pediatrics question bank

A 4 week-old female is seen for a well child visit. Birth history is significant for breech presentation and weighing 9 pounds 8 ounces at delivery. Which of the following is indicated to rule out developmental dysplasia of the hip?

A careful physical examination at birth and repeated evaluation at each well visit until the child walks is indicated to identify DDH. A high degree of suspicion is necessary in children with risk factors for DDH, such as positive family history, ligamentous laxity, breech presentation, female gender, large fetal size, and first-born status.

The birth weight of an infant has usually tripled by

A child triples his/her birthweight by one year

At what age should a child's eyes be consistently well aligned?

A child's eyes should be consistently well-aligned by five to six months of age

Upon stroking of the lateral aspect of the sole from the heel to the ball of the foot, the great toe dorsiflexes and the other toes fan. This is a positive

A Babinski test is performed by stroking the lateral aspect of the sole from the heel to the ball of the foot, the great toe dorsiflexes and the other toes fan in a positive test.

A 4 year-old child presents with a rapid onset of high fever and extremely sore throat. Which of the following findings are suggestive of the diagnosis of epiglottitis?

A croupy cough with drooling in a patient who appears very ill is consistent with epiglottitis. Examining the throat is contraindicated, unless the airway can be maintained.

A 13 year-old female presents to the office with right knee and thigh pain and the inability to bear weight since waking yesterday morning. The mother states the child had a fever of 100.9 degrees F this morning and continues to be non- weight bearing. Examination reveals a warm, erythematous, swollen knee. Which of the following tests would be most beneficial in the diagnosis and treatment of this patient?

A culture of the joint fluid will confirm the diagnosis and offer information regarding infectious agent.

In the neonate, unequal thigh folds may indicate which of the following?

A dislocated hip displaces proximally in developmental hip dysplasia, causing a shortening of the leg that may present as unequal thigh folds.

An x-ray reveals a break in the cortex of one side of the ulna shaft without a separation or break of the opposite cortex describes what type of fracture?

A greenstick fracture is a break in the cortex of one side of bone shaft without a break in the opposite cortex.

A mother brings a 3 month-old infant to the office because she is concerned about a red, vascular, nodular growth on the child's back. It appears to be enlarging slightly and the vessels are slightly dilated. It seems to cause the child no discomfort. The most likely diagnosis is

A hemangioma is a bright red to deep purple vascular nodule or plaque that often develops at birth, may enlarge, and may regress and disappear with aging.

A 6 month old male presents with a scrotal mass. The scrotum is swollen and testicles are non-tender. The scrotum does transilluminate. Which of the following is the treatment of choice?

A hydrocele presents with a swollen, non-tender scrotum. Due to the scrotum being filled with fluid, the mass will transilluminate. No treatment is required unless the hydrocele persists after the age of 2 years.

Which of the following primitive reflexes should begin to disappear at about 2-3 months of age in a normal infant?

A newborn infant will turn its head toward anything that strokes its cheek or mouth, searching for the object by moving its head in steadily decreasing arcs until the object is found. The rooting reflex starts to disappear at about 2-3 months of age.

Which of the following is the most appropriate study for diagnosing Hirschsprung disease?

A rectal biopsy showing the absence of ganglion cells in both the submucosal and muscular layers of the involved bowel is the most appropriate diagnostic study for Hirschsprung disease.

Which of the following is the treatment of choice for a torus (buckle) fracture involving the distal radius?

A torus or buckle fracture occurs after a minor fall on the hand. These fractures are very stable and are not as painful as unstable fractures. They heal uneventfully in 3-4 weeks

An obese 11-year-old Cherokee boy presents with symmetrical, hyperpigmented, velvety plaques in the intertriginous areas of the axilla, groin, and posterior neck. There are a few acrochordons scattered in the plaques. The plaques are painless, and do not itch. What is the most likely diagnosis?

Acanthosis nigricans is a thickening and hyperpigmentation of the intertriginous regions of the skin. Acanthosis nigricans is more common in those with Native American, African-American, or Hispanic heritages than in Caucasians. The condition is commonly associated with other conditions such as insulin resistance and obesity, but it has also been associated with certain genetic syndromes, malignancies and drug reactions. Since acanthosis nigricans is often an asymptomatic disorder, cosmetic concerns are typically the primary indications for treatment. Treatment of the underlying cause is the preferred method of management. Otherwise, topical retinoids are the preferred treatment of the plaques because of their keratinolytic effects. Cutaneous hyperpigmentation is present in nearly all patients with Addison's disease (B) because the cortisol deficiency leads to an overproduction of melanin. The hyperpigmentation is generalized across the entire body with Addison's, but it is most conspicuous in areas of high friction or pressure, such as the elbows, knees, waist and knuckles. Most patients with Addison's complain of fatigue, weakness, anorexia and weight loss. These symptoms and the generalized nature of the rash are not consistent with our patient. Dowling-Degos disease (C) is a rare, genetic disorder that presents with reticulated hyperpigmentation and has a predilection for flexural areas. This reticulated pattern is not consistent with the plaques seen on the presented patient. Lesions also typically appear in early adulthood, not at 11-years-old. Pellagra (D) is also known as niacin (vitamin B3) deficiency. Pellagra presents with a symmetric hyperpigmented rash, in the exposed areas of skin. The rash is a photosensitive dermatitis and thus only appears where there is sun exposure. The patient may also have a red tongue, diarrhea, vomiting, insomnia, anxiety, disorientation, delusions, dementia, and encephalopathy. Niacin deficiency is very rare in the Western world but may be seen with alcoholism, anorexia nervosa, gastric bypass or malabsorptive diseases.

Who is the most likely adult to sexually abuse a child?

Adults within the immediate or extended family perpetrate the most child sexual abuse, usually this is a trusted member of the family.

Which of the following is the treatment of choice for stage one Lyme disease in a patient less than 8 years of age?

Amoxicillin or cefuroxime are first-line therapy in a patient less than 8 years of age due to the harmful effects of doxycycline on teeth and bones in children.

A 4-year-old presents with severe pain in the left ear. The tympanic membrane is red, dull, bulging, and immobile. The child has no known drug allergies. The most appropriate first-line drug would be

Amoxicillin will cover the most common pathogens and is considered first-line therapy.

A 3-month-old female is brought by her mom for a routine physical examination. The patient's mom has no complaints. On examination you note a well-developed, well-nourished infant in no apparent distress. There is no cyanosis noted. Heart examination reveals a normal S1 with a physiologically split S2. There is a grade III/VI high-pitched, harsh, pansystolic murmur heard best at the 3rd and 4th left intercostal spaces with radiation across the precordium. Which of the following is the initial diagnostic study of choice in this patient?

An echocardiogram is the initial diagnostic study of choice in the diagnosis of a VSD.

A newborn weighs 8 pounds at birth. On average, what should the infant weigh at 1 year of age?

An infant will triple birth weight within the first year of life. A newborn that weighs 8 pounds at birth will weigh approximately 24 pounds at 1 year of age.

A foreign body lodged in the trachea that is causing partial obstruction will most likely produce what physical examination finding?

An inspiratory wheeze is called stridor, which indicates a partial obstruction of the trachea or larynx.

A 3 week-old male infant presents with recurrent regurgitation after feeding that has progressed to projectile vomiting in the last few days. The mother states that the child appears hungry all of the time. She denies any diarrhea in the child. Which of the following clinical findings is most likely?

An olive-sized mass may be palpated in the right upper abdomen in pyloric stenosis and if found, is pathognomonic for pyloric stenosis.

A 4 year-old patient presents with episodic wheezing and a non-productive cough for the last 4 weeks. His symptoms are worse at night. Past medical history reveals a history of atopic dermatitis. Physical examination at this time is unremarkable. Which of the following is the most likely diagnosis?

Asthma is a chronic inflammatory disorder of the airways. It is characterized by episodic or chronic symptoms of airflow obstruction, breathlessness, cough, wheezing, and chest tightness. The strongest identifiable predisposing factor for the development of asthma is atopy.

A mother brings her 6 year-old boy for evaluation of school behavior problems. She says the teacher told her that the boy does not pay attention in class, that he gets up and runs around the room when the rest of the children are listening to a story, and that he seems to be easily distracted by events outside or in the hall. He refuses to remain in his seat during class, and occasionally sits under his desk or crawls around under a table. The teacher told the mother this behavior is interfering with the child's ability to function in the classroom and to learn. The mother states that she has noticed some of these behaviors at home, including his inability to watch his favorite cartoon program all the way through. Which of the following is the most likely diagnosis?

Attention deficit hyperactivity disorder is characterized by inattention, including increased distractibility and difficulty sustaining attention; poor impulse control and decreased self-inhibitory capacity; and motor overactivity and motor restlessness, which are pervasive and interfere with the individual's ability to function under normal circumstances.

A 2 month-old infant presents for a routine health maintenance visit. The mother has been concerned about the infant's hearing since birth. Physical examination reveals no apparent response to a sudden loud sound. Which of the following is the most appropriate diagnostic evaluation?

Brainstem auditory-evoked potentials evaluate the sensory pathway and identify the site of any anatomical disruption. The test does not require any active response from the patient and is useful in the evaluation of suspected hearing loss in an infant.

Which of the following clinical manifestations is most commonly seen in viral croup?

Viral croup typically presents with a barking cough and stridor

A 2-year-old child presents to the emergency department with increasing respiratory distress. The mother states that the child had a "cold" 2 weeks ago. Last week the cough progressed and is described as barky in nature, associated with stridor. The child appeared to be getting better, but last night,developed a fever and increased respiratory distress. Physical examination reveals a temperature of 102°F. The child is in moderate respiratory distress. A portable lateral neck x-ray film reveals severe subglottic and tracheal narrowing. Which of the following is the most likely diagnosis?

Bacterial tracheitis usually presents following a viral upper respiratory infection, especially laryngotracheobronchitis (croup). It should be suspected when a patient develops high fever and respiratory distress after a few days of apparent improvement or if the patient fails to respond to the usual treatment for croup. The findings of subglottic and tracheal narrowing on the lateral neck x-ray film highly support this diagnosis.

A 3-year-old boy is brought to the urgent care clinic by his mother. She tells you he has had a fever and cough for the past 2 days. His temperature is 38.6°C (101.5°F) and his respiratory rate is 35/min. Auscultation of the lungs reveals inspiratory stridor with a prolonged inspiratory phase. A chest X-ray shows subglottic narrowing. Which of the following is most likely the best treatment for this child's condition?

Based on the constellation of findings, this patient most likely has croup (laryngotracheobronchitis). Croup typically presents with the classic triad of barking cough, inspiratory stridor, and hoarseness. It is most often caused by a viral infection (parainfluenza virus). Outpatient treatment usually consists of oral steroids (eg, oral dexamethasone) or nebulized epinephrine for severe cases. Corticosteroids are beneficial due to their anti-inflammatory actions. Since laryngeal edema is commonly seen in patients with croup, corticosteroids are extremely effective in decreasing this inflammatory process. Improvement is typically seen within 6 hours after administration of oral steroids. Since most cases of croup are caused by viruses, the use of antibiotics (A) is not indicated. Antibiotics should only be used to treat specific bacterial complications. Anticholinergics (B) (eg, tiotropium, ipratropium bromide) are long-acting bronchodilators used in the management of chronic obstructive pulmonary disease (COPD). Anticholinergics are not used in the treatment of croup. Bronchodilators (C) are typically used in the treatment (and management) of a variety of obstructive lung diseases (eg, asthma, COPD). They are not indicated in the treatment of croup.

An infant presents with bullous impetigo involving the face, extremities, and trunk. Which of the following is the best treatment for this child?

Bullous impetigo that is localized may be treated with topical mupirocin, but extensive involvement is best treated with oral antibiotics that are penicillinase-resistant, such as dicloxacillin.

A 6-year-old child with sickle cell anemia presents with fever and pain over the right tibia. There is tenderness along the anterior tibia, but no pain with motion of the knee or ankle. Which of the following is the most appropriate initial treatment?

Cefotaxime provides coverage for osteomyelitis caused by staphylococcus or salmonella.

A 5 year-old is being evaluated for autism. Of the following, the finding most consistent with this diagnosis is

Children with autism do not tend to make eye contact, and even avoid it.

A mother brings in her five year-old boy for his school physical. She voices some concerns about his readiness for school, saying he seems to be socially immature. She has noticed he does not interact with other children well, and that when he plays with them, he has a tendency to "place them" and then run around them as if they were statues. He rarely cries when he is hurt, and he shrugs off any attempt to hug him. He has good attention to details, and will sit and draw the same geometric shapes over and over again, but does not seem interested in learning the alphabet. He avoids eye contact with anyone. Which of the following is the most likely diagnosis?

Children with autism do not tend to make eye contact, and even avoid it. They do not accept comfort when hurt and stiffen up when hugged. They do not tend to play with others, and do not tend to imitate grown-ups in play. They approach play in a more mechanical way, using others as props rather than interacting with them.

A 12 year-old boy presents to the office with pain in his legs with activity gradually becoming worse over the past month. He is unable to ride a bicycle with his friends due to the pain in his legs. Examination of the heart reveals an ejection click and accentuation of the second heart sound. Femoral pulses are weak and delayed compared to the brachial pulses. Blood pressure obtained in both arms is elevated. Chest x-ray reveals rib notching. Which of the following is the most likely diagnosis?

Coarctation is a discrete or long segment of narrowing adjacent to the left subclavian artery. As a result of the coarctation, systemic collaterals develop. X-ray findings occur from the dilated and pulsatile intercostal arteries and the "3" is due to the coarctation site with proximal and distal dilations.

A 24 year-old male presents for routine physical examination. On physical examination, you find that the patient's upper extremity blood pressure is higher than the blood pressure in the lower extremity. Heart exam reveals a late systolic murmur heard best posteriorly. What is the most likely diagnosis in this patient?

Coarctation of the aorta commonly presents with higher systolic pressures in the upper extremities than the lower extremities and absent or weak femoral pulses

An 8-year old male presents with pain in his legs after exercise. Physical examination reveals radial-femoral pulse delay and the blood pressure in the lower extremities is 15 mmHg lower than in the upper extremities. Which of the following lab or radiological findings would be most commonly noted in this patient?

Coarctation of the aorta presents with a disparity in both pulses and blood pressure between the upper and lower extremities. Laboratory or radiologic findings include inferior border rib notching, cardiomegaly, and ventricular hypertrophy on EKG.

A 16 year-old male is brought into your office by his girlfriend. She states that "he hasn't been himself lately" and seems to fluctuate from being almost "euphoric" to being depressed and irritable. The patient states that "he is really okay" and that he "just feels a little irritable occasionally." On physical examination his pulse is 120 beats/minute, blood pressure is 180/110 mmHg, he is sweating and his pupils are widely dilated. Which of the following is the most likely diagnosis?

Cocaine is a stimulant and presenting clinical manifestations of intoxication include agitation, tachycardia, hypertension, diaphoresis, and dilated pupils.

A woman brings her 3 month-old son to the clinic. Upon examination, it is noted he has a round face, a large protruding tongue, dry skin, an umbilical hernia, and his weight gain is below average. He appears apathetic and the mother says the infant is usually constipated. Which of the following is the most likely diagnosis?

Congential hypothyroidism presents gradually, and at 3-6 months findings include poor appetite and feeding, sluggishness, constipation, enlarged abdomen and umbilical hernia, enlarged tongue, and the child does not meet developmental milestones.

Which of the following results from the deposition of unconjugated bilirubin in the brain?

Kernicturus results from the deposition of unconjugated bilirubin in the basal ganglia and brainstem. Rett's syndrome is a neurodegenerative disorder of uknown cause. Neurocysticercosis is caused by infection with a tapeworm. Struge-Weber syndrome is caused by abnormal development of meningeal vasculature

The most accurate way to determine the exact degree of spinal curvature in a child with scoliosis is by which of the following?

The scoliotic curve is measured by the Cobb method using AP and lateral x-ray films of the entire length of the spine.

A 7 year-old boy wets the bed nearly every night. Which of the following is the best pharmaceutical agent to use in treating this patient?

Desmopressin, while not curative, will relieve symptoms.

Which of the following is a common side effect of the psychostimulants?

The side effects of the psychostimulants, such as Ritalin, include appetite suppression, weight loss, and sleep disturbances

A 5-year-old boy presents to the office for a follow-up of complaints of pruritus in the perianal area that is worse at night. A cellophane tape test is positive for Enterobius vermicularis. Which of the following is the treatment of choice?

Treatment of choice for enterobiasis (pinworms) is mebendazole

Small grayish vesicles and punched-out ulcers in the posterior pharynx in a child with pharyngitis is representative of which organism?

Coxsackievirus presents with small grayish vesicles and punched-out ulcers in the posterior pharynx.

A 14-year-old male is brought to an endocrinologist because his parents are concerned he is "too short." His pediatrician is contacted for his medical records, and his growth chart is obtained. If this patient's short stature is secondary to constitutional growth delay, which of the following curves would likely correspond to this presentation?

Curve B corresponds to constitutional growth delay, attaining a normal height by 18 years. The most common cause of short stature and pubertal delay is constitutional growth delay. This condition has a classic presentation on growth charts: normal birth weight and height, drop in percentiles on growth curve between 6 months and 3 years, re-establishment of normal growth velocity, and following the growth curve at the 5th to 10th percentiles. Adolescents will have a normal growth spurt and normal adult height. Constitutional growth delay is characterized by a bone age that is less than chronological age; the child's short height is often appropriate when assessed in the context of their skeletal age. The only management is reassurance and regular follow-up.

A 6 year-old female presents to the clinic with her parents because of lower back pain and difficulty walking for one week. The pain is described as a dull ache that is constant in the midlumbar region with radiation into the abdomen. She has a low grade fever of 100 degrees F which the parents note has been persistent for several days. Upon exam it is noted that the child has restricted forward flexion and extension secondary to pain. She has tenderness over the L3 vertebrae. An area of cellulitis from an insect bite is discovered over her left scapula. Which of the following is the most likely diagnosis?

Discitis is an infectious disease that occurs in the mid lumbar region of children at the age of about 6 years old. Symptoms are low back pain that radiates into the abdomen or lower extremity. A low-grade fever with nausea and vomiting occasionally occur.

A 4 year-old boy presents to the ED after sustaining a crush injury to his distal third phalanx. Physical exam reveals an associated nail bed injury. Which of the following is the appropriate management?

Distal phalanx fracture should be immobilized and if there is an associated nailbed injury the fracture is considered "open" and the patient should be given antibiotics and follow-up with ortho in one week

Which of the following groups is most likely to present with Duchenne's muscular dystrophy?

Duchenne's muscular dystrophy, a genetic defect on the short arm of the X chromosome, affects toddler-aged males.

A 1-day-old develops bilious vomiting without abdominal distension. Abdominal xray reveals a double-bubble sign. Which of the following is the intervention of choice for this patient?

Duodenal atresia presents within the first day of life with bilious vomiting without abdominal distention . A double-bubble sign is noted on abdominal xray film. Treatment of choice is a duodenoduodenostomy.

An 8-year-old boy is brought to a physician because of palpitation, fatigue, and dyspnea. On examination, a continuous machinery murmur is heard best in the second left intercostal space and is widely transmitted over the precordium. The most likely diagnosis is

Patent ductus arteriosus is classically described in children as a continuous machinery-type murmur that is widely transmitted across the precordium.

A 23 month-old male presents to the office with his father who reports the patient has had an acute onset of severe pharyngitis, fever of 103.5 degrees F and what sounds like harsh, high-pitched breath sounds. His dad states the child has started drooling and seems to be worsening. The child is not presently crying but has muffled voice sounds. The child has not been immunized due to religious reasons. What is the most appropriate next step?

Epiglottitis requires endotracheal intubation to maintain the airway but should be performed only in the operating room or emergency room with a competent physician prepared to place an endotracheal tube or less often to perform a tracheostomy.

Which of the following laboratory abnormalities is most commonly noted in bulimia nervosa?

Episodes of binge eating are followed by purging in the bulimic patient. Vomiting and laxative abuse are the most common methods of purging, leading to hypokalemia.

A young child is brought to the clinic because the mother noticed a rash while bathing the child. There is a very red slightly raised eruption on the child's face across both cheeks. The child has been in good health and does not appear ill today. The most likely diagnosis is

Erythema infectiosum is generally asymptomatic, presenting with red papules on the face that coalesce to give a "slapped cheek" appearance.

A 2 month-old infant has been diagnosed with pneumonia due to Chlamydia trachomatis. Which of the following is the treatment of choice?

Erythromycin or sulfisoxazole is the treatment of choice for an infant with Chlamydial pneumonia.

A 5 year-old child has just been hospitalized with meningococcemia. Family members and close contacts should be given which of the following as prophylaxis?

Exposed household, school, or day-care contacts of children with meningococcemia should receive chemoprophylaxis to eliminate the organism from the nasopharynx. The drug of choice is rifampin (10 mg/kg, up to 600 mg) every 12 hours for 48 hours. Sulfisoxazole, ceftriaxone, and ciprofloxacin are alternative choices for prophylaxis.

A 19 year-old woman has been consuming up to six beers daily since she was 16. She is now pregnant with her first child, has had little prenatal care, and is due to deliver in four weeks. Of the following, which neonatal problems should you anticipate?

Fetal alcohol syndrome is a common cause of low birth weight.

A 20 year-old female presents with episodes of binge eating, overuse of laxatives, and periods of starvation. Which of the following is the best treatment option for this patient?

Fluoxetine, a SSRI, is the drug of choice for the treatment of bulimia nervosa

An 8-year-old male presents to a geneticist for further workup of a series of developmental delays, learning disabilities, and dysmorphic features. His past medical history is significant for delays in motor development, including sitting without support at 10 months and walking at 20 months. Academically, he is currently struggling with math in school. On exam, he displays a long, thin, face, a large jaw, and protruding ears seen here. Given this patient's likely diagnosis, what physical finding is he likely to demonstrate following puberty?

Given the patient's characteristic physical features of a long face, protruding ears, male sex, and learning disability, he likely has Fragile X syndrome. Following puberty, these individuals characteristically have macroorchidism. Fragile X is caused by CGG trinucleotide expansion within the FMR1 gene located on the X chromosome. Affected individuals have characteristic physical features such as macrocephaly, a large jaw, frontal bossing, and macroorchidism following puberty. Additionally, affected individuals may also exhibit strabismus, connective tissue dysplasia including mitral valve prolapse and hyperextensible joints. They may also exhibit specific behaviors such as attention deficit, hand flapping, hand biting, and averting one's gaze. Most affected males have mild to severe mental retardation.

There is considerable debate about the use of tympanostomy tubes in the management of recurrent otitis media in children. Tympanostomy tube placement has been proven to

Hearing is improved with tympanostomy tubes by eliminating middle ear effusion when the tubes are functioning properly.

A 6-year-old male presents with hemarthrosis of the left knee. Coag studies: PT 12.5s (normal12-14 sec), INR 1.0, aPTT 58s (normal 18-28 sec), platelet 430,000/microliter (normal 150,000-450,000/microliter), and bleeding time 4m (normal 2-12m). Which is the best treatment option for this patient?

Hemophilia A presents with a prolonged aPTT and normal platelet count and function. Hemophilia A is treated with factor VIII concentrate or cryoprecipitate

At what age should the first hepatitis B vaccine be administered

Hepatitis B vaccine is first given at birth, then at 1 to 2 months, and again at 6 to 18 months

A 2 year-old female presents with purulent nasal discharge bilaterally with fever and cough for several days. Her mom had taken her out of daycare for a similar occurrence 2 months ago, that was treated with Amoxicillin. Exam further reveals halitosis and periorbital edema. Treatment should be initiated with which of the following?

High dose amoxicillin-clavulanate is the treatment of choice for resistant bacterial sinusitis, especially in children presenting with risk factors (daycare attendance, previous antibiotic treatment 1-3 months prior, age younger than 2 years).

Which of the following is the treatment of choice for homocystinuria?

Homocystinuria is a disorder of amino acid metabolism and is best treated with high doses of Vitamin B6

A 3 year-old child playing in an abandoned shed is bitten by a black widow spider. The mother rushes the child to the emergency department within 20 minutes of the incident. Which of the following if the best initial intervention?

Hospital admission for symptomatic care should be considered in children, pregnant women, and patients with preexisting cardiovascular disease.

Which of the following is the pathophysiologic mechanism of hyaline membrane disease?

Hyaline membrane disease (Resp distress syndrome) results from alveoli collapse due to lack of adequate lung surfactant and immature lungs

You are called to the nursery to see a male infant, born by uncomplicated vaginal delivery. He weighs 2,600 grams and has one deep crease on the anterior third of each foot. Respirations are 88 breaths/minute with expiratory grunting and intercostals retractions. He is cyanotic on room air and becomes pink when placed on 60% oxygen. Chest x-ray shows atelectasis with air bronchograms. Which of the following is the most likely diagnosis?

Hyaline membrane disease is the most common cause of respiratory distress in the premature infant. The infant typically presents with tachypnea, cyanosis and expiratory grunting. A chest x-ray reveals hypoexpansion and air bronchograms.

A 9 year-old boy who has had cold-like symptoms for the past few days is brought to the clinic by his mother who states that her son had gross hematuria this morning. Prior to the cold-like symptoms the boy has been in excellent health. He is up-to-date on all of his immunizations. The patient does not have any edema, hypertension or purpura. Urinalysis reveals the urine to be cola-colored with a 2+ positive protein and 2+ hemoglobin. Microscopic analysis reveals 50-100 RBCs/HPF, no WBCs, bacteria, casts or crystals. What is the most likely diagnosis?

IgA nephropathy presents after an upper respiratory illness with deposition of IgA within the mesangium of the glomerulus.

Which of the following is the most important measure in the prevention of epiglottitis?

Immunization with the HIB vaccine has greatly reduced Haemophilus influenzae as a cause of epiglottitis

A 3-year-old presents with sore throat and fever. The child appears toxic and is having trouble handling oral secretions. Which of the following is the next most appropriate step in the evaluation of this patient?

In a patient with suspected epiglottis, lateral neck x-ray films may be helpful in demonstrating a classic "thumbprint" sign that will guide intervention

A severely dehydrated child with gastroenteritis who is unable to tolerate oral rehydration should receive which of the following intravenous therapies?

In a severely dehydrated child, restoring intravascular volume to ensure adequate tissue perfusion is the immediate objective. This is best done with either Ringers lactate or normal saline. The addition of potassium would only be done after initial fluid boluses and after ensuring adequate kidney function. The addition of glucose to the IV solution may result in an osmotic diuresis worsening the dehydration.

A 9 year-old presents with increasing shortness of breath while playing basketball recently. On examination, radial pulses are exaggerated while femoral pulsations are weak. Chest radiograph shows rib notching and a mildly enlarged heart. Which of the following is the most likely diagnosis for this patient?

In older children, the ECG and chest x-ray usually show left ventricular hypertrophy and a mildly enlargedheart. Rib notching may also be seen in older children (>8 years old) with large collaterals.

The most definitive treatment for primary enuresis is

Intranasal desmopressin is effective in 50% of patients treated and is the treatment of choice.

Which of the following is an indication for hospitalization in a patient who has acute bronchiolitis?

Indications for hospitalization include moderate tachypnea with feeding difficulties.

In infants, the eyes should move in parallel without deviation by the age of

Intermittent alternating convergent strabismus is frequently noted for the first 6 months of life, but referral is indicated if it persists beyond 6 months.

An 18 month old presents with abdominal pain and bloody diarrhea. On physical examination a sausage shaped mass is noted in the upper mid-abdomen. Which of the following is most likely the diagnosis?

Intussusception, telescoping of proximal bowel into distal bowel, is most common in children younger than age 2, who present with abdominal pain and bloody "currant" jelly" stool. On physical examination a sausage-shaped mass is noted in the mid abdomen. Patients with pyloric stenosis presents with vomiting and an olive size mass in the mid-abdomen. Those with Hirschsprung's disease present with vomiting and abdominal distention. Duodenal atresia is noted in the first day of life, with vomiting without abdominal distension

The initial sign or symptom of iron poisoning in a 3 year-old child is usually

Iron causes localized necrosis and hemorrhage at the point of contact in the GI system resulting in abdominal pain, vomiting, bloody diarrhea, and hematemesis.

A mother's concern about her child's choppy, gasping snore prompts the practitioner to suspect the possibility of which of the following as the cause of respiratory obstruction?

Large adenoids are considered part of the differential diagnosis of snoring and upper airway obstruction.

A 2-year-old presents with sudden onset of cough and stridor. On examination the child is afebrile and appears well with a respiratory rate of 42 per minute. What is the next best step in the evaluation and treatment of this patient?

Laryngoscopy is indicated not only for diagnosis, but also removal of the foreign body.

What scabicide has been associated with neurotoxicity in infants and young children?

Lindane (Kwell) is concentrated in the CNS and toxicity from systemic absorption in infants has been reported.

A 6 year-old boy is brought to the pediatric clinic by his mother for an evaluation of his asthma. He coughs about 3 days out of the week with at least 2-3 nights of coughing. Which of the following would be the most appropriate treatment for this patient?

Low dose inhaled corticosteroids are the preferred treatment for mild persistent asthma.

A 3-week-old male is brought to your office because of a sudden onset of bilious vomiting of several hours duration. He is irritable and refuses to breastfeed, but stools have been normal. He was delivered at term after a normal pregnancy, and has no health problems to date. A physical examination shows a fussy child with a distended abdomen. Radiography of the abdomen shows a "double bubble" sign. Which one of the following is the most likely diagnosis?

Midgut volvulus may present in one of three ways: as a sudden onset of bilious vomiting and abdominal pain in a neonate; as a history of "feeding problems" with bilious vomiting that appears to be a bowel obstruction; or less commonly, as failure to thrive with severe feeding intolerance. The classic finding on abdominal plain films is the "double bubble" sign, which shows a paucity of gas (airless abdomen) with two air bubbles, one in the stomach and one in the duodenum. However, the plain film can be entirely normal. The upper gastrointestinal contrast study is considered the gold standard for diagnosing volvulus. Infantile colic (A) usually begins during the second week of life and typically occurs in the evening. It is characterized by screaming episodes and a distended or tight abdomen. Its etiology has yet to be determined. There are no abnormalities on physical examination and ancillary studies, and symptoms usually resolve spontaneously around 12 weeks of age. Necrotizing enterocolitis (D) is typically seen in the distressed neonate in the intensive-care nursery, but it may occasionally be seen in the healthy neonate within the first 2 weeks of life. The child will appear ill, with symptoms including irritability, poor feeding, a distended abdomen, and bloody stools. Abdominal plain films will show pneumatosis intestinalis, caused by gas in the intestinal wall, which is diagnostic of the condition. Intussusception (B) is seen most frequently between the ages of 3 months and 5 years, with 60% of cases occurring in the first year and a peak incidence at 6-11 months of age. The disorder occurs predominantly in males. The classic triad of intermittent colicky abdominal pain, vomiting, and bloody, mucous stools is encountered in only 20%-40% of cases. At least two of these findings will be present in approximately 60% of patients. The abdomen may be distended and tender, and there may be an elongated mass in the right upper or lower quadrants. Rectal examination may reveal either occult blood or frankly bloody, foul-smelling stool, classically described as "currant jelly." An air enema using fluoroscopic guidance is useful for both diagnosis and treatment.

Physical exam findings in a 4-year-old child that include blue sclerae and recurrent fractures indicate which of the following?

Mild osteogenesis imperfecta presents with blue sclerae, history of recurrent fractures and presenile deafness.

While examining a newborn, the PA notes symmetrical abduction of the upper extremities and extension of the fingers when the newborn's head is allowed to drop a few centimeters while supporting the body. This describes which of the following reflexes?

Moro's reflex is noted by symmetrical abduction of the upper extremities and extension of the fingers after dropping the head a few centimeters while supporting the body. The parachute reflex is noted by extension of all extremities with forward flexing of the infant as if to fall. Galant's reflex is noted by lateral curvature of the trunk with stroking one side of the back. Placing response is noted by flexion of the knee and hip and placing the foot on the table when allowing the newborn's feet to lightly touch the surface of the table.

Which of the following is the leading cause of injury-related death in children between the ages of 1 and 15?

Motor vehicle injuries are the leading cause of death in children.

An afebrile 2 year-old female presents with a three-day history of foul smelling, blood-tinged, mucoid drainage from the left nostril. Which of the following is the most likely diagnosis in this patient?

Nasal foreign body typically presents in children under 3 years of age. The symptoms include mucopurulent drainage, epistaxis, foul odor and nasal obstruction.

A 9 year-old male is brought in by his mother who reports the patient has exhibited an extremely negative attitude for the past year. He seems angry much of the time and frequently loses his temper. Arguing over even trivial details is common place and he seems to take delight in annoying his family. His grades and conduct at school remain excellent. He has few friends, though he has never been seen bullying or destroying others' property. What is the most likely diagnosis?

Oppositional defiant disorder (ODD) best fits this scenario and is differentiated from conduct disorder by the lack of bullying and the lack of destruction of property. Many children with ODD do drift into conduct disorders over time. His good grades and conduct at school lessen the probability of untreated ADD. Personality disorders (i.e. antisocial personality disorder) can not be diagnosed at this early an age.

A 14-year old male active in sports, has been complaining of intermittent anterior right knee pain for several months. He denies any specific injuries. On examination, there is no erythema, swelling, deformities, joint laxity, or crepitus. Palpation reveals tenderness over the tibial tubercle and bursa of the right knee. This finding is characteristic of which of the following disorders?

Osgood-Schlatter disease causes pain at the tibial tubercle and it is caused by fragmentation of the tip of the proximal tibial physis.

A 10-year-old male presents with pain in his left leg that is worse at night. Aspirin relieves the pain and the patient denies injury. On examination, there is point tenderness over the tibia, and the patient has a slight limp that favors the left leg. Radiographs show a 1 cm radiolucent nidus surrounded by osteosclerosis. The most likely diagnosis is

Osteoid osteoma is a benign tumor in children age 5 to 20, presents with increasing pain, worse at night and relieved by aspirin

A 12 year-old male presents with pain in his left leg that is worse at night. Aspirin relieves the pain and the patient denies injury. On examination, there is point tenderness over the tibia, and the patient has a slight limp that favors the left leg. Radiographs show a 1 cm radiolucent nidus surrounded by osteosclerosis. Which of the following is the most likely diagnosis?

Osteoid osteoma is a benign tumor in children age 5 to 20, presents with increasing pain, worse at night and relieved by aspirin.

A 15-year-old boy who runs on his high school cross-country team presents to your office complaining of pain just below his right knee. He states that the pain started 3 months ago and is worse at night. He denies any recent injuries or trauma. You order an X-ray and obtain the image below. Which of the following is the most likely diagnosis?

Osteosarcoma is the third most common malignancy of adolescents and is the most common malignant bone tumor in adolescents. It often occurs around the time of growth spurts (adolescents) and patients usually present after several months of pain. Teenagers who are active in sports tend to complain about pain in their lower femur, or right below the knee. If the tumor is large, it can appear as a swelling. The affected bone is not as strong as normal bones and may fracture with minor trauma. There are no systemic symptoms. Most commonly affected bones are the metaphyses of long tubular bones (femur, tibia or humerus). The classic finding on X-ray is a "sunburst" pattern due to the aggressive lytic bones lesions that form that are associated with periosteal reaction. MRI is the most sensitive test to determine the extent of the tumor, but a tissue sample is required to confirm the diagnosis. Micrometastases are common at presentation so treatment often includes surgical resection of the tumor and chemotherapy. Ewing sarcoma (A) is less common and usually affects the diaphyses of long bones. It has a classic "onion skinning" pattern on X-ray. Osgood-Schlatter disease (B) is due to microfractures of the tibial tubercle from running and jumping activities, often in adolescents. Pain is worse with activity and often is so bad that activities must be stopped. Patellar tendonitis (D) is an overuse injury also caused by running or jumping. It is characterized by tenderness over the patellar tendon and occurs with activity. Imaging will be normal.

A newborn is seen for an initial two week visit. Physical examination reveals a thrill and a continuous machinery murmur in the left second intercostal space. Which of the following is the most likely diagnosis?

Patent ductus arteriosus is characterized by a classic harsh, machinery-like murmur that is continuous through systole and diastole. This is heard best at the left second interspace and is commonly associated with a thrill.

An 8 year-old boy is brought to a health care provider complaining of dyspnea and fatigue. On physical examination, a continuous machinery murmur is heard best in the second left intercostal space and is widely transmitted over the precordium. The most likely diagnosis is

Patent ductus arteriosus is classically described in children as a continuous machinery-type murmur that is widely transmitted across the precordium

Which of the following is the treatment of choice for Kawaski's disease?

Patients with Kawasaki's disease present with fever, bilateral conjunctival injection, pharyngeal erythema, edema of the hands and feet, rash, and LAD. Tx of choice is high-dose aspirin and IV immunoglobulin

Which of the following leads to retropatellar pain?

Patients with an increased Q angle have more force directed laterally during knee flexion and are at greater risk of patellofemoral pain syndrome.

Which of the following therapies is recommended for a 13 month-old child with sickle cell disease?

Patients with sickle cell disease should receive prophylactic penicillin V starting at 2 months of age and folic acid starting at 1 year of age. Ferrous sulfate is not globally recommended for patients with sickle cell disease.

A 3 year-old presents with a 24-hour history of diarrhea. The patient is afebrile and the stool is noted to be loose and watery. No blood is noted in the stool. Fecal WBC is negative. Which of the following is the most likely diagnosis?

Patients with viral gastroenteritis are afebrile and noted to have loose, watery, non-bloody diarrhea. Fecal WBC is negative

A 4-year-old patient presents with a sore throat and fever. Throat culture shows Group A beta-hemolytic streptococcus. Which of the following is the treatment of choice?

Penicillin VK 250 mg po qid for 10 days is the recommended treatment for streptococcal pharyngitis. Erythromycin is an alternative in patients allergic to penicillin.

Annual blood pressure determinations should be obtained beginning at the age of

Periodic measurements of blood pressure should be part of routine preventive health assessments beginning at the age of 3 years.

A 13 year-old boy with leukemia presents with epistaxis for 2 hours. The bleeding site appears to be from Kiesselbach's area. The most appropriate intervention is

Petrolatum gauze will provide pressure to the bleeding point while the cause of bleeding is corrected.

Which of the following is an indication for a pediatric patient to receive the 23-valent polysaccharide vaccine (Pneumovax)?

Pneumovax is licensed for use in children over the age of 23 months and is indicated for all pediatric patients at increased risk for pneumococcal disease.

A 13 year-old male with known cystic fibrosis presents to the emergency department, accompanied by his parents, with increased coughing, wheezing and low grade fever. Rales are audible on auscultation of the lungs. Treatment should target which of the following organisms?

Pseudomonas aeruginosa is the most predominant pathogen in patients with cystic fibrosis.

What is the most effective prophylaxis against respiratory syncytial virus (RSV) infection in the general pediatric population?

Proper hand washing and reduction in exposure is most effective in general population to prevent RSV.

A 16 day-old male presents in the office with a history of vomiting after feeding for the past 2 days. The vomiting has become progressively worse and the mother describes it as very forceful, sometimes hitting the floor 6 feet away. She says the neonate is always hungry. On physical examination, it is noted that he is 2 ounces below birth weight, and has a small palpable mass (about 1.5 cm) in the epigastrium. The most likely diagnosis is

Pyloric stenosis begins between 2 to 4 weeks of age with vomiting that becomes projectile after each feeding. An olive-size mass can often be felt in the epigastrium.

Which of the following is a milestone usually achieved by a 15-month old infant?

Question 19 Explanation: A 15-month-old infant should be able to walk alone

Which of the following is the most common pathogen implicated as the cause of bronchiolitis in children?

RSV is the most common cause of bronchiolitis in children.

A 10 year-old female experiences fever and polyarthralgia. On examination you note a new early diastolic murmur. Laboratory results are positive for antistreptolysin O. The patient has no known drug allergies. Which of the following is the recommended prophylaxis for this condition?

Recurrences of rheumatic fever are most common in patients who have had carditis during their initial episode and in children. The preferred method of prophylaxis is Benzathine penicillin G every four weeks.

A 12-month-old girl presents with recurrent urinary tract infections. The external genital examination is normal. Renal ultrasonography shows hydronephrosis. Voiding cystourethrography does not reveal any detrusor abnormality. Which of the following is the most likely diagnosis?

Retrograde passage of urine from the urinary bladder into the ureter is called vesicoureteral reflux (VUR), a condition which causes urinary tract infection in 30-40% of infants and toddlers. Causes are many, but most VUR is caused by a congenital abnormality of ureterovesicular junction, particularly a defect in the normal flap-valve mechanism in the intramural ureteral canal. An upper tract bacterial infection is a common result. Over time, recurrent kidney infections can lead to renal scarring, dysfunction and systemic hypertension in the adult population. Diagnosis is made by ultrasonography and voiding cystourethrography (VCUG). Symptoms may resolve spontaneously over months to years. Treatment may include prophylactic antibiotics and ureteral re-implantation. Bladder diverticulum (A) is a herniation of bladder mucosa through a defect in the bladder muscle (detrusor). It can occur with VUR. A VCUG would show results of an abnormal bladder wall. Cryptorchidism (B) refers to undescended testes, which are not found in girls. Epispadia (C) is when the urethral meatus opens on the dorsum of the glans penis, the penile shaft or between the clitoris and labia. This can be found during external genital inspection.

During an influenza epidemic, a 6 year-old child is seen with fever and a severe sore throat. A throat swab is taken for culture and the child is sent home. The next day, he is reported to have persistent vomiting and increased lethargy. On examination, he is delirious and disoriented. No rash is noted. His reflexes are hyperactive. The liver edge is 3 cm below the right costal margin in the midclavicular line. Which of the following is the most likely diagnosis?

Reye syndrome is typically post-influenza or URI. The patient develops lethargy, drowsiness, and vomiting. Babinski reflex is positive and hyperreflexia is noted. The liver is normal or enlarged.

A 7-year-old patient presents with an abrupt onset of fever, chills, malaise, muscular aching, headache, and nasal stuffiness. The patient is taking two baby aspirin every 6 hours for fever, muscle aches, and headache. Which of the following complications is the patient most at risk for?

Reye's syndrome is a rare and severe complication of influenza, particularly in young children. The pathogenesis is unknown, but the syndrome is associated with aspirin use in viral infections.

A 13 year-old child presents with a lesion on his right forearm that is occasionally pruritic. On examination, a 2 cm ring of erythema with a scaly border and central clearing is noted. A suspected diagnosis is confirmed by the presence of

Ring-shaped lesions with scaly borders and central clearing are most likely caused by fungal infection. Microscopic examination of scrapings reveals hyphae on KOH prep.

A 14 month-old male who attends day care presents with a two-day history of frequent watery stools. His mother states that he had a fever and vomiting the day before but these have resolved. His mother denies pain in the child. The child is mildly dehydrated but otherwise appears well. Stool samples are free of blood and white blood cells. The lab reports no ova or parasites noted in the stool samples. Which of the following is the most likely diagnosis?

Rotavirus is the most common cause of gastroenteritis in children and this is frequently passed in the daycare setting.

A mother brings in her 2 year-old child stating that the child has had a 3-day history of a nonproductive cough, thick copious rhinorrhea, conjunctivitis, and a fever to 103 degrees. Physical examination reveals a well-hydrated child, with numerous 1-2 mm white papules on both buccal mucosa, normal heart and breath sounds. This presentation is most consistent with early

Rubeola (measles) is characterized by cough, coryza, and conjunctivitis, along with a fever as a prodrome. Koplik spots appear prior to the onset of the typical erythematous, maculopapular rash and are pathognomonic for rubeola.

A 3 year-old male presents with a history of high fever, red watery eyes, rhinitis and cough for the past several days. The parents brought him in today due to development of a rash. They deny a history of childhood immunizations. On examination, the child has a barky cough, fever and clear rhinorrhea and sits listlessly on the exam table. The rash is red and maculopapular located on the face and trunk. Koplik spots are noted on the buccal mucosa. Which of the following is the most likely diagnosis?

Rubeola or measles is characterized by a prodrome of high fever, conjunctivitis, coryza (rhinitis) and a harsh cough. A maculopapular rash quickly spreads from the face to the trunk while the respiratory symptoms are at the worst.

A 14 year-old male who is overweight presents with complaints of left knee and anteromedial thigh pain for the past month. He states the pain gets better with rest and denies any known trauma. On examination of the gait, a slight limp is noted. X-ray films of the left knee are normal. The most likely diagnosis is

Slipped capital femoral epiphysis is most common in overweight adolescent males who present with complaints of pain that is referred to the thigh or medial side of the knee associated with a limp. X-ray films of the knee are normal since the condition involves the hip.

An 8 year-old child is brought in by his mother with a two day history of spreading, non-pruritic red rash. The rash was preceded by moderate fever, sore throat and rhinorrhea. Examination reveals a moderately ill appearing child with a fine, macular-papular rash on an erythematous base spread diffusely over the trunk with some accentuation in the skin folds. The face is flush with perioral pallor. There is palpable anterior cervical lymphadenopathy. Which of the following is the most appropriate diagnostic study to establish the diagnosis?

Scarlatina rash is due to infection with group A strep. A throat culture would be the most appropriate diagnostic study to establish the diagnosis in this patient.

An obese 12 year-old male presents with a 1 month history of right thigh pain worsened with weight bearing.Examination is normal, however, radiographs reveal a posterior and medial displacement of the femoral head. Which of the following is the most likely diagnosis?

Slipped capital femoral epiphysis is the posterior and usually medially displacement of the femoral head in overweight adolescents during puberty. The growth plate is susceptible to slippage due to the rapidly thickened cartilage physis secondary to growth hormone, the lack of sex hormone secretion for physis stability, mechanic stress secondary to obesity, and the anatomy and hip mechanics.

A child has been under treatment for attention-deficit hyperactivity disorder (ADHD). No response has occurred with behavioral adaptations. Which of the following categories of medication should this patient be given?

Stimulants, such as Ritalin, Dexedrine, and Cylert, are effective in 50 to 80% of children with ADHD.

Which of the following would be expected on physical examination of a newborn diagnosed with Tetrology of Fallot?

Tetralogy of Fallot is commonly associated with a palpable right ventricular lift

A 16-year-old male presents with decreased body hair and gynecomastia. On physical exam, the patient is noted to be tall and thin, with a wide arm span. Genital exam reveals a small phallus and small, soft testicles. Which of the following is the most likely the diagnosis?

The Klinefelter syndrome is an autosomal recessive disorder due to an extra X chromosome. Manifestations typically present at puberty with incomplete masculinization, decreased the body hair, gynecomastia, small phallus, and small, soft testicles. Noonan's syndrome presents with short stature, web neck, and pectus excavatum. Turner's syndrome presents with short stature, webbed neck , and phenotypical female. Trisomy 18 presents with mental retardation, prominent occiput and low-set ears

A 6-year-old boy was well until this morning when he awoke with papulovesicular eruptions over his arms, hands, legs, and feet. He refuses to eat or drink. What is the most likely etiology of his symptoms?

The above child has classic symptoms and signs of hand-foot-and-mouth disease, a common childhood infection caused by a Coxsackie or enterovirus. The clinical course begins abruptly with a maculopapular or vesiculopapular exanthem that includes the palms and soles, as well as the extremities. Involvement of the trunk may also be present. The oral enanthem typically involves the mucous membranes of the tongue and buccal mucosa and less commonly the palate or tonsils. The intraoral macules become vesicular and subsequently rupture to form ulcers with grey-yellow bases and circumferential erythema. Intraoral lesions are exquisitely painful, especially upon contact with salty or sour substances. As such, some children will refuse to drink and require admission for intravenous hydration. Preceding symptoms such as malaise or fever are typically absent. Herpes (B) may cause herpetic gingivostomatitis, the manifestation of primary infection with HSV-1. It is most common in children between six months and five years of age. Infection begins with prodromal symptoms such as fever and malaise. Subsequently, the child develops inflammation, bleeding, and friability of the gingiva, along with clusters of intraoral vesicles that progress to painful ulcerations. In contrast to hand-foot-and-mouth disease, the oral enanthem is often preceded by several days of non-specific symptoms. Children may spread herpes to other areas of their body through auto-innoculation with saliva, but the auto-innoculation would not be expected to affect both palms and both soles. Treponema pallidum (D) causes syphilis. While secondary syphilis may cause a variety of appearances of exanthems, including involvement of the palms and soles, mucous membrane involvement is less common. Rickettsia rickettsiae (C) causes Rocky Mountain Spotted Fever (RMSF), which is a tick-borne illness most common in the spring and summer. RMSF begins with several days of non-specific symptoms, including fever, headache, arthralgias, and myalgias. The rash of RMSF typically does not occur until the third or fourth day of illness. The classic rash is initially macular and later petechial, begins on the wrists and ankles, and spreads inward. Palm and sole involvement is characteristic but may not occur until later in the course of infection. Prompt treatment is required to prevent mortality or significant morbidity.

A 37-year-old-woman delivers a male infant with Down syndrome at 38 weeks. His Apgar scores at one and five minutes are both 8, with scores of 1 for acrocyanosis and irregular breathing. He passes meconium several hours later. Between 24 and 48 hours after birth, the infant has multiple episodes of vomiting despite lack of feedings and does not have additional bowel movements. Vomitus is bilious and nonbloody. On physical exam, the infant is crying, he appears jaundiced, and his abdomen is distended. An abdominal radiograph is performed, which is shown here. Which is the first step in the management of this patient?

The clinical presentation and radiograph are consistent with duodenal atresia. Nasogastric or orogastric tube placement is the first step in management prior to surgical intervention. Duodenal atresia is the congenital failure of the duodenal lumen to recanalize during fetal development. The condition is often seen in infants with Down syndrome and is associated with a number of congenital anomalies including biliary atresia, as well as cardiac and renal malformations. Duodenal atresia presents with vomiting 1-2 days after birth. Abdominal radiograph reveals the "double bubble" sign, caused by gastric and proximal duodenal dilation with narrowing at the pylorus.

A 3 year-old presents with profuse watery diarrhea for the past three days. The child vomited twice yesterday, but not today. On exam, the child is febrile, with pulse of 142, respiratory rate of 18, and blood pressure of 60/40 mmHg. On exam, the child is alert and responsive, with no focal findings. Which of the following is the most appropriate intervention?

The goal of therapy for a child with severe gastroenteritis and dehydration is to restore fluid loss. Oral rehydration with an appropriate electrolyte solution is the best option if the child is not actively vomiting and is alert enough to take oral fluids. IV fluids should be reserved for those who are unable to take fluids orally.

A 15-year-old male was seen last week with complaints of a sore throat, headache, and mild cough. A diagnosis of URI was made and supportive treatment was initiated. He returns today with complaints of worsening cough and increasing fatigue. At this time, chest x-ray reveals bilateral hilar infiltrates. A WBC count is normal and a cold hemagglutinin titer is elevated. The most likely diagnosis is

The insidious onset of symptoms, the interstitial infiltrate on chest x-ray, and elevated cold hemagglutinin titer make this diagnosis the most likely.

A 12 month-old child with tetralogy of Fallot is most likely to have which of the following clinical features?

The main characteristic of tetralogy of Fallot is cyanosis. Hypercyanotic spells or "tet spells" are paroxysmal episodes in which the cyanosis acutely worsens. Crying, feeding, or defecating can bring on these episodes.

Rates of alcohol use in the adolescent are reportedly higher in:

There is a three-to fourfold increase in risk for alcohol dependence in adopted children whose biological parents are alcohol dependents.

A 13-year-old boy on chemotherapy for acute lymphoblastic leukemia presents with progressive lower back pain for two weeks. Per the mother, he has had subjective fevers and a "bulge in his lower back" that is warm to touch. He is currently afebrile and has no focal neurologic deficits. An MRI is obtained, as seen in the image above. Which of the following regarding this patient's condition is true?

The patient has a posterior epidural abscess with adjacent osteomyelitis and myositis. The most common cause of an epidural abscess is hematogenous spread of infection, not direct extension from skin or soft tissue infection. Major risk factors include diabetes, intravenous drug abuse, chronic renal failure, alcoholism, and immunosuppression. The most common organism involved is Staphylococcus aureus. Other organisms include streptococci, anaerobes, gram-negative bacilli, and Pseudomonas aeruginosa. Patients present with localized back pain with tenderness to percussion. Fevers are common (reported in up to 75% of patients). MRI is the diagnostic modality of choice, but erythrocyte sedimentation rate is a sensitive marker and may be used in conjunction with plain radiographs to screen for infectious spinal disease. Vancomycin is an important component of the antibiotic regimen because it covers methicillin-resistant Staphylococcus aureus (MRSA) and is usually combined with a third-generation cephalosporin. Cefepime (A) is a fourth-generation cephalosporin with Pseudomonas coverage. Metronidazole or clindamycin can be added for anaerobic coverage. The most common cause of an epidural abscess is hematogenous spread of infection not direct extension from skin or soft tissue infection (B). While the classic triad consists of back pain, fever, and neurologic deficits (D), only a small proportion of patients actually have all three components at presentation.

A 3-year-old girl refuses to use her left arm after her mother tugged on her arm to help her cross a street. She does not appear distressed but is holding her arm in pronation with the elbow slightly flexed. Which of the following is the most appropriate next step?

The patient has a subluxation of the radial head, also known as a nursemaids elbow. This injury occurs when a longitudinal pull on the forearm while the arm is pronated allows fibers of the annular ligament to slip between the capitellum and the head of the radius. This result in inability to supinate the forearm. It most commonly occurs between the ages of one and four years when the annular ligament is still relatively weak. The classic history is a parent or guardian tugging on a childs arm while it is pronated. In infants, subluxation may occur when the child rolls over and inadvertently traps the forearm under the body with resultant longitudinal traction of the arm. Children may complain of elbow pain or may simply refuse to use the arm. The arm is held in pronation with slight flexion at the elbow. There may be tenderness over the radial head, but swelling, ecchymosis, and deformity are absent. If radial head subluxation is suspected based on history and examination, radiographs are not needed. Several methods are available to reduce the subluxation. The hyperpronation method involves the examiner supporting the elbow with one hand with moderate pressure on the radial head. The examiners other hand is then used to grasp the childs forearm and pronate it through a full range of motion. A clunk is felt as the radial head slips back into place. In the supination-flexion method, the examiner applies gentle pressure over the radial head and then supinates and flexes the forearm in one smooth motion. After successful reduction, the child may still be unwilling to use the arm for fear of causing pain. After a brief period of distraction, the child will typically use the arm normally. If reduction is successful, no further treatment is needed. Parents should be advised to avoid longitudinal traction on the forearm to prevent recurrence. Radiographs of the forearm and elbow (B) are not necessary if the history and exam is suggestive of radial head subluxation. Arthrocentesis and synovial fluid analysis (C) is the workup for joint infection. The clinical history of sudden onset of symptoms does not suggest a septic joint. Placing the patient in a splint (D) is the treatment for a fracture. Radial head subluxation is more likely in this scenario.

A 3 year-old boy is seen in the office with a 5-day history of fever, erythema, edema of the hands and feet, a generalized rash over the body, bilateral conjunctival injections, fissuring and erythema of the lips, and cervical adenopathy. Antistreptolysin A (ASO) titer and throat culture are negative. The most serious systemic complication associated with this disorder is

The patient most likely has Kawasaki syndrome. The major complication with this disorder is coronary artery aneurysms, which are reported in up to 20% of affected children. The etiology of this disorder is uncertain, although a bacterial toxin with super antigen properties may be involved.

A 15 year-old male presents with a 1 week history of hacking non-productive cough, low grade fever, malaise and myalgias. Examination is unremarkable except for a few scattered rhonchi and rales upon auscultation of the chest. The chest x-ray reveals interstitial infiltrates and a cold agglutinin titer was negative. Which of the following is the most likely diagnosis?

The patient's clinical symptoms as well as chest x-ray findings and negative cold agglutinin titer are most consistent with viral pneumonia.

By what age should the posterior fontanel be closed?

The posterior fontanel is typically closed at birth, but if open should close by 2 months of age

Which of the following is the most appropriate intervention in suspected child abuse?

The primary goal should be the safety and well-being of the child. Hospitalization may be the only way the clinician has to remove the child from the care of a possible abusive home if no other recourse is available due to a lack of social services and investigators.

According to the Advisory Committee on Immunization Practices, which of the following is the recommended age range for the first or initial MMR (measles, mumps and rubella) vaccination?

The recommended age range for the initial vaccination against MMR is 12-15 months.

Which of the following increases the risk of sudden infant death syndrome?

The risk of sudden infant death syndrome is increased with infants sleeping prone, exposure to cigarette smoking, low birth weight, and preterm birth

A G1P1 29-year-old mother presents to her 5-week-old son's pediatrician complaining that her son is an "angry" baby and that it is driving her crazy. She is worried that he is never going to become calmer. Over the last couple of weeks, he has been crying for several hours a day on "most days" of the week. She is breastfeeding him every 2-3 hours and changing his diaper after every feed (his urine and stool output is within normal limits). She estimates that he sleeps for 16 hours a day, and when he is awake, she usually carries him in a sling to promote attachment and to provide comfort. On exam, his height, weight, and head circumference are all around the 50th percentile, consistent with his trend since birth. He is alert and active, visually tracks your finger, and has normal muscle tone and reflexes. By what age should this condition improve?

This 5-week-old healthy male infant is crying for at least 3 hours a day, at least 3 days a week, without evidence of a condition that would provoke crying (e.g. - hunger, wet/soiled diaper, fatigue, emotional neglect, organic illness). Thus, he likely has infantile colic, which should resolve by around 4 months of age. Infantile colic usually begins between 2 and 6 weeks of age, with an estimated prevalence of 10-20% of infants. Although the cause is unknown, some theories include: immature gastrointestinal systems or central nervous systems; allergies; sensitive disposition; and maternal misinterpretation of normal crying. Risk factors include smoking, higher socioeconomic status, older parental age, and parental depression.

A 4-year-old presents with a history of having failed two courses of antibiotic therapy for acute otitis media. Initially she was on high-dose amoxicillin for 10 days followed by amoxicillin-clavulanate (Augmentin). Mother has been compliant with administering the medication. Which of the following is the next most appropriate intervention?

This child has unresponsive acute otitis media having failed two courses of appropriate antibiotic therapy. Tympanocentesis is indicated to identify the causative organism and appropriate antibioticselection.

A 3 week-old infant is evaluated for persistent projectile vomiting described as breast milk without bile or blood. The abdomen is distended before vomiting and a small, mid-epigastric mass is palpable after vomiting. Which of the following is the most appropriate diagnostic study for the evaluation of this patient?

This infant has pyloric stenosis and an upper GI series will reveal a narrowed distal stomach with double tract of barium

A 3 month-old male presents with a hoarse cough and thick purulent rhinorrhea for the past 2 days. The mother noted that yesterday he appeared to get worse and seemed to have increasing problems breathing and trouble feeding. Examination reveals a temperature of 100.2 degrees F and respiratory rate of 80/minute with nasal flaring and retractions. Lung examination reveals a prolonged expiratory phase with inspiratory rales. He is tachycardic. Pulse oximetry reveals oxygen saturation of 89%. Chest x-ray reveals hyperinflation with diffuse interstitial infiltrates. Which of the following is the most appropriate intervention?

This infant most likely has bronchiolitis. While most cases are mild and can be treated at home, hospitalization is recommended for infants with hypoxia on room air, moderate tachypnea with feeding difficulties and marked respiratory distress with retractions. Additionally hospitalization is recommended for infants less than 2-3 months of age, a history of apnea or an underlying chronic cardiopulmonary disease.

A mother brings in her 3 month-old infant and states that she has noticed a rash on her infant's scalp. Physical examination reveals the presence of erythematous and scaling crusty lesions involving the vertex of the scalp. Which of the following is the most appropriate initial intervention?

This infant most likely has scalp seborrheic dermatitis ("cradle cap"). Initial treatment consists of warm olive oil compresses to remove any crusts followed by use of baby shampoo or mild hydrocortisone cream.

A 3-day-old male infant born at 39 3/7 weeks to a 29-year-old G2P1001 mother exhibits yellow discoloration of the skin notable from the forehead to the level of the umbilicus. Which of the following would be concerning and prompt further evaluation of this infant?

This infant's clinical picture is consistent with a diagnosis of neonatal jaundice. A rising serum total bilirubin at a rate of greater than 5 mg/dL/24hr is indicative of pathologic jaundice and warrants further investigation. It is important to differentiate pathologic from physiologic neonatal jaundice, as there are numerous potential underlying causes to pathologic hyperbilirubinemia that, if present, must be identified so that further work-up and treatment can be initiated. Indications to pursue further evaluation of neonatal jaundice include: 1) conjugated bilirubin > 2 mg/dL or > 20% of total bilirubin, 2) jaundice in the first 24 to 36 hours, 3) bilirubin rate of rise > 5 mg/dL/24hrs, 4) total bilirubin > 12 mg/dL in a term infant, 5) jaundice persisting beyond 10-14 days of life, and 6) presence of any additional signs or symptoms indicative of underlying disease.

A 5 year-old male is brought to the office by his father who reports a 2 day history of low grade fever and coryza. The child awoke this morning with bright red cheeks. Physical examination reveals edematous, confluent plaques over the malar region of the face and reticular rash over the child's extensor surfaces. Which of the following is the most likely diagnosis?

This is a classic presentation of Fifth Disease, a childhood exantham associated with human parvovirus B19.

A 4-year-old patient presents with increasing redness and swelling involving her right eye for the past 2 days. The mother states that the child has become increasingly irritable, less active, and appears to have an increased temperature. The child had a recent "cold" and nasal congestion prior to onset of these symptoms. Examination reveals an ill-appearing 4-year-old child lying quietly on the exam table. Temperature is 102° F. Visual activity is 20/40 in the right eye and 20/30 in the left eye. The right eye reveals mild proptosis and severe erythema, increased warmth, and swelling involving the eye and surrounding tissues. Which of the following is the most appropriate diagnostic evaluation?

This is the typical presentation of orbital cellulitis. A CT scan of the orbit and sinuses is indicated to check for the presence of a subperiosteal abscess and underlying sinusitis, which is often the cause of orbital cellulitis.

A newborn male is evaluated 30 minutes after birth. He was born at 39 weeks gestation to a 27-year-old primigravid via cesarean section for cervical incompetence. The pregnancy was complicated by gestational diabetes, and the amniotic fluid was clear. Upon delivery, the patient had strong respiratory effort and a strong cry. His Apgar scores at 1 and 5 minutes were 7 and 8, respectively. The patient now is exhibiting increased work of breathing and is becoming progressively more tachypneic. His birth weight is 3,568 g (7 lb 14 oz). His temperature is 99.0°F (37.2°C), blood pressure is 60/44 mmHg, the pulse is 146/min, and respirations are 72/min. On physical exam, the patient is grunting with nasal flaring and subcostal retractions. Breath sounds are decreased at the bases bilaterally. The patient has central cyanosis. His chest radiograph can be seen here. Which of the following is the most likely etiology of this patient's presentation?

This newborn is presenting with increased work of breathing and progressive tachypnea in the first hour of life, which is consistent with a diagnosis of transient tachypnea of the newborn. Transient tachypnea of the newborn (TTN) presents with respiratory distress and marked tachypnea within two hours of delivery. TTN is caused by delayed resorption and clearance of alveolar fluid, which results in mild pulmonary edema. Chest radiography typically demonstrates bilateral perihilar streaking and hyperinflation of the lungs. TTN is benign and usually self-resolves by day two of life, and management is supportive.

A 2 year-old presents to the emergency department in acute respiratory distress. The parents relate a history of a recent upper respiratory illness that was followed by a sudden onset of barking cough during the night, but this morning they noted increased difficulty breathing. The child is noted to have stridor at rest, but has no evidence of cyanosis. Which of the following is the most appropriate initial intervention?

This patient most likely has laryngotracheobronchitis (viral croup). Treatment with nebulized racemic epinephrine and glucocorticosteroids is indicated for patients with stridor at rest.

A four-year-old boy is brought to your office by his concerned mother who states that he intermittently turns blue. She reports that he has been growing and developing normally, but recently he has been having crying fits where he turns blue and then squats down into a ball. Vital signs are stable. Physical examination reveals a pansystolic murmur at the left lower sternal border. Which of the following is true regarding the disease affecting this patient?

This patient has cyanotic spells, relieved by squatting, consistent with Tetrology of Fallot (TOF). One can expect a boot-shaped deformity on chest radiograph due to right ventricular hypertrophy. In TOF, patients have the classic clinical features of pulmonary stenosis (the most important determinant for prognosis), right ventricular (RV) hypertrophy, and an (overriding) aorta that lies directly over a ventricular septal defect (VSD). Because of pulmonary stenosis (elevated RV pressures) and the overriding aorta, patients experience early cyanosis from right to left intracardiac shunting. Patients may squat to improve symptoms because compressing the femoral arteries increases total peripheral resistance and decreases the right to left shunt, directing more blood from the right ventricle to the lungs and less across the VSD and into the aorta.

A 9-year-old boy presents with an inability to retract his foreskin. He has not been able to urinate for 2 days. The visible portion of the glans appears dusky. Which of the following therapies is most appropriate?

This patient presents with phimosis and evidence of vascular compromise, requiring performance of a dorsal slit procedure. Phimosis occurs when the foreskin of an uncircumcised penis becomes constricted, preventing retraction of the prepuce from the glans. It results in urinary outlet obstruction, glans ischemia, and infarction. Many cases are physiologic, resulting from normal development. Patients typically present with an unretractable foreskin and symptoms consistent with urinary obstruction, including decreased urinary stream. Management of phimosis can be difficult. Dilation of the prepuce can be performed using forceps but often does not result in relief of phimosis. When there are signs of glans vascular compromise (e.g., discoloration), a dorsal slit procedure should be performed. During this procedure, the foreskin is anesthetized and incised dorsally, allowing for retraction. A circumcision (A) may be necessary for definitive care, but treatment should not be delayed. Patients with no signs of obstruction or vascular compromise can be treated with topical corticosteroids (B) for 6 weeks but require close follow-up. Intracavernosal phenylephrine (D) is a treatment modality for priapism but plays no role in the treatment of phimosis.

A 17-year-old male presents to your office for evaluation. In addition to struggling academically, you notice he is tall for his age, has small testes, and gynecomastia. Upon further workup, what genetic finding will this patient most likely have?

This patient's clinical findings are most consistent with Klinefelter syndrome. This is a genetic disorder in which patients have an extra X-chromosome and a karyotype of 47, XXY. Klinefelter syndrome is caused by the presence of an additional X-chromosome. Affected individuals are typically asymptomatic throughout childhood and present in adolescence or early adulthood. The typical clinical presentation includes tall stature, small testes, and gynecomastia. Further workup reveals a 47, XXY karyotype and typically, azoospermia. Almost all men with Klinefelter syndrome are infertile. Late onset of puberty should prompt further evaluation, however often at diagnosis, the window for any therapeutic interventions has passed.

A 2-year-old presents with fever and drooling. The patient appears toxic and is sitting up with chin forward. Thumbprint sign is noted on lateral neck x-ray film. Which of the following is the treatment of choice for this patient?

acute epiglottitis is a medical emergency and immediate treatment with an artificial airway in a controlled environment is indicated. Steroids and racemic epinephrine are ineffective and not indicated. The antibiotic of choice is ceftriaxone, or cefotaxime. Ampicillin is not considered first line due to increased resistance of H. influ to ampicillin

A 3-year-old male presents to the emergency department with difficulty breathing. On physical examination, the patient is febrile and tachypneic. The patient's lateral neck radiograph is shown here. Which of the following signs on physical examination is most specific for this patient's disease?

This patient's presentation is consistent with epiglottitis. Among the choices, drooling is the physical finding most specific for epiglottitis. Epiglottitis is a serious and rapidly progressive infection of the epiglottis and contiguous structures that affects children from age 3-7. It presents with fever, tachypnea, stridor, drooling, the "tripod position," and the "thumbprint sign" on lateral neck radiograph. It is classically caused by H. influenzae type B (Hib) but S. aureus and Streptococcus are now common causative agents due to the Hib vaccine. Epiglottitis is is a true airway emergency and requires immediate intubation in the OR. Patients remain intubated while receiving IV antibiotics for up to 72 hours. It must be differentiated from croup, which presents similarly and is not immediately life-threatening; croup shows the "steeple sign" on CXR and presents with cough and a lack of drooling.

A 16 year-old athlete with no past medical history collapses after running 50 yards down the field. He is unresponsive, pulseless and cyanotic. Which of the following is the most likely cause of this student's collapse?

This presentation is consistent with hypertrophic cardiomyopathy which may initially be difficult to diagnose.Infants but not older children frequently present with signs of CHF. Older children may be asymptomatic, with sudden death as the initial presentation

A 7-year-old child with a history of type 1 diabetes mellitus for 3 years presents for routine follow-up. The mother states that the child has been having nightmares and night sweats. Additionally, his average morning glucose readings have risen from an average of 100 mg/dL to 145 mg/dL over the past week. This child is most likely experiencing

This refers to nocturnal hypoglycemia, which stimulates counter-regulatory hormone release resulting in rebound hyperglycemia.

A six-year-old child presents with circular patches of baldness and hair that fluoresces yellow-green under a Woods lamp. Which of the following would be the best treatment?

Tinea capitis is best treated with oral griseofulvin until the lesions clear, usually 4-8 weeks.

A four-year-old boy and his mother present to general pediatrics clinic for follow-up. She is in distress because she has tried everything to get him toilet trained. Her prior child was toilet trained at the age of 2. Her 4-year-old is developmentally appropriate. He can sit upright and can communicate that he needs to use the bathroom. The mother has tried to place him on the toilet at regular intervals and has given him rewards for using the toilet successfully. However, he is not motivated to stay dry and asks to keep using diapers. The mother is desperate and asks what she can do to get her son toilet trained immediately. Which of the following is the best advice to give the mother of this patient?

Toilet training occurs between 2-4 years of age. If the child is not motivated to stay dry, the best advice is to wait for a few months then reassess. This will give the child and mother a break and allow for both to approach the situation fresh. Regardless, it is normal for children to not use the toilet up until age 5. Toilet training occurs between ages 2 and 4 with 5 being the cutoff age when an inability to use the toilet may be considered a problem. To be ready for toilet training, the child must be aware of bladder filling, able to consciously tighten their external sphincter, has normal bladder growth, and be motivated to stay dry. Further, the child must have met motor milestones and be able to walk to the toilet, sit upright, as well as have met language milestones to communicate the need to use the bathroom. The process involves placing the child on a toilet at regular intervals and using positive reinforcement as a reward. If the child is very reluctant, the best strategy is to stop trying to toilet train for a few months and then reassess

A 17 year-old female is seen who has a history of eating large amounts of food at night 3 to 4 times weekly. These episodes are always followed by extreme guilt and either induced vomiting or hours of strenuous exercise. She excels at work and school and maintains many active relationships. Her physical examination reveals a normal BMI and an otherwise normal exam. What is the best initial intervention for this patient?

Uncomplicated bulimia generally does not require hospitalization as the physical manifestations encountered in bulimia are generally mild if present at all. Some patients with bulimia have concomitant substance abuse issues but her successful relationships and work activities lessen the probability. Antidepressants, not anxiolytics, have been found helpful in lessening the binge/purge cycles and improving overall well-being even outside of comorbid mood disorders.

A 3 year-old girl is diagnosed with atopic dermatitis. Which of the following disorders is this child at risk for in the future?

Up to 50% of patients with atopic dermatitis develop asthma and/or allergic rhinitis in the future.

A mother of a newborn infant presents to the office concerned about reducing the risk of sudden infant death syndrome (SIDS). The infant was delivered at 39 weeks gestation weighing 7 pounds 9 ounces. There is no family history of SIDS and this is her first child. Which of the following is appropriate advice to reduce the risk of SIDS?

Use of a pacifier during sleeping is a current recommendation to decrease the risk of SIDS.

A 7-day-old girl is brought to the general pediatrics clinic by her mother. She was the product of a full-term uncomplicated pregnancy and was delivered via cesarean for breech presentation. The mother received regular prenatal care throughout the pregnancy. This morning, after changing the child's diaper, the mother noticed that the newborn had blood at the vaginal introitus. The mother has no other complaints, and the infant is eating and voiding appropriately. Vital signs are stable. Physical exam reveals moderate mammary enlargement and confirms the vaginal spotting. The remainder of the exam is unremarkable. What is the next step in management?

Vaginal spotting or bleeding is normal in female infants less than 3 months of age and will stop as soon as maternal estrogens are cleared from the baby's blood. The parents of this child only require reassurance. Normal findings in the newborn include non-purulent vaginal discharge, vaginal spotting, and mammary enlargement. All of these symptoms are not a cause for concern and generally regress within a few days. They result from the mother's hormones being transferred to the fetus in utero as well as via breast milk. No workup is required.

A 12-year-old boy presents with fever and severe sore throat. Which of the following physical exam findings helps differentiate a peritonsillar abscess from epiglottitis?

While epiglottitis and peritonsillar abscess share several similar presenting signs and symptoms, the key differentiating finding is a uvular deviation which is only found with a peritonsillar abscess. Peritonsillar abscesses occur primarily in young adults. This condition most often follows Group A streptococcal pharyngitis and exudative tonsillitis. The abscess is polymicrobial and generally presents with fever, malaise, sore throat, dysphagia, and otalgia. On physical exam, the patient may have trismus and a muffled voice (classically called a "hot potato voice").

Spina bifida occulta is usually detected by which initial diagnostic evaluation?

X-ray or MRI is the definitive test to diagnose spina bifida occulta, showing the vertebral bony defect.

A 4-month-old female presents with her parents to your office. The patient's parents are concerned because she used to push up to her elbows during tummy time, but she now struggles to lift her head. She has been urinating and stooling well, but her parents report decreased interest in food. All prenatal screening for the patient was unremarkable. There is no family history of genetic abnormalities in the patient's mother. The patient's father was adopted and knows little about his biological family. The patient has two older siblings who are both developmentally normal. On physical exam, the patient appears well-developed and well-nourished. She is in the 40th and 48th percentiles for height and weight, respectively. She has no dysmorphic features. Her abdomen is soft and non-tender. Hepatosplenomegaly is present. Ophthalmologic exam reveals the finding seen here. Of the following, which is most likely to be found in this patient?

hyporeflexia This 4-month-old patient presents with loss of motor milestones, a "cherry-red" spot on the macula, and hepatosplenomegaly, which suggests a diagnosis of Niemann-Pick disease. Niemann-Pick disease also presents with hyporeflexia. Niemann-Pick disease (NPD) is a sphingolipidosis caused by a deficiency in sphingomyelinase. NPD shares many features with Tay-Sachs disease, as both present in infants aged 2-6 months with progressive neurodegeneration, hypotonia, feeding difficulties, and a "cherry-red" macula. The two can be distinguished by the fact that only NPD presents with hepatosplenomegaly, and NPD presents with hyporeflexia or areflexia, whereas Tay-Sachs disease presents with hyperreflexia. In addition, NPD is characterized by an accumulation of sphingomyelin and other lipids in macrophages, which gives affected cells a "foamy" appearance.

An examination of the hips of a newborn, a "clunk" is noted with abduction of the right hip to almost 90 degrees while lifting the greater trochanter. Which of the following is the most likely diagnosis?

the Ortolani test is positive in the newborn patient with developmental dysplasia of the hip. The test is performed by lifting the greater trochanter while abducting the hip to 90 degrees.