and subendothelial immune complex deposition in the kidney - MPGN (membroproliferative glomerulonephritis) Normal C3 and C4, antistreptolysin Ab-, ANA -, ANCA + - ANCA-ass vasculitits crescent formation on light microscopy and linear deposition of immunofluorescence - anti-GBM Ab (Goodpastures)

congenital dermal melanocytosis = mongolian spots...

delayed disappearance of dermal melanocytes.

CF: coughing and GI symptoms (pancreatic insufficiency) what are associated symptoms?

digital clubbing (secondary to long-standing hypoxia) and hypoNa+ (secondary to increased salt loss in sweat).

roseola

does not spare face like I thought; Illness is heralded with a high fever (38.3°C to 41.1°C [101°F to 106°F) that persists for 3-5 days, with the development of a pink, rosy discrete rash that begins on the trunk and spreads to the neck, face, and extremities. The rash usually appears within 12-24 hours of fever resolution. - is fever then rash.

when to fix cryptochidism

don't fix until like after 6 mo... hypospadias and epispadias fix between 3-6 mo using foreskin. Hypospadias and cryptorchidism are not associated with increased risk of UTI or pyelonephritis, making long-term antibiotic use or ultrasound screening inappropriate.

patient's normal early development and subsequent drop in growth and height percentile in adolescence should raise suspicion for a late-onset developmental delay - in absence of other symptoms and a positive family history of delayed puberty and growth, constitutional delay is most likely how do you assess constitutional delay?

dx w/ bone age assessment! most important Constitutional delay manifests with childhood short stature but normal adult height. It has a strong genetic basis, making positive family history a clue to diagnosis. Pubertal development may also be delayed. Bone age (determined by measuring the extent of ossification at the wrist bone epiphyses) is delayed and correlates better with the age at which the patient's height would be normal, rather than with the patient's chronologic age. bone age assessment is most important if you suspect constitutional delay but other ones to consider w/ small growth! - renal function tests and UA (check for CKD) -> growth delay - IGF-1 levels (GH deficiency) - ESR and CBC (preimary anemia or anemia secondary to chronic disease) - Karyotyping (turner syndrome) - Head MRI (pituitary etiology) - Thyroid function tests (hypothyroidism)

Epiglottitis what to do?

endotracheal intubation (not cricothyrotomy!) - so start w/ endotracheal intubation.

ARDS image and description pathology

enlarged capillaries and granular membranes lining the alveolar ducts.

Hemolysis from ABO incompatibility can -> acute bili encephalopathy.. tx =

exchange transfusion -> lower bili levels.. - Exchange transfusion uses donor blood that lacks clotting factors, so extensive exchange transfusion can predispose the patient to bleeding.

PKU

fair skin, eczema, musty body odor, mental retardation.. - reduce phenylalanine and increase tyrosine in diet

septic hip joints in kid

get blood and synovial cultures emperic Abx -> take to OR for irrigation/ debridgement.

Beckwith-Wiedmann syndrome

hemihypertrophy, macroglossia, visceromegaly, neurofibromatosis and WAGR syndrome = Wilms tumor, Aniridia (absence of iris), GU anomolies, mental Retardation/ Intelectual disability.. umbilical hernia

At 2 months of age, infants should receive what vaccines?

hep B, rotavirus, diptheria-tetanus-pertussis, H flu type B, inactivated poliomyelitis, and pneumococcal conjugate vaccine.

embryology of digeorge syndrome

hypoplasia of 3rd pharyngeal POUCH

this infant is 4 day old.. has possible bacterial meningitis what do you use?

if < 1 mo - GBS, E coli, LIsteria - so use cefotaxime (not ceftriaxone bili) + ampicillin don't need vancomycin - while waiting blood cultures and CSF cultures

Unexplained fever, fatigue, and nonspecific symptoms with splenomegaly should raise concern for malignancy in children

if it was AML = elevated myeloperoxidase (from granules of neutrophils) if negative for myeloperoxidase = ALL.. - ALL has lots of lymphocytes on blood smear.. - ALL has dramatic response to chemo > 95% remission #$%$%' AML (subtype of APL) -> DIC and is treated by ATRA.. - APL often has DIC present after initiation of chemo in these patients coronary aneurysms ass w/ Kawasaki's..

supracondylar humerous fracture from FOOSH..

impingement or injury to brachial arteyr = critical concner it runs along humerus.. therefore distal pulses and capillary refill should be monitored closely. If left untreated too long, a Volkmann ischemic contracture can develop. - Some concerning signs include a cold hand with poor perfusion, pallor, and a diminished pulse. An absent pulse is a late finding and should prompt emergent consultation with a pediatric orthopedic surgeon. The first step in management is to attempt closed reduction with percutaneous pinning (CRPP) in the operating room, followed by reassessment of the distal vascular status. If treatment is not initiated promptly, there is increased risk of Volkmann ischemic contracture, which is an ischemic contracture that shortens the muscles of the forearm and causes fixed elbow flexion, forearm pronation, wrist flexion, and metacarpal-phalangeal joint extension. Neuropraxia of the anterior interosseous nerve is common, and tests for this condition should be done before and after CRPP.

Endotoxin released by bacteria such as meningococci can cause fulminant sepsis leading to disseminated intravascular coagulation endotoxin incites a release of prothrombotic and inflammatory agents with resulting microthrombi and insufficient vasculature, rather than fenestration of vessels caused directly by endotoxin.

in DIC secondary to overwhelming sepsis.. there is widespread microthombi in small vessels -> platelet and coag factor consumption -> mixed bleeding/ clotting.. thromboytopenia, increased PTT and PT, decreased clotting factors, increased D-dimer answer is Platelet and clotting factor consumption not vascular fenestration by bacterial endodoxin. (this is more like inflammation/ WBC and stuff)

how does congenital syphilis present?

including cutaneous lesions on the palms and soles, hepatosplenomegaly, lymphadenopathy, jaundice, anemia, and rhinitis. Up to two-thirds of infants are asymptomatic at birth. - collapsed nasal bridge seen in this patient describes the "saddle-nose" deformity classically seen in patients with congenital syphilis tx w/ penecillin

why do African Americans and other people of color have darker skin?

increased melanosomes w/in melanocytes.

West syndrome:

infantile spasms, regression of psychomotor development, and hypsarrhythmia on electroencephalogram (EEG) - EEG to dx and tx w/ ACTH and anti-epileptic drugs Congenital cytomegalovirus infection can result in seizures that might resemble the "spells" described above. However, these classically result from paraventricular lesions including calcifications that are best seen with head CT.

what is pseudohypoPTH (hypoparyathoiridism)?

inherited disorder due to deficient end organ response to PTH.. hypoCa2+, hyperphosphatemia and elevated PTH #$%#$% congenital hypoparathyroidism would have low PTH level obviously

choriocarcinoma testicular CA

is a germ cell testicular CA.. close ass of syncytiotrophoblasts and cytotrophoblasts cells.. - germ cell testicular CA makes up most of testicular cancers seminomas and non-seminomas.. Choriocarcinoma ; non-seminomatous testicular cancer hase elevated B-hCG. - often have GYNECOMASTIA, alpha subunit of B-hCG looks like LH and stimulates testicular leydig cells to secrete a lot of T -> peripehral aromatization -> E2 -> gynocomastia! #$% I think of it as Cory ass w/ blood and bHCG pregnant.

erythema multiforme

is ass w/ HSV or mycoplasma in kids.. may also be due to vaccination, drugs, or malignancy erythema multiforme; secondary to HSV in this case - EM rash involves palms, soles extremities more than trunk... erytehmatous purpuric plaques and Bullae w/ CENTRAL CLEARING... TARGET LESIONS seen in MINORITY of cases painful and may have fever... steven's johnson syndorme is worse = trunk, face, systemic symptoms and progresses to toxic epidermal necrolysis. dusky macules < 10% BSA detatchement. image = Erythema multiforme also can have on shins and stuff.

pneumatosis intestinalis

is from Necrotizing enterocolitis.. MC life threating conidtion of the newborn GI tract.. - Risk of NEC is higher in premies - sx w/in first 2 wks of life = abdominal distension, delayed gastric emptying.. can have hematochezia! #$% (bloody stools), may ahve respiratory distress as illness gets worse Xray; hepatic portal venous gas and bubbly apperance of pneumatosis intestinalis (gas in bowel wall) = pathognomonic for NEC.. First do non-operative management.. NG decompression, NPO, TPN, broad spectrum abx (vanc, gent, clinda) - Surgery probably

Fanconi anemia - inherited disease caused by mutations in certain genes needed for DNA crosslink repair

is the most common inherited form of aplastic anemia Mostly Ar but some X-linked short stature, abnl pigmentation, #$%#$% AML #$%#$% , tumors of head and neck, radial polydactyly, can have missing thumbs is higher frequency in ashkenazi jew's..

person is pregnant 30 wks gestation has HIV well managed on her Highly active antiretroviral therapy comes into office what to do?

just continue giving HAART! $#%#$% - peripartum zidovudine and nevirapine recommended for those who are not receiving HAART previously for disease management.. but if already on HAART just keep doing that!

what is a risk complication of ASD?

makes patients prone to tachyarrhythmias later in life

Precocious puberty, after classification has been confirmed by history and results of physical exam and bone age studies, should be evaluated by

measurement of LH levels. Elevated LH levels indicate central precocious puberty; low LH levels indicate peripheral precocious puberty.

Horner syndrome in young kid what are you thinking?

miosis (pinpoint pupil), ptosis (drooping eyelid), and anhidrosis - in young kid indicates neuroblastoma: HTN, subQ tumor nodules, periorbital bruising. Multiple sclerosis can cause Horner syndrome and other neurologic symptoms, but it does not occur in infants or cause elevated urine catecholamines. Neuroblastoma = It usually occurs in children <5 years old, and most commonly in those <2 years old. Symptoms are due to the location of the primary disease or by the tumor′s secretion of abnormal catecholamines, which can be detected in the urine. - amplification of N-MYC gene, which confers a worse prognosis.

guaiac test

most common form of FOBT (fecal occult blood testing) checks for blood in stool.

Things that make you think GERD?

neck torsion and chin elevation position, arching back

what is it if it can cross midline? mass in kid

neuroblastoma; 3rd MC pediatric CA (behind leukemia and brain tumors)... - most of neuroblastomas are sporadic only 1% inherited - MC site = adrenal gland.. - can have Horner's sydnrome miosis, ptosis, anhidrosis... - fever wt loss and HTN (if there is defective catecholamien syntehsis and accumulation of vanillylmandelic acid and homovanillic acid elevated in urine)... - kids 2-12 mo best prognosis.. MYCN oncogene status is important.. - tx resection.

This patient, with a 4-day history of upper respiratory tract infection, presents with gross hematuria. He has proteinuria, RBCs, and RBC casts on urinalysis. This patient likely has IgA nephropathy (Berger disease)

normal C3 levels and negative antistreptolysin Ab and negative ANA - diffuse mesangialdepositis of IgA

Presence of proptosis, periorbital swelling, painful eye movements, and ophthalmoplegia is suspicious for

orbital cellulitis. Almost 70% of cases of orbital cellulitis are associated with preexisting sinusitis, particularly ethmoid sinusitis or pansinusitis. Orbital cellulitis is an ophthalmological emergency and needs to be managed appropriately on a case-by-case basis.

______ MC primary bone malignancy in kids?

osteosarcoma - Codman's triangle and sunburst appearance - elevation of periostium - destruction of trabeculae, lack of endosteal bone response, and a mixture of radiodense and radiolucent areas. - sites that are common - distal femur, proximal tibia, proximal humerus, and proximal femur. so right around knee joint! $%

knobby chest deformity = rickents (vit D)..

peripheral neuropathy = copper deficiency.. increased head circumference = paget's disease Type I collagen disorder AD, type 1 collagen. - fragile bones, HEARING LOSS, blue sclera

patient has TOF.. immediate exacerbation what to do? - if they have a tet spell?

place knee-chest position! #$% also give supplemental o2 and morphine sulfate. - don't need emergent surgery may need surgery later

This patient, with development of secondary sexual characteristics at age 6, likely has precocious puberty. Precocity is defined as development of secondary sexual characteristics in girls <8 years old and in boys <9 years old. These can include thelarche (breast development), adrenarche (pubic hair development), and menarche (initiation of menstruation) GnRH released from hypothalamus -> acts on pituitary to release LH and FSH.. LH -leydig cells-> E2 and FSH -sertoli cells-> inhibin (both E2 and inhibin act back to inhibit GnRH production and LH and FSH production) guys - LH -leydig cells-> T - FSH- sertoli cells-> inhibin A girls - the LH acts on theca cells to make androsteredone - FSH acts on granulosa cells takes androsteredone and turns it into E2

presence of comedones is also important to recognize because they are never normal in the prepubertal age group and could be a sign of early activation of the hypothalamic pituitary adrenal axis. evaluating dx tools; pubertal staging, bone age, and FSH and LH levels. - FSH and LH level, which will likely be elevated. - Treatment of precocity consists of gonadotropin-releasing hormone (GnRH) AGONISTS (eg, leuprorelin and nafarelin) to preserve height potential by preventing the closure of the epiphyses. GnRH agonists also desensitize the pituitary to GnRH. #$%#%$ A brain MRI may be used to reveal pituitary tumors. Although pituitary tumors may cause presentation similar to this patient, it is infrequent, occurring in only 10% of cases. In patients with breast development, advanced bone age, and early pubic hair growth caused by central nervous system (CNS) lesion, anosmia (inability to smell) will often be present. Spironolactone can be used to treat pubertal acne

< 1 yo UTI

probably vesicureteral reflux

Ehrlichiosis is transmitted by the bite of the dog tick. Symptoms include high fever and headache. An important distinction between human monocytic ehrlichiosis (HME) in kids versus adults: in kids, the rash is seen in about 66%-69% cases versus about 36% in adults. Hence a common triad for children is fever, headache, and rash. Typically the rash ranges from maculopapular to petechial in nature and usually not pruritic

rash may be similar to rocky mountain spotted fever in kids but HME tends to have LEUKOPENIA ass.. tx = doxy

what are indications for using Amox-clav (augmentin) rather than just amoxacillin for AOM

recently treated with antibiotics in the previous 30 days, have a current purulent conjunctivitis, or who have a history of recurrent AOM with H. influenzae. so for our patient we want to Prescribe amoxicillin, 90 mg/kg/day divided into two doses for 10 days

heriditary angioedema = c1 esterase deficiency is inherited in AD.. A 7-year-old girl is brought to the emergency department by her parents because of difficulty breathing and tongue swelling - she is afebrile.. past 2 days abdominal cramping V/D.. - swelling lips, lump in throat onset breathing issues.. - dad had similary episodes in past needed PICU for airway obstruction pending.. - severe abd pain + N/V lasting 4 days.. responds poorly to steroids and racemic epi - plasma bradykinin (vasodilator) -> angioedema.

recurrent episodes of angioedema lasting 2-3 days and episodes of abdominal pain. which are caused by edema in the gastrointestinal mucosa that gradually resolve. - plasma bradykinin levels have been shown to be seven times higher than normal.Histamine and other mast cell mediators are not directly involved, which explains the lack of response to antihistamines and distinguishes this form of angioedema from that associated with urticaria - Patients often require hospitalization and intensive care during such episodes because of the risk of airway obstruction. Edema may also affect the mucosa of the gastrointestinal tract, causing abdominal cramping and pain. - measure C1 esterase inhibitor protein [ ]

DMD is an X-linked recessive genetic disease due to a mutation in the dystrophin gene. It presents in early childhood with progressive proximal muscle weakness and calf pseudohypertrophy. Death usually occurs before the age of 30 what are some complications that -> death?

respiratory muscle failure. complications of DILATED cardiomyopathy (arrhythmias and HF)

________ prodrome of fever, malaise, and distinctive posterior cervical and occipital lymphadenopathy, followed by a rash that begins on the face and spreads downward to the rest of the trunk and limbs.

rubella has

cradle cap

seborrheic dermatitis... best tx w/ selenium sulfide, tar, or zinc pyrithione shampoo after you FRIST do EMOLLIENTS, frequent brushing, and shampoo have failed.

Epiglottitis, historically caused by Haemophilus influenzae type B, is a rapidly progressive infection of the supraglottic structures that may lead to complete airway obstruction how to treat?

securing an airway is the initial step in management, followed by cultures and antibiotics. direct laryngoscopy and intubation... This patient is currently maintaining his airway and is not in respiratory failure. Therefore, emergency surgical airway placement is not yet indicated. In children under 12, the emergency approach of choice is a needle cricothyroidotomy, as this is easier to perform without extensive damage to surrounding laryngeal structures relative to traditional surgical cricothyroidotomy.

contraindications to vaccinations.

severe allergy to a vaccine component or a prior dose of vaccine and encephalopathy within 7 days of prior pertussis vaccination. egg allergies; don't get yellow fever but may get influenza under supervision immunocompromised/ pregnant should not get live vaccines = PO polio, and MMRV... HIV if CD4 > 200 then can get MMR and V (must give separate in this situation) but not PO polio

difference between smallpox and chickenpox

smallpox similar to chickenpox but in smallpox they are all same stage healing.

how to calculate CF patients genetics.. mom don't know (carrier in white population 1 in 25).. dad has cystic fibrosis

so risk would be 1/25 = 5% and then divided by 2 = 2.5%

infant conjugated hyperbiliruibnemia

so rule out sepsis w/ WBC count (CBC) and negative cultures.. once you did that get US to look at biliary atresia (traingular cord sign on US).

what is most common cause of AOM? - may be asymptomatic or myriad symptoms, including ear pulling, ear pain, ear drainage, fever, lethargy, irritability, decreased activity, and decreased appetite

strep pneumo = MC cause - 2nd most common = nontypable H flu - then M. catorrhalis

what is most common cause of otitis media

strep pneumoniae.. - then after that it is H flu and M. Catarrhalis

High-grade reflux in females that persists into adulthood with no clear secondary cause should be managed how?

surgically w/ ureteral re-implantation or ureteronecystostomy! #$%#$% - especially if there are breakthrough UTI's on medical therapy.

Ebstein anomoly (atrialized ventricle...

the ECG shows a delta wave and short PR interval characteristic of WPW syndrome. - WPW syndrome should not include atrioventricular nodal slowing agents because these may make the arrhythmia worse. Procainamide would be the medication of choice. - or patients with symptomatic WPW syndrome, catheter ablation of the accessory pathway is recommended. Ebstein anomoly The presentation varies considerably from heart failure from birth to palpitations much later in life, reflecting the wide variety of possible anatomic variations. - in adults/ adolescents MC presentation= arrhythmia in kids it is like ASD or PS (pulm stenosis)... - Ebstein anomaly is associated with atrial tachyarrhythmia, some of which may be due to Wolff-Parkinson-White (WPW) syndrome Radiofrequency ablation of the pathways responsible for SVT due to Ebstein anomaly is the preferred definitive treatment.

An 18-year-old man with a medical history of recurrent episodes of idiopathic thrombocytopenia purpura visits his doctor because of a 2-month history of joint pain. He reports bilaterally swollen and red knees that are "stiff in the morning." Physical examination reveals a photosensitive rash across his nose. Laboratory examination reveals an antinuclear antibody titer of 1:320 and a platelet count of 57,000/mm³. His temperature is 38.0°C (100.4°F), blood pressure is 115/78 mm Hg, pulse is 88/min, and respiratory rate is 20/min. The patient begins taking a medication to rapidly reduce the symptoms of his disease. Which of the following is a potential adverse effect of long-term use of this first-line medication? - what are other medications for SLE that are not first line and what are their side effects?

this is SLE - corticosteroids = first line therapy.. -> long term effect = avascular necrosis (AVN) shows up as groin pain, thigh pain, butt pain. alopecia (hydroxychloroquine), pancytopenia(methotrexate), and hemorrhagic cystitis (cyclophosphamide), but not corticosteroids (which are 1st line therapy for SLE)

A 15-day-old full-term boy is brought to the clinic by his parents because of shortness of breath, failure to tolerate feeding, and inadequate weight gain. On physical examination there is no cyanosis evident on mucosal membranes. Pulmonary rales are heard bilaterally with an active precordium and a soft 2/6 holosystolic murmur most prominent at the left lower sternal border. ECG is significant for biventricular hypertrophy

this is VSD and not TOF because TOF would have had cyanosis.. also there is decreased pulm blood flow in TOF.. - just RV hypertrophy pulmonary rales heard = pulmonary vascular markings on X-ray.. have increased blood to the lungs! (L -> R shun) = VSD! #$% - both R and L V hypertorphy

acute iron intoxication

this is a life-threatening condition which can present with GI symptoms, shock, metabolic acidosis, hepatotoxicity/hepatic necrosis, and bowel obstruction. The treatment of choice for this condition is deferoxamine. black stools and small radiopaque tablets on abdominal Xray (radiopaque (lights up) Fe tablets)

what is this image of and how to treat?

this is duodenal atresia.. double bubble + bilious vomiting first few hrs of life... - constipation after meconium is passed.. - abdominal distension.. polyhydramniose.. - increased risk in DS patients.. - initial tx = gastric decompression w/ NG tube

A 14-month-old boy presents to the emergency department with his mother for refusal to feed. Earlier in the day, he was playing in the living room with his 4-year-old sister when their mother left briefly to answer the phone. On returning to the living room, she noticed that the children were still playing together with no noticeable change in behavior. Later in the day, the boy refused to feed but was otherwise asymptomatic. His mother denies emesis but does endorse continued flatus and increased drooling. The patient has no significant past medical history, takes no medications, and is up to date on vaccinations. He goes to daycare 5 days a week. In the emergency department, he is afebrile, his heart rate is 115/min, his blood pressure is 100/68 mm Hg, his respiratory rate is 21/min, and oxygen saturation is 100% on room air. On physical exam, heart sounds are normal, breath sounds are clear, normoactive bowel sounds are present, the neck is mildly tender to palpation anteriorly, no lesions are seen in the oropharynx, and the abdomen is non-distended and non-tender. Which of the following is the most logical next step in evaluating this child?

this patient has swollowed foreign body so get - radiologic studies of neck, chest, abdomen. Foreign body ingestion (FBI) usually 6 mo - 3 yo.. - chocking, gagging, coughing at first -> dysphagia, increased salivation, emesis, pain in neck throat, sternal notch, won't eat. if asymptomatic and object inert just ovserve.. if symptoamtic and maybe harmful (battery) -> endoscopic retrieval. if foreign body aspiraiton; dx and therapeutic bornchoscopy immediately!! radiopaque CXR = aspiration also can show air trapping (hyperinflation).. most common place to be lodge = right mainstem bronchus. (image) note the aspiration = in the airways so will have wheezing and stridor and respiratory distres sand hoarseness and stuff.. - the ingestion will just have drooling and tender neck and stuff no airway issues.

Scarlet fever consists of an upper respiratory tract infection that is associated with a characteristic rash. It is caused by Streptococcus pyogenes. The rash often appears within 24-48 hours after symptom onset. It usually spares the face, instead affecting the neck, trunk, and extremities. The rash is a diffuse, erythematous, papular eruption , which is often bright red and blanches on pressure; it is also often accompanied by circumoral pallor

treat w/ penecillin A white tongue with red, swollen papillae (white strawberry) that later progresses to a beefy red (red strawberry) tongue can be seen in a patient with scarlet fever.

how to treat otitis media.. - Children have a shorter, more horizontal Eustachian tube than adults, which predisposes them to OM Streptococcus pneumoniae, Haemophilus influenzae, and Moraxella catarrhalis. - ear pain, hearing loss, and vertigo - Pneumatic otoscopy reveals an immobile tympanic membrane

tx w/ amoxicillin 10 days... - but in those who had B-lactam abx in last 30 days use amox-clav 10 days = first line..

Meningococcemia tx.. N/V, abd pain, fever, hypoTN and skin lesions.. concominant AI -> adrenal cirsis.. hypoNa, hyperK, eosinophilia.. Waterhouse-Friderichsen syndorme... how to tx..

tx w/ hydrocortisone! #$% - don't need to give aldosterone in adrenal crisis don't give platelets in meningococcemia main issue is AI! #$%

how to diagnose congenital HIV

until 18 mo can't use ELISA, so use PCR for congenital HIV dx before 18 mo #$%#$%

w/in first yr of life sudden onset bilious emesis + abd distension, diffuse tender/ guarding...

use GI series to dx intestinal malrotation...

RSV treatment when to give o2 and when not to?

use IV fluids and supportive care.. only use O2 if < 90% SaO2

what do we use fomepizole for?

use it for suspected methanol or ethylene glycol poisoning (these are the 2 w/ gaps) .. inhibits competitively alcohol dehydrogenase first enzyme in metabolism of methaonol and ethylene glycol into toxic metabolites.

Wilms tumor (nephroblastoma) is the most common primary malignant neoplasm of the kidney in childhood workup: dx US follow up w/ CT or MRI to see extent.. - labs electrolytes BUN and Cr, CBC and LFT's (extrarenal involement).. - nephrctomy + cehmo overal cure is like 90% neuroblastoma on other hand = - Abdominal tumors can present with abdominal pain or fullness, constipation, bladder dysfunction, and scrotal or lower-extremity edema...

usually around 3 yo... - painless, does not cross midline.. - HTN, hematuria, fever, wt loss, N/V.. can be part of WAGR (wilm's, aniridia (absent iris), GU issues, Mental retardation).. Beckwith-Wiedemann syndrome; hemihypertrophy, macroclossia, visceromegaly.. - wilm's Denys-Drash: male pseudohermaphroditism, early onset renal filure, mesangial sclerosis and neurofibromatosis.. - wilms

talk about pyloric stenosis onset and electrolytes

usually presents at 3-6 wks of life - met alkalosis -> intracellular shift of H+ out of cells -> and K+ into cells -> lower K+ also we pee out more K+ at the DCT in exchange for H+

sickle cell anemia what is the vaccine you need to use?

vaccine against encapsulated organism; 23-valent polysaccharide vaccine after they have received 2 doses of 13-valent pneumococcal conjugate vaccine (Prevnar 13) - CONJUGATE vacine = Prevnar 13

11β-Hydroxylase deficiency should be suspected in young patients with virilization (females) or precocious puberty (males) and hypertension.

virilization - development of male sex characteristics.. if in young female consider 11B-hydroxylase deficiency.

hypsarrhythmia pattern on an interictal electroencephalogram.

west sydrome = infantile spasms.. most present by 1 yo.. - Patients often exhibit neurodevelopmental delay and cognitive impairment. Seizure types other than spasms may also occur. In addition to the electroencephalogram (EEG), all patients with infantile spasms should undergo neuroimaging to identify any underlying etiology responsible for the patient's symptoms.

When to give Varicella-zoster Ig for neonates?

when they fall into high risk groups 1. moms have signs/ sx of varaella w/in 5 days before or 2 days after delivery 2. hospitalized premature infants born at > 28 wks of gestations whose moms are not immune 3. hospitalized premature infants born < 28 wks gestation or w/ birth wt < 1000 g. healthy full term infant that is > 10 yo don't need ppx if there are no signs/ sx of infection in the baby just need to isolate from mom if mom has chicken pox while she is infectious. Postnatally-acquired varicella is severe in neonates younger than 2 weeks. Therefore, they should be prophylaxed with varicella zoster immunoglobulin. Infants older than 14 days typically have a mild course and require only isolation. Infants should be isolated from the mother for as long as she is infectious (until lesions crust).