Pediatric Quiz Flashcards

1
Q

You are giving a lecture on poisoning in children to a group of medical students. One student asks you the best way to prevent poisoning. You explain the preventive measures that should be taken and that anticipatory guidance counseling should be provided during health supervision visits.

Of the following, the health supervision visit at which it is MOST appropriate to begin discussing poisoning prevention strategies with parents is:

Choices: 2mo, 6mo, 9mo, 12mo, or 2 wks?

A

B. 6 months

Because many children will begin to explore their environment by either crawling or creeping before 9 months, childproofing the home by safely storing poisonous substances and medications should be discussed at the 6-month visit. Waiting until the 9- or 12-month visit may be too late

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2
Q

A 10-year-old boy presents to your office with the complaint of cola-colored urine. He had been well until 2 days ago, when he developed symptoms of rhinorrhea and mild cough. His sister had cold symptoms approximately 1 week ago. His review of systems is notable for a mild sore throat without fever. Family history is notable for an uncle who is on hemodialysis. On physical examination, the boy has a temperature of 37.3°C, a heart rate of 84 beats/min, a respiratory rate of 20 breaths/min, blood pressure of 106/62 mm Hg, and normal growth parameters. Laboratory evaluation reveals the following:
• Electrolytes, normal
• Blood urea nitrogen, 24 mg/dL (8.6 mmol/L)
• Creatinine, 0.9 mg/dL (80 mmol/L)
• Albumin, 3.4 gm/dL (34 g/L)
• Complement component 3 (C3), 128 mg/dL; normal, 80 to 200 mg/dL
• Complement component 4 (C4), 27 mg/dL; normal, 16 to 40 mg/dL
• Antinuclear antibody, negative
Urinalysis:
• Specific gravity, 1.015
• pH, 6.5
• 3+ blood
• 2+ protein
• Leukocyte esterase, negative
• Nitrite, negative

Of the following, the study MOST likely to result in a diagnosis is:
A. antistreptolysin O titer
B. hearing screen
C. noncontrast computed tomography scan of the abdomen
D. immunoglobulin A level
E. creatine phosphokinase level

A

B. Hearing Screen

The differential diagnosis in this clinical setting includes IgA GN, membranoproliferative glomerulonephritis (MPGN), and Alport syndrome. Although IgA GN and MPGN are more common, a genetic syndrome is suggested in light of the uncle on hemodialysis. The most appropriate course of action is to screen this patient for Alport syndrome with a hearing screen to look for bilateral high frequency hearing loss. pproximately 80% of cases of Alport syndrome are X-linked inherited. Types of familial nephritis include Alport syndrome and thin glomerular basement membrane disease.

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3
Q

A full-term male newborn is admitted to the regular nursery after an uneventful delivery. Apgar scores are 8 at 1 minute and 9 at 5 minutes. He feeds well initially, but 32 hours after birth you are called by the nurse because he is feeding poorly and seems “lethargic.” You arrange for transfer of the newborn to the intensive care nursery, where the resident on duty obtains a blood culture and provides appropriate antibiotic coverage.Of the following, if sepsis is ruled out, the test MOST likely to be diagnostic in this newborn is
A. electroencephalogram
B. head ultrasonography
C. liver function tests
D. serum ammonia and urine organic acids
E. a complete blood cell count with differential

A

D. serum ammonia and urine organic acids

The child in this vignette is a healthy, full-term newborn who has developed mental status changes within 48 hours after birth. Although sepsis is the most likely diagnosis in this scenario, the newborn’s presentation is also typical of an inborn error of metabolism, such as a urea cycle defect or organic acidemia. Therefore, serum ammonia and urine organic acid testing should be considered and done in parallel with blood cultures and antibiotic administration.

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4
Q

A 10-year-old girl is brought to your office after falling off her bicycle 4 hours ago. She did not lose consciousness after the fall but appeared confused and had 2 episodes of vomiting. These symptoms resolved in 30 minutes and she is now acting normally. She has had no further episodes of emesis and denies headache. On physical examination, she is awake, alert, and her Glasgow Coma Scale score is 15. Her vital signs are within reference range. She has abrasions on her forearms and her forehead. Her neurologic examination results are normal.Of the following, the MOST appropriate next step is
A. computed tomography of the brain without intravenous contrast
B. computed tomography of the brain with intravenous contrast
C. reassurance
D. skull radiographs
E. magnetic resonance imaging of the brain

A

C. reassurance

Observation is recommended for patients with isolated, nonprogressive, nonpersistent signs because they have significantly less than a 1% likelihood of clinically important intracranial injury (CIICI). Observing patients in this subgroup led to a lower CT scanning rate without an increase in morbidity.

Child in the vignette has an isolated, nonpersistent risk factor (history of vomiting). Four hours of observation have elapsed since the injury occurred, during which her symptom resolved. Her risk of CIICI is substantially less than 1%. Parents can be reassured, and the child can be safely discharged.

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5
Q

A 10-year-old girl presents with a 6-week history of double vision and droopy eyelids. Symptoms fluctuate and tend to worsen with fatigue. She has no difficulty walking up stairs or lifting her arms, and she has no complaints of pain. Physical examination reveals a healthy-appearing girl with normal vital signs. General physical examination findings are normal, as are mentation and language. She tips her head back to look under her drooping eyelids and has limited abduction and adduction of both eyes. She has weakness of eye closure. Her limb strength, tone, bulk, reflexes, and gait are normal.

Of the following, the MOST likely diagnosis is
	A. dermatomyositis
	B. Guillain-Barré syndrome
	C. myasthenia gravis
	D. pseudotumor cerebri
	E. tick paralysis
A

C. myasthenia gravis

vignette has an acquired, fluctuating, bilateral muscle weakness that affects face muscles and eye movements but not thinking or language. The prominent ptosis and fluctuation of symptoms that worsen with fatigue are consistent with myasthenia gravis.

Neuromuscular junction diseases affect the acetylcholine transmission signal between the nerve terminal and the muscle. The most common is myasthenia gravis, which is caused by autoantibodies directed against the acetylcholine receptor in the muscle. The presence of these antibodies variably blocks the nerve signal to the muscle, causing weakness.

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6
Q

A 10-year-old boy presents to the emergency department for fever. His mother states that he had a splenectomy 2 years ago for severe hereditary spherocytosis. He has an oral temperature of 39.5°C, pulse rate of 110 beats/min, respiratory rate of 20 breaths/min, and blood pressure of 125/80 mm Hg. On examination, he is in no distress and his lungs are clear to auscultation bilaterally. The remainder of his examination is unremarkable. Results of a chest radiograph are normal with no consolidation. A blood culture is drawn and sent to the laboratory. The following are the results of the boy’s complete blood cell count:

  • White blood cell count, 15,000/µL (15 × 109/L), with 45% polymorphonuclear leukocytes, 10% band neutrophils, 36% lymphocytes, 6% monocytes, and 3% eosinophils
  • Hemoglobin, 12.3 g/dL (123 g/L)
  • Platelet count, 625 × 103/µL (625 × 109/L)
Of the following, the MOST appropriate next step would be to administer
	A. oral penicillin 
	B. aspirin   
	C. conjugate pneumococcal vaccine 
	D. intravenous ceftriaxone
	E. oral azithromycin
A

D. intravenous ceftriaxone

Patients with asplenia from a congenital, functional, or postsurgical state are at increased risk for bacteremia that often leads to sepsis. Because of the high incidence of sepsis among asplenic patients, fever in these patients should be treated immediately with empiric, broad-spectrum parenteral antibiotics such as ceftriaxone. Oral antibiotics are not sufficient treatment for presumed bacteremia. Vaccination against encapsulated organisms would not be indicated to treat the acute infection.

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7
Q

You are evaluating a newborn in the nursery who was delivered at term following an uneventful pregnancy to a 25-year-old gravida 1, para 0 woman by normal spontaneous vaginal delivery. The newborn’s birth weight was 3.2 kg and she is breastfeeding well. On examination, you note redundant skin on the nape of her neck and puffiness of the dorsum of her hands and feet (Item Q62).Of the following, the test MOST likely to provide a diagnosis for this newborn is

	A. renal ultrasonography 
	B. liver function tests  
	C. a cytogenetic analysis 
	D. serum electrolytes 
	E. .an echocardiogram
A

C. A cytogenic analysis

The newborn described in this vignette has findings suggestive of Turner syndrome (TS), so the diagnostic test of choice would be a cytogenetic analysis. About half of affected girls having 45,XO karyotype. The remainder of girls with TS have sex chromosome mosaicism

TS –> redundant skin on the nape of the neck as well as hand and foot edema are very suggestive of this condition.

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8
Q

You are seeing a 16-year-old girl because she has not had a menstrual period in 6 months. She had menarche at 12 years of age and her periods had become progressively more irregular before completely stopping 6 months ago. Her past medical history is unremarkable and she is currently asymptomatic. She is on the cross country running team and is planning on running a marathon in the next year. She denies being sexually active or having any body image issues. Her vital signs are normal, her body mass index is 19.5, and her sexual maturity rating is stage 5. Results of the remainder of her physical examination are unremarkable. The pregnancy test result is negative. Additional tests, including a complete blood cell count, electrolytes, thyroxine, thyroid-stimulating hormone, luteinizing hormone, and follicle-stimulating hormone concentrations, yield normal results.Of the following, the MOST important next step in the management of this girl would be to have

A. increase her overall nutritional intake 
B. increase her calcium and vitamin D intake 
C. switch to a nonendurance sport 
D. begin combined oral contraception 
E. stop exercising till her me.nses returns
A

A. Increase her overall nutritional intake

A negative energy balance in female athletes may lead to the female athlete triad, which is composed of amenorrhea, osteoporosis, and disordered eating. Restoring a positive energy balance by increasing overall food intake may be all that is needed to restore menses and improve bone density.

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9
Q

You are evaluating a 16-year-old girl who has a 5-day history of progressive cough, shortness of breath, and fever. Her vital signs include a temperature of 39.0°C, a heart rate of 100 beats/min, and a respiratory rate of 35 breaths/min. On physical examination, she has mild respiratory distress, prolonged expiration, and decreased breath sounds over the left chest. You obtain a chest radiograph and decide to admit her to the hospital for further treatment.

[CXR: Left side white out]

Of the following, the MOST appropriate empiric antibiotic regimen to order is

	A. ampicillin and gentamicin 
	B. doxycycline 
	C. cefuroxime and nafcillin
	D. azithromycin 
	E. ceftriaxone
A

E. Ceftriaxone

Empiric therapy with a third-generation parenteral cephalosporin (ceftriaxone or cefotaxime) is appropriate for hospitalized infants and children who have complicated pneumonias (such as the girl in the vignette with probable empyema), for infants who are not fully immunized, or in areas where high-level penicillin resistance of pneumococcal strains is present.

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10
Q

You are caring for a 10-year-old girl who has cystic fibrosis. In addition to her regular pulmonary care regimen, she is consuming a high-protein diet that provides 125% of her calculated daily requirements for energy and protein. Her diet includes 3 meals plus 2 snacks per day, along with pancreatic enzyme supplements that provide 20,000 lipase units per meal and 12,000 lipase units per snack. She also takes daily supplements of fat-soluble vitamins. Despite this regimen, she has lost 2 kg over the past 6 months, and her stools have become increasingly bulky and frequent. At the time of her office visit today, her height is at the 50th percentile, and her weight is at the 10th percentile for her age. The remainder of the physical examination demonstrates mild expiratory wheezing at the lung bases but is otherwise unchanged from previous examinations. Of the following, you are MOST likely to recommend

A. a hydrolyzed protein–based nutritional supplement
B. reducing fat content of the diet 
C. supplementing with medium-chain triglyceride oil 
D. increasing mealtime doses of pancreatic enzymes 
E. nocturnal enteral feedings
A

D. increasing mealtime doses of pancreatic enzymes

Difficulties in maintaining optimal nutrient intake are the consequence of both malabsorption secondary to pancreatic insufficiency and increased energy requirements resulting from increased work of breathing. The most likely cause of this weight loss is inadequate lipase supplementation at mealtime. Her current mealtime supplement of 20,000 lipase units falls well below the amount recommended for her age which is 24,000 to 40,000 lipase units per meal.

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11
Q

A 12-year-old girl is in your office, complaining of an increase in the quantity of her vaginal discharge. She is a swimmer and uses tampons frequently. She experienced menarche about a year ago and notes that the discharge increases just before the onset of her menses. She denies any sexual activity and has no urinary or gastrointestinal symptoms. A pregnancy test result is negative. You examine the discharge and note that the pH is 4.5, the whiff test result is negative, and there are no clue cells or white blood cells on microscopy. Of the following, the MOST appropriate next step in the management of this girl is to

A. culture the vaginal discharge for Gardnerella vaginalis 
B. question her further about sexual abuse
C. advise her to discontinue the use of tampons
D. encourage her to douche with her periods 
E. reassure her that the discharge is normal
A

E. reassure her that the discharge is normal

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12
Q

A 14-year-old boy comes to his pediatrician’s office on Long Island, NY, in early September for evaluation of a 3-week history of fatigue and diffuse achiness. There are no noted joint swellings or rashes, and he has had no fever throughout this illness. He has been playing in soccer tournaments across Long Island over the summer. He was seen in an urgent care center the previous weekend for these complaints, and his physical examination was unremarkable. His complete blood cell count and erythrocyte sedimentation rate were also normal, but his Lyme enzyme immunosorbent assay (EIA) result was 2.20 (negative,

A

C. Western Blot Analysis for Lyme Antibodies

Serologic testing for Lyme disease is performed in a 2-tiered approach. The initial screening test is most often done by enzyme immunosorbent assay (EIA). Although the EIA is easily performed and has good sensitivity, it does not have sufficient specificity. Therefore, a positive or equivocal EIA should be confirmed by the more specific western blot that assesses responses to individual proteins of B burgdorferi. The immunofluorescent antibody (IFA) test is an alternative screening test that is analogous to the EIA. Polymerase chain reaction (PCR) can be used to detect B burgdorferi DNA in cerebrospinal fluid or synovial fluid, but it is not a routine assay for confirming a Lyme EIA result.

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13
Q

A 13-year-old girl is struggling in her eighth grade classes. She is a social girl who is an active participant on her soccer team. Her mother brings you the results of her psychoeducational testing. The Vanderbilt rating form revealed no significant issues with attention or hyperactivity. Cognitive testing revealed a verbal IQ of 95 and a nonverbal IQ of 100 (the normal range for the population is 85-115). There was no significant difference between the score of 95 on the verbal section and the score of 100 on the nonverbal section. Educational testing demonstrated that her reading score was at a sixth grade level and her math score at the eighth grade level.Of the following, the MOST appropriate diagnosis for this girl is

	A. attention-deficit/hyperactivity disorder 
	B. autism spectrum disorder 
	C. learning disabilty 
	D. intellectual disability 
	E. motor coordination disorder
A

C. Learning Disability

The girl described in the vignette has evidence of a learning disability with difficulty in reading. In order for a learning disability to be diagnosed, there must be a significant discrepancy between the child’s achievement scores in reading, writing, or math and the child’s cognitive ability (IQ). The discrepancy must impact the child’s educational success.

The girl described in the vignette does not have significant impairment in social reciprocity or restricted and repetitive behaviors; therefore, she does not have an autism spectrum disorder. Her Vanderbilt screening tool does not raise concern for attention-deficit/hyperactivity disorder. Her cognitive profile is average and not indicative of an intellectual disability. The girl is described as an active participant in her sports team, which indicates there is no evidence of a primary motor coordination disorder.

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14
Q

A 13-year-old boy has complained of dull medial thigh pain for a month, but his parents have only noticed a mild limp for the last 3 days. He has had no trauma, fever, rash, weight loss, or fatigue. His body mass index is at the 95th percentile for his age and he is at sexual maturity rating 3. There is mild tenderness over the thigh. The knee is not red or swollen, and there is no ligamentous laxity. Physical examination of his hip shows mildly limited abduction with decreased internal rotation.

Of the following, the MOST appropriate diagnostic test for this boy is

A. anterior-posterior and frog-leg radiographs of the hip
B. bone scan of the hip
C. magnetic resonance imaging of the knee and thigh
D. serum creatine kinase, complete blood cell count, and C-reactive protein levels
E. ultrasonography of the hip
A

A. Antirior-Posterior and Frog-Leg radiographs of the Hip

Slipped capital femoral epiphysis (SCFE) is the most common hip condition of early adolescence. Those affected are usually above the 90th percentile for weight for age.

The most important diagnostic test for SCFE is a lateral frog-leg radiograph of the hips that shows widening of the physis, shortening of the epiphysis, or posterior-inferior displacement of the femoral head. Once the diagnosis is made, the patient should be placed on bed rest immediately. The treatment of choice is in situ fixation with a single screw. Attempts at reduction have led to an increased risk of avascular necrosis and are contraindicated.

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15
Q

A 13-year-old boy presents with a 1-day history of right-sided scrotal pain. There is no history of sexual activity or trauma. He had some facial swelling about a week ago that has since subsided. He also complains of a mild headache and feels fatigued. He has no history of dysuria, frequency, or penile discharge. On physical examination, he is febrile with a temperature of 38.4°C. There is right hemiscrotal swelling with a tender swollen testicle on palpation. The left testicle and cord are normal. Results of urine microscopy are within reference range. Further laboratory studies reveal a white blood cell count of 12,100/μL (12.1 × 109/L) and a C-reactive protein of 25 mg/L (238.1 nmol/L). Ultrasonography with a Doppler flow shows an edematous right testicle with increased flow.

Of the following, the MOST likely cause of his scrotal pain is

	A. epididymitis due to chlamydia infection
	B. germ cell testicular tumor
	C. inguinal hernia
	D. torsion of the testicle
	E. viral infection of the testicle
A

E. viral infection of the testicle

The boy described in the vignette has scrotal pain that is most likely caused by orchitis. Infection limited to the testicle is much less common and usually is caused by a virus such as mumps. Evidence of testicular involvement begins 4 to 6 days after the parotid swelling. One or both testicles become swollen and tender; fever, nausea, and vomiting may be present. The cremasteric reflex is usually present and color Doppler ultrasonography shows increased perfusion. Recovery is usually spontaneous and complete within 1 to 9 days.

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16
Q

A 12-year-old boy presents to your office for follow-up after his third wrist fracture in 3 years. As part of his evaluation in the emergency department, a complete metabolic panel was obtained and revealed a low calcium concentration (7.5 mg/dL [1.88 mmol/L]), low phosphorus concentration (2.8 mg/dL [0.90 mmol/L]), normal magnesium concentration (1.9 mg/dL [19 g/L]), and normal albumin (4 g/dL [40 g/L]). With the exception of his recurrent fractures, the boy has had no other medical problems and has not been taking any long-term medications. His height and weight are both at the 75th percentile. His physical examination results are unremarkable except for his casted left wrist.Of the following, the MOST appropriate next step in this boy’s evaluation and management is to measure

	A. serum parathyroid hormone 
	B. serum 1,25-dihydroxyvitamin D 
	C. serum 25-hydroxyvitamin D 
	D. urine N-telopeptide 
	E. ionized calcium
A

C. serum 25-hydroxyvitamin D

The boy described in the vignette has hypocalcemia, hypophosphatemia, and a history of recurrent fractures. The most common cause of hypocalcemia and hypophosphatemia is prolonged vitamin D deficiency. This deficiency can best be determined by measuring 25-hydroxyvitamin D (25[OH]D) levels because it is the primary storage form of vitamin D.

A serum PTH can be helpful in the evaluation of a child who has hypocalcemia and hyperphosphatemia. However, a PTH level is not helpful in the evaluation of patients who have hypocalcemia and hypophosphatemia because the PTH concentration is invariably elevated when both serum calcium and phosphorus concentrations are low.

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17
Q

While performing a newborn examination 18 hours after birth, the mother relates to you that her son has had small green emesis after each of the last 3 feedings. The infant was born at 39 weeks’ gestation by spontaneous vaginal delivery that was complicated by moderate meconium-stained amniotic fluid. He emerged vigorous and did not require tracheal suctioning. Since birth, he has been fed a cow’s milk protein formula every 3 hours and has had 2 wet diapers and 1 stool smear. Physical examination reveals an alert infant with a soft, nondistended abdomen and an externally patent rectum. The infant has another emesis of bilious material immediately after your examination You obtain an abdominal radiograph that is interpreted as normal.

[IMAGE OF GREEN EMESIS]

Of the following, the MOST appropriate next step in the management of this infant is to:

A. obtain abdominal ultrasonography
B. catheterize the urethra for urine culture
C. observe the infant for additional symptoms
D. perform an upper gastrointestinal series
E. prescribe a complete milk protein hydrolysate formula

A

D. perform an upper gastrointestinal series

When bilious emesis is the primary presenting symptom in the newborn period, an upper gastrointestinal series (UGI) should be performed to evaluate for malrotation. Malrotation with midgut volvulus is a surgical emergency and must be rapidly ruled out in the infant with bilious emesis. A radiograph of the abdomen may reveal evidence of obstruction in malrotation with volvulus, but it also may be normal. The preferred diagnostic study is a UGI, which reveals the position of the ligament of Treitz in relationship to the spine. Although abdominal ultrasonography may identify abnormalities associated with malrotation, it possesses less diagnostic sensitivity than UGI.

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18
Q

You are called to the emergency department to evaluate a 5-year-old girl who was riding a bike without a helmet and fell, striking her head approximately 1 hour ago. The accident was witnessed by her mother who states that the child cried on impact and was able to move all extremities. The girl was driven by the mother directly to the emergency department and has had increasing somnolence over the past 30 minutes.The patient has a temperature of 37.6°C, a heart rate of 130 beats/min, and a respiratory rate of 30 breaths/min. She is drowsy but awakens when spoken to; she complains of a headache. She has a large area of swelling over her right frontal area but no noticeable bone depression. Her pupils are equal and reactive and the results from the remainder of the physical examination are normal.Of the following, the MOST appropriate next step in the management of this child is to

A. discharge the child with head injury precautions
B. obtain skull radiographs
C. obtain computed tomography of the head
D. obtain magnetic resonance imaging of the brain
E. admit the child to the hospital for observation

A

C. obtain computed tomography of the head

Although she will need to be admitted to the hospital, the initial step in her management should be to obtain a computed tomography of her head because of her clinical appearance and the mechanism of injury. Magnetic resonance imaging offers the advantage of providing no radiation, but the lower cost and speed of a computed tomography scan make it the most appropriate initial step. The girl described in the vignette may have sustained a skull fracture, but plain radiographs of the skull will not define potential cerebral abnormalities. Discharge to home is contraindicated.

Epidural hematomas are often said to present with a period of normal consciousness that occurs after a head injury and is followed by a rapid deterioration in level of consciousness.

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19
Q

You are assessing an infant who was born by spontaneous vaginal delivery at 37 weeks’ gestation to a 19-year-old primigravida who received no prenatal care. The growth parameters include a weight of 2,500 grams (10th percentile), head circumference of 30 cm (3rd percentile), and length of 46 cm (10th percentile). The physical examination is notable for faint facial jaundice, diffuse petechiae on the trunk and extremities, a 1/6 systolic murmur at the left sternal border, a liver edge palpable 2 centimeters below the right costal margin, and a spleen tip palpable 4 cm below the left costal margin.

[IMAGE: Baby w/ distended belly + two spots w/ arrows]

Of the following, the study MOST likely to lead to the correct diagnosis is:

A. serology for Toxoplasma-specific IgM
B. serology for parvovirus B19 IgM
C. blood culture for Listeria monocytogenes
D. urine culture for cytomegalovirus
E. surface culture for herpes simplex virus

A

D. urine culture for cytomegalovirus

The infant described in the vignette has physical findings that are most consistent with congenital cytomegalovirus (CMV) disease and therefore should have a confirmatory urine culture for the virus. The clinical findings seen in an infant with symptomatic CMV infection include intrauterine growth retardation, jaundice, petechiae, hepatosplenomegaly, and, occasionally, blueberry muffin spots . Further evaluation may reveal chorioretinitis and cerebral calcifications. Diagnosis is often made by rapid CMV shell vial testing or culture of the urine. In addition, real-time polymerase-chain-reaction testing of saliva has recently been developed.

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20
Q

You are asked to speak to emergency medical service responders on the subject of pediatric head injury. As part of your presentation, you plan to emphasize the appropriate initial management of severe traumatic brain injury complicated by increased intracranial pressure in preventing secondary injuries. Of the following, the finding MOST closely associated with a poor outcome in such patients is

A. hypertension
B. hyperoxia 
C. hypotension 
D. hypercarbia
E. hypocarbia
A

C. hypotension

In an effort to prevent secondary injury, assurance of adequate ventilation, oxygenation, and perfusion is the first priority in the initial and ongoing management of patients with increased intracranial pressure. Children who did not receive treatment for hypotension were 3.4 times as likely to die and 3.7 times as likely to suffer disability.

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21
Q

You are called to evaluate a newborn infant who has cyanosis shortly after birth. The infant was born at 41 weeks’ gestation by spontaneous vaginal delivery and he weighs 3,800 grams. Results from the prenatal testing were unremarkable, including negative group B streptococcal status screening. Artificial rupture of the membranes occurred 3 hours before delivery and revealed scant clear fluid. The infant emerged vigorous. After bonding with his mother, he was brought to the warmer where he was noted to be dusky. His pulse oximetry reading was 78% on his right hand on room air. On arrival to the nursery, you find a cyanotic infant breathing comfortably in a 100% oxygen hood. His vital signs include a heart rate of 140 beats/min, respiratory rate of 50 breaths/min, blood pressure of 68/34 mm Hg, and temperature of 37°C. Auscultation reveals clear lung fields bilaterally and a 2/6 soft systolic ejection murmur over the left sternal border. You place pulse oximetry probes on his right hand and left foot, obtaining saturation readings of 89% and 77%, respectively.

Of the following, the MOST likely diagnosis is:

A. total anomalous pulmonary venous return 
B. transposition of the great vessels 
C. persistent pulmonary hypertension 
D. tricuspid atresia 
E. group B streptococcal pneumonia
A

C. persistent pulmonary hypertension

The infant described in the vignette demonstrates clinical findings that are suggestive of persistent pulmonary hypertension (PPHN).

In infants with PPHN, increased pulmonary pressure leads to right-to-left shunting at the level of the patent foramen ovale (PFO) and the patent ductus arteriosus (PDA). Oxygenated blood that has passed through the lungs flows into the right subclavian and right carotid artery via the ascending and transverse aorta. The deoxygenated blood flowing right to left through the PDA then mixes with the oxygenated blood in the descending aorta. This leads to the clinical finding of the preductal (right han) oxygen saturation that measures more than 10 points higher than the postductal (left foot) oxygenation saturation.

In most infants who have cyanotic heart disease, including tricuspid atresia and total anomalous venous return, the preductal and postductal oxygen saturations are the same. The preductal oxygen saturation may be 10 or more points lower than the postductal oxygen saturation in select types of cyanotic heart disease, including some forms of transposition of the great vessels

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22
Q

A 14-year-old girl comes in because of a dark rash around her neck that she first noticed 4 or 5 months ago. She has been otherwise healthy. The girl had menarche at 11 years of age, and her periods are fairly regular. Findings on physical examination are normal except for a body mass index of 28.2 and a velvety thickening of the skin on the sides and nape of her neck

[IMAGE acanthosis nigracans on nape of neck]

Of the following, the MOST appropriate laboratory evaluation for this patient is

A. antinuclear antibody and rheumatoid factor
B. antithyroglobulin antibody
C. fasting blood glucose and insulin levels
D. potassium hydroxide preparation of a skin scraping
E. testosterone, follicle-stimulating hormone, luteinizing hormone
A

C. fasting blood glucose and insulin levels

Acanthosis nigricans (AN) in children and adolescents is most often a manifestation of obesity-related insulin resistance

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23
Q

A 14-year-old girl who has a history of juvenile idiopathic arthritis is brought to the emergency department with severe epigastric abdominal pain. She has been taking ibuprofen to control her joint pains. Physical examination demonstrates a well-developed, well-nourished girl in moderate distress from pain. The remainder of the general physical examination shows direct tenderness in the epigastrium without rebound. The rectal examination shows normal sphincter tone and a scant amount of dark brown, hemoccult-positive stool in the rectal vault. Initial laboratory data include the following:

  • White blood cell count, 10,500/µL (10.5 × 109/L)
  • Hemoglobin, 11.7 g/dL (117 g/L)
  • Erythrocyte sedimentation rate, 32 mm/h
  • Serum electrolytes, normal
  • Blood urea nitrogen, normal
  • Creatinine, normal

Of the following, the MOST likely mechanism responsible for her symptoms is

	A. decreased pepsinogen production
	B. duodenal stress ulceration
	C. gastritis induced by peptidoglycans
	D. hypersecretion of gastric acid
	E. inhibition of prostaglandin synthesis
A

E. inhibition of prostaglandin synthesis

The girl described in the vignette presents with abdominal pain, hemoccult-positive stools, and anemia. These findings indicate chronic gastrointestinal blood loss, and the presence of epigastric tenderness strongly suggests a lesion in the upper gastrointestinal tract (ie, proximal to the ligament of Treitz). The most likely diagnosis is gastritis or peptic ulcer disease caused by her use of ibuprofen. Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, inhibit prostaglandin synthesis. Prostaglandin E stimulates gastric mucus production and helps maintain the protective mucin glycoprotein-bicarbonate layer, without which gastric mucosa is exposed to the deleterious effects of gastric acid as well as to the proteolytic effects of chief cell-produced pepsin, which can result in gastritis or peptic ulcers.

24
Q

A 14-year-old boy presents with fever, joint aches, and a rash for the past 3 days. He was started on amoxicillin for a sore throat a week ago. One of his classmates had a recent diagnosis of strep throat. He has a history of being successfully treated for acute otitis media with amoxicillin a month ago and has been well in the interim. On physical examination, he has swollen lymph nodes, periarticular swelling, and dusky urticaria-like lesions on his skin

[IMAGE erythematous urticarial macular rash on leg]

Of the following, these findings are MOST likely due to

	A. amoxicillin allergy
	B. infectious mononucleosis
	C. penicillin allergy
	D. scarlet fever
	E. serum sickness–like reaction
A

E. serum sickness–like reaction

This teenager has the symptoms of a serum sickness–like reaction to amoxicillin. The cardinal features of classical serum sickness are rash, fever, and polyarthralgia or polyarthritis, which begin 1 to 2 weeks after first exposure to the responsible agent and resolve within a few weeks of discontinuation.

Serum sickness–like reactions typically involve a constellation of signs and symptoms, which can include arthralgias, lymphadenopathy, and urticarial rash with or without fever. Fever, when present, is typically low-grade. Children with serum sickness–like reactions may present with acute onset of joint pain that often leads to inability to walk. The most characteristic rash is an urticarial or serpiginous macular rash that starts in the anterior lower trunk, groin, periumbilical, or axillary regions, and spreads to the back, upper trunk, and extremities. The rash generally lasts a few days to 2 weeks.

25
A 14-year-old boy presents to the emergency department with a several-hour history of fever, headache, and increasing lethargy. On physical examination, his temperature is 39.5°C, his heart rate is 115 beats/min, and his respiratory rate is 20/min. His growth parameters are in the 50th percentile for his age. He has nuchal rigidity and a faint petechial rash on his ankle. Results from the remainder of his physical examination are normal. In the last 2 years, he has had 2 other episodes of invasive bacterial infection caused by this organism, including bacteremia and a previous episode of meningitis. Of the following, the test MOST likely to establish the underlying cause of this boy’s recurrent infections is: A. a CH50 test B. a serum immunoglobulin test C. abdominal ultrasonography D. a human immunodeficiency virus antibody test E. magnetic resonance imaging of the brain and spine
A. a CH50 test The adolescent described in the vignette has recurrent invasive bacterial disease caused by Neisseria meningitidis. Terminal complement (C5, C6, C7, C8, or C9) and properdin (alternative pathway) deficiencies increase the risk of invasive meningococcal disease. The CH50 test screens for the combined activity of terminal complements C1 to C9 and would be the best initial diagnostic test for the boy described in the vignette.
26
A 14-year-old boy presents to the emergency department and complains of left-sided scrotal pain for the past few hours. He feels nauseated and has vomited once but has no urinary symptoms. On physical examination, his left testicle is high in the scrotum and no cremasteric reflex can be elicited. The scrotal skin is normal, but the left testicle is slightly swollen and very tender to palpation. The results of the remainder of his examination, including his abdominal examination, are unremarkable. Of the following, the MOST likely diagnosis for this boy is ``` A. acute epididymitis B. testicular tumor C. incarcerated inguinal hernia D. torsion of the appendix testes E. torsion of the testicle ```
E. torsion of the testicle Torsion may occur at any age, but it occurs most commonly during the neonatal and adolescent years. In addition to scrotal pain, the patient may have gastrointestinal symptoms, including nausea and vomiting. There may be a vague history of trauma and past episodes of mild pain that suggest partial twists that spontaneously resolved. These patients often have testicles that lie horizontally in the scrotum with a “bell clapper” deformity that predisposes them to twist. The testicle moves superiorly in the scrotum when it twists, resulting in an absent cremasteric reflex; rarely, torsion can be bilateral. Another cause of scrotal pain is torsion of the vestigial appendix testes or the appendix epididymis. Examination may reveal a painful small mass on the upper pole of the testis that is often seen through the scrotal skin as a blue mass and referred to as the “blue dot sign” Unlike in testicular torsion, in both epididymitis and torsion of the appendix testis, the testis is oriented vertically and is not elevated in the scrotum.
27
A 14-year-old girl presents to the emergency department for evaluation of severe headache, fever, and lethargy. The parents note that she first became ill with an acute upper respiratory illness with congestion and low-grade fever. The parents also report that over the past few days the girl has had an elevated temperature of 39.1°C, complaints of increasing frontal headaches (greater on the left side), and increased sleepiness. On physical examination, the girl is uncomfortable and awake but sleepy. On examination of head, eyes, ears, nose, and throat, there is tenderness over the frontal sinuses. Her neck is supple. Neurologic examination reveals a left sixth cranial nerve palsy. The remainder of her examination is unremarkable. Computed tomography scan with contrast reveals pansinusitis with meningeal enhancement over the left frontoparietal lobe and an epidural collection of approximately 2 cm. Of the following, the BEST choice for empirical antibiotic therapy, pending neurosurgical evaluation, is ``` A. ampicillin and gentamicin B. cefazolin and gentamicin C. ceftriaxone and vancomycin D. metronidazole and vancomycin E. nafcillin and vancomycin ```
C. Ceftriaxone and Vancomycin The child described in the vignette has a brain abscess documented on computed tomography scan. Brain abscesses can arise as a complication of a number of infections through direct extension (eg, sinusitis, mastoiditis, and odontogenic infections) or hematogenous spread (eg, endocarditis), especially in children with cyanotic congenital heart disease. Trauma and surgical procedures are other predisposing factors for development of brain abscesses. These infections are frequently polymicrobial. In this case, the epidural abscess arose as a direct extension from frontal sinusitis, so the likely organisms reflect the pathogens of sinusitis: Streptococcus pneumoniae, Haemophilus influenzae, other streptococcal species, anaerobes, and, less likely, Staphylococcus aureus. Of the listed regimens, ceftriaxone and vancomycin provides coverage for these organisms, and these drugs adequately cross the blood-brain barrier.
28
The parents of a 1-month-old infant who was found to have a solitary kidney in utero are asking questions about the status of the infant’s kidney function. The infant is otherwise healthy. Upon the insistence of the family, a serum creatinine and ultrasonography are performed. The serum creatinine is 0.5 mg/dL. Renal ultrasonography demonstrates a 5.5 cm kidney on the right side of the body and absent kidney on the left.Of the following, the statement that BEST characterizes the renal status of the infant is A. nephrotoxic agents should be minimized because of decreased nephron mass B. this infant is likely to require dialysis by adolescence C. this infant will require annual ultrasonography to screen for renal malignancy D. this infant is at higher than usual risk for dehydration in the setting of gastroenteritis E. toilet training may be more challenging in the future for this infant
A. nephrotoxic agents should be minimized because of decreased nephron mass Renal Agenesis. Avoidance of nephrotoxic medications such as nonsteroidal anti-inflammatory drugs, intravenous radiocontrast, and aminoglycoside antibiotics should be considered in children with a single kidney because of the reduced renal reserve in these patients.
29
An 18-month-old boy is brought to your clinic because of redness of his left cheek that his mother noticed yesterday. He has been otherwise well and has had no fever. Physical examination findings reveal a tender, erythematous area of slight induration on his left cheek. His mother tells you that the boy recently started sucking ice chips. [Baby w/ red spot on cheek] Of the following, the MOST likely diagnosis is ``` A. cold panniculitis B. cellulitis C. dental abscess D. juvenile xanthogranuloma E. lipoma ```
A. Cold Panniculitis Cold panniculitis, also known as “popsicle panniculitis,” is a lesion of the skin caused by prolonged exposure to cold objects. Sucking on popsicles or ice chips results in the most common presentation on the cheeks, but any area of the body exposed to cold can be affected. The mechanism of pathogenesis is similar to that of fat necrosis and likely occurs because in infants the subcutaneous fat is more apt to solidify than in adults. Typical lesions are bluish red nodules that may be painful. They usually arise within 1 to 2 days of cold exposure, and they may persist for several weeks.
30
A 9-year-old boy is brought to your office after several visits to the emergency department because of severe abdominal pain. During these episodes, numerous blood and urine tests were obtained, and all the results of these studies were reported to be within reference limits. During one of these episodes, the boy vomited a large amount of yellow-green material, and the pain resolved shortly thereafter. Physical examination demonstrates a happy, well-appearing child whose height and weight are both at the 75th percentile for age. His abdomen is soft, nondistended, nontender, and without masses or organomegaly. Results from the remainder of the physical examination are unremarkable. Of the following, you are most likely to recommend A. abdominal ultrasonography B. a referral for endoscopy C. a referral to a psychologist D. routine follow-up with instructions to call immediately if symptoms recur E. an upper gastrointestinal tract radiographic series
E. an upper gastrointestinal tract radiographic series The boy described in the vignette has been evaluated on several occasions for abdominal pain without a clear diagnosis. During one of these episodes, he experienced bilious emesis. Although he now appears well, the occurrence of this symptom, even by history alone, should always suggest a diagnosis of small bowel obstruction. Statistically, the sudden onset of abdominal pain and bilious emesis in a previously well child, adolescent, or adult is most frequently associated with intestinal malrotation, and the diagnostic study of first choice is an upper gastrointestinal (GI) radiographic series. Older children often present with a history of chronic, recurrent abdominal complaints, including pain, vomiting (bilious or nonbilious), and diarrhea that are secondary to intermittent midgut volvulus, which can lead to progressive intestinal ischemia and become life threatening.
31
A mother who has had no prenatal care delivers a healthy newborn with a normal physical examination at term. The mother is found to be infected with human immunodeficiency virus, and the infant is started on antiretroviral prophylaxis.Of the following, the MOST appropriate initial test to send for detecting HIV in this infant is ``` A. a p24 antigen test B. a DNA polymerase chain reaction test C. a viral culture D. an RNA polymerase chain reaction test E. an immunoglobulin G antibody test ```
B. a DNA polymerase chain reaction test The infant described in the vignette has been exposed to HIV in utero and may be infected. Detection of viral nucleic acid using the HIV DNA polymerase chain reaction (PCR) assay is most helpful for making the diagnosis in infants bc detection of antibody (immunoglobulin G) to HIV in infants is not helpful because of the presence of transplacentally acquired maternal antibodies.
32
A resident who is rotating with you in the newborn nursery asks for advice on the management of a 4.1-kg, 1-day-old male newborn whose heel stick specimen revealed an initial glucose value of 45 mg/dL (2.5 mmol/L). Results from repeat testing after the infant was fed revealed a blood glucose concentration of 82 mg/dL (4.6 mmol/L). A third glucose test performed 24 hours after birth revealed a blood glucose concentration of 95 mg/dL (5.3 mmol/L). The resident reports that on physical examination the infant is sleepy but easily aroused and in no acute distress. The remainder of the results of his physical examination are normal except that his penis appears to be less than 2 cm in length. Of the following, the MOST appropriate next step in the management of this infant is to A. obtain a stretched penile length measurement B. begin an intravenous glucose infusion C. obtain a complete blood cell count and blood and urine culture D. measure gonadotropin and growth hormone concentrations E. obtain head magnetic resonance imaging
A. obtain a stretched penile length measurement The infant described in the vignette has hypoglycemia and a penis that appears to be less than 2 cm in length. These findings should suggest the possibility of panhypopituitarism (eg, growth hormone, gonadotropin, and corticotropin deficiency). Infants who have panhypopituitarism may present with both hypoglycemia due to lack of insulin counterregulatory hormones (growth hormone and cortisol) and micropenis due to inadequate exposure in utero to growth hormone and gonadotropins (luteinizing hormone and follicle-stimulating hormone).With the immediate concern for hypoglycemia alleviated, the most appropriate next step in this boy’s evaluation is to determine whether he has micropenis. Inspection is not an adequate technique for assessing penile size. Instead, a stretched penile length (SPL) measurement should be obtained.
33
The case manager of an 18-month-old child is concerned because the child is having recurrent wheezing episodes. The infant was hospitalized at 6 months of age with confirmed respiratory syncytial virus (RSV) infection. The wheezing sometimes but not always responds to albuterol treatment. There is no maternal history of asthma or allergies. The case manager is concerned that these episodes may be because of maternal neglect. Of the following, you are MOST likely to tell the case manager that after an initial RSV infection, the child A. the recurrent wheezing represents RSV colonization and inadequate preventive care B. can have recurrent wheezing which consistently responds to albuterol treatment C. can have recurrent wheezing occur in up to 50% of babies with RSV bronchiolitis D. should not have recurrent wheezing if there is no maternal history of asthma or atopy E. should not have wheezing for more than 6 months after hospitalization
C. can have recurrent wheezing occur in up to 50% of babies with RSV bronchiolitis Respiratory syncytial virus (RSV) respiratory infections are the most common cause of virus-induced wheezing in infants and children less than 2 years of age. Acute wheezing occurs in approximately 80% of children hospitalized with RSV infection. Infants who have been hospitalized with RSV LRTI have been shown to have an increased risk of wheezing episodes after the hospitalization compared to infants who have not been hospitalized with RSV LRTI.
34
A 16-year-old girl who plays lacrosse is seen in your office with a 2-month history of chest pain during activity. Episodes of pain last 3 to 5 minutes and have been associated with dizziness, palpitations, and presyncope. Her physical examination is normal with the exception of a soft 2/6 systolic ejection murmur at the lower left sternal border. You obtain electrocardiogram results that are interpreted as normal. Of the following, the MOST appropriate next step in evaluation of this teenager is to A. order magnetic resonance imaging of the brain with contrast B. order serum C-reactive protein and white blood cell count with differential C. order tilt-table testing D. reassure her and her family that no further testing is needed E. restrict her from exercise pending evaluation by a cardiologist
E. restrict her from exercise pending evaluation by a cardiologist The girl in the vignette has chest pain during exercise associated with lightheadedness and presyncope, symptoms that raise suspicion for a potentially serious disorder. Her physical examination reveals a systolic murmur at the lower left sternal border. If the murmur increases in intensity when the patient changes posture from lying down to sitting or standing, hypertrophic obstructive cardiomyopathy should be suspected. In view of this, she should be referred to a cardiologist (promptly) and restricted from exercise pending that evaluation.
35
A 15-year-old boy is brought to your office and complains of neck pain after diving into the shallow end of a backyard pool. He reports that when his head struck the bottom of the pool, he felt immediate pain in his neck and tingling in his arms. On examination, he has a contusion on the top of his head and significant point tenderness over cervical vertebrae 5 and 6. He has mildly decreased grip strength in his left hand. Results of his neurologic examination are otherwise normal.Of the following, the MOST appropriate next step in this patient’s management is to A. obtain a cervical spine computed tomography B. obtain cervical spine radiographs C. immobilize his cervical spine D. administer analgesia E. refer him for emergent neurosurgical evaluation
C. immobilize his cervical spine These complaints should raise clinical suspicion for a cervical spine or spinal cord injury. The most appropriate immediate intervention is to immobilize the patient’s entire spine while simultaneously assessing the patient’s vital functions.
36
A 15-year-old girl is brought to your clinic by her mother for fatigue and refusal to eat meat for the past year. The mother read on the internet that vegetarianism can cause anemia and asks to have her daughter, a vegetarian, tested. You order laboratory tests to rule out iron deficiency. Of the following, the laboratory findings that would be MOST consistent with frank iron deficiency anemia in this patient are ``` A. Hg 9, MCV 105, Serum Ferritn 100 B. Hg 10, MCV 95, Serum Ferritin 40 C. Hg 9, MCV 85, Serum Ferritin 100 D. Hg 12, MCV 70, Serum Ferritin 40 E. Hg 9, MCV 70, Serum Ferritin 10 ```
E. Hg 9, MCV 70, Serum Ferritin 10 The adolescent girl in the vignette has frank iron deficiency anemia, as indicated by low values for hemoglobin (Hb), mean corpuscular volume (MCV), and ferritin.
37
A 16-year-old boy is brought to the emergency department because he had a seizure after returning home from a party with his friends. His mother reports that he was agitated and aggressive when he first arrived home about 45 minutes ago. He then became unresponsive and developed generalized extremity shaking that lasted for approximately 5 minutes. He has a history of marijuana abuse, but his friends report that he only smoked a cigar. In the emergency department, he is difficult to arouse, his heart rate is 140 beats/min, his respiratory rate is 30 breaths/min, his blood pressure is 135/95 mm Hg, and his pupils are midsized, equal, and sluggishly reactive with notable nystagmus. Of the following, the MOST likely explanation for his symptoms is ``` A. concussion B. epilepsy C. inhalant abuse D. nicotine toxicity E. phencyclidine exposure ```
E. Phencyclidine Exposure The patient in the vignette is most likely exhibiting signs consistent with phencyclidine exposure. Tachycardia, hypertension, agitation, seizures, and nystagmus are hallmarks of this exposure. Loss of consciousness is not commonly caused by concussion, and, if it occurs, is typically of short duration. The symptoms of inhalant exposure are also typically short lived, resolving within 30 to 45 minutes. Symptoms from inhalant exposure generally are related to central nervous system stimulation or depression and include dizziness, excitation, lethargy, hallucinations, and ataxia.
38
A 15-year-old girl presents with burning on urination and pain in her vulva for the last 2 days. She has no fever or other systemic symptoms. She admits to oral sexual activity but has never had vaginal intercourse. On examination, you note a few blisters and some small ulcers on her vulva. She has no visible vaginal discharge. You decide to give her medications for pain while awaiting the results of your tests. Of the following, the test is MOST likely to confirm your diagnosis is A. culture of blister fluid for herpes simplex virus with typing B. IgM and IgG serology with typing for herpes simplex virus C. serologic testing for syphilis via rapid plasma reagin test D. urinalysis and culture E. urine nucleic acid amplification test for gonorrhea and chlamydia
A. culture of blister fluid for herpes simplex virus with typing HSV. The best method to diagnose the infection is by polymerase chain reaction testing (preferred because of greater sensitivity) or culture of vesicular fluid (by unroofing a blister). The sensitivity of viral culture decreases as lesions heal, so sampling a vesicle if one is present is preferred. Typing is important because subclinical viral shedding and recurrences are much less frequent with HSV-1 infections than with HSV type 2 (HSV-2). For patients who have no active lesions but a history of genital ulcers, diagnosis must rely on serologic testing.
39
A 15-year-old boy who has idiopathic partial epilepsy presents to the emergency department after a prolonged seizure. His maintenance medication is oxcarbazepine. Of the following, the MOST appropriate next step in managing this patient is to A. obtain emergent magnetic resonance imaging of the brain B. change his medication C. obtain serum calcium, magnesium, and phosphorus concentrations D. schedule outpatient electroencephalography E. obtain a serum oxcarbazepine concentration
E. obtain a serum oxcarbazepine concentration
40
A 4-year-old girl enters your practice for the first time. Her mother reports that her birth history was unremarkable and early developmental milestones were within normal limits. However, over the past year her development has seemed to plateau. Her medical history is significant for recurrent ear infections and, more recently, rather loud snoring. On physical examination, you note slightly coarse facial features, a protuberant abdomen with a small reducible umbilical hernia, enlarged liver, and mild contractures of the fingers. Of the following, according to these findings, the test that would be MOST diagnostically helpful is ``` A. a lipid profile B. plasma amino acids C. a sleep study D. urine glycosaminoglycans E. urine organic acids ```
D. urine glycosaminoglycans The clinical findings described in this vignette are typical for a child with a form of mucopolysaccharidosis (MPS) disorder. Development for many children with an MPS disorder is initially normal, subsequently plateaus, and then regresses because of accumulation of metabolic by-products (glycosaminoglycans). This is typical of some MPS subtypes, including Hurler, Hunter, and Sanfilippo syndromes. Diagnostic screening includes urine testing for glycosaminoglycans and definitive testing for specific enzymes, depending on the findings on the urine test. Recurrent otitis media and snoring secondary to tonsillar and adenoidal hypertrophy are also quite common early features. Later findings include bony changes (dysostosis multiplex), joint contractures (especially in the fingers), hepatosplenomegaly, and secondary umbilical hernias.
41
A 6-year-old girl who has corrected congenital heart disease and a history of furunculosis presents to the emergency department with fever and increased work of breathing. Physical examination reveals a temperature of 39.5°C, heart rate of 130 beats/min, respiratory rate of 30 breaths/min, and blood pressure of 85/55 mm Hg. Her oxygen saturation is 90% in room air. A new blowing murmur is noted on cardiac examination, and auscultation of the chest reveals diffuse crackles bilaterally. She has hepatosplenomegaly and a capillary refill of 3 to 4 seconds. Her skin examination yields normal results. Her white blood cell count is 30.0 × 103/µL (30.0 × 109/L), with 71% polymorphonuclear leukocytes, 23% lymphocytes, and 6% monocytes. Chest radiograph shows diffuse pulmonary interstitial edema. Of the following, the MOST appropriate initial antibiotic therapy for this patient is ``` A. ampicillin-sulbactam B. ceftazidime C. doxycycline D. trimethoprim-sulfamethoxazole E. vancomycin ```
E. vancomycin The patient described in the vignette presents with clinical sepsis, and her history of corrected congenital heart disease and furunculosis in conjunction with fever and a new murmur on physical examination strongly suggests acute infectious endocarditis (IE). In children with a fulminant presentation of IE, Staphylococcus aureus is the most common cause. Therefore, prompt initiation of antimicrobial therapy with a bactericidal agent such as vancomycin is warranted. ancomycin is a glycopeptide antibiotic that inhibits bacterial cell wall synthesis by binding tightly to peptidoglycan precursors (D-alanyl-D-alanine portion) and blocking polymerization. +MRSA coverage
42
A 5-year-old boy presents to the pediatrician’s office for increased bruising. He had cough and rhinorrhea 1 week ago that have now resolved. There is no fever, bone pain, or weight loss. His activity level has been normal. On physical examination, he has large bruises on his arms, trunk, and both shins, petechiae on the face, and purpura in the oral mucosa. There is no hepatosplenomegaly or lymphadenopathy. The following are the results of the boy’s complete blood cell count: * White blood cell count, 7,500/µL (7.5 × 109/L), with 45% polymorphonuclear leukocytes, 46% lymphocytes, 6% monocytes, and 3% eosinophils * Hemoglobin, 12.3 g/dL (123 g/L)• Platelet count, 5 × 103/µL (5 × 109/L) * Serum uric acid, normal * Lactate dehydrogenase, normal Of the following, the MOST likely finding on bone marrow examination of this patient is ``` A. decreased bone marrow cellularity B. increased megakaryocytes C. increased lymphoblasts D. myelodysplasia E. decreased megakaryoblasts ```
B. increased megakaryocytes Thrombocytopenia can occur because of either decreased platelet production in the bone marrow or increased platelet destruction in the circulation. Immune thrombocytopenia purpura (ITP) is the most common cause of acquired thrombocytopenia in children. TP is a diagnosis of exclusion and is based on the following criteria: (1) platelet count less than 100 × 103/µL (100 × 109/L) with no other cytopenias or abnormalities on the peripheral blood smear; and (2) the absence of other clinical conditions that could cause thrombocytopenia. In ITP, the bone marrow typically shows normal to increased numbers of megakaryocytes most likely as a compensatory mechanism in response to the increased destruction of platelets.Decreased marrow cellularity or decreased megakaryoblasts would suggest aplastic anemia or bone marrow failure. Increased lymphoblasts or myelodysplasia would be concerning for malignancy.
43
A 6-year-old girl is brought to the clinic because of vulvar and anal itching over the last week. Her mother denies vaginal discharge or stool abnormalities. Physical examination findings reveal mild anal erythema and a normal vulva with no vaginal discharge. Microscopic examination of tape placed on the anus reveals the structures shown below: [PINWORM EGGS] Of the following, the MOST appropriate pharmacologic agent to use is ``` A. albendazole B. anidulafungin C. benznidazole D. ciprofloxacin E. itraconazole ```
A. albendazole In children, infection with the roundwormEnterobius vermicularis, or pinworm, is a common cause. Adult female pinworms reside in the large intestine and migrate to the perianal and perineal area at night, depositing up to thousands of eggs on the skin. Transmission of the eggs from one person to another occurs via the fecal-oral route. Eggs can survive up to 3 weeks and are ingested from fingernails, bedding, toys, or other surfaces; autoinfection occurs as well. Albendazole and pyrantel pamoate (preggers) may be used to treat pinworm infection. For both drugs, a single dose is administered and then repeated in 2 weeks.
44
A 6-year-old boy presents to the emergency department with a prolonged seizure in the absence of a fever. Results from laboratory studies show that he has severe hypocalcemia. Consequently, he is started on calcitriol. In speaking to his parents, you discover that he has been receiving speech therapy since 2 years of age for expressive language delays and a hypernasal voice quality. A chest radiograph performed when he was 3 years old during a hospitalization for a bacterial pneumonia showed that he had an incidental right-sided aortic arch. Of the following, the test which would be MOST helpful in determining the cause of these findings is ``` A. a cytogenetic analysis B. a nasendoscopy C. parathyroid hormone measurement D. 22q fluorescence in situ hybridization E. urine electrolytes ```
D. 22q fluorescence in situ hybridization he child described in this vignette has many of the classic features of DiGeorge syndrome or velocardiofacial syndrome (DGS/VCFS), which is caused by a microdeletion on chromosome 22q11. This contiguous gene syndrome is best identified using a fluorescent (FISH) probe that is specific for this cytogenetic region and may or may not be detected on a routine cytogenetic study. Classic findings seen in some patients with a 22q11 deletion include heart defects (conotruncal heart defects, ventricular septal defects, right-sided aortic arch), velopharyngeal insufficiency, cleft palate, learning disabilities, intellectual disabilities, speech delays, significant hypocalcemia secondary to hypoparathyroidism, and recurrent infections secondary to immune (T-cell) defects caused by thymic hypoplasia. In addition, there is a fairly high rate of behavioral or psychiatric disturbances in teens and young adults
45
A 5-year-old boy presents with a history since 2 years of age of coughing, swelling of the lips, mouth, and eyes, and vomiting after ingestion of peanut butter. He has been seen by an allergist in the past. Skin prick testing showed a wheal of 10 mm and a flare of 20 mm to peanut. His reaction to histamine showed a wheal of 7 mm and a flare of 15 mm. He did not react to the negative control. He has been avoiding peanuts and nut products. His mother is worried about risk-taking behaviors in teens and wants to know if her son will outgrow his peanut allergy by the time he is in high school.Of the following, the BEST information to tell the boy’s mother is that A. you will send him to the allergist for periodic skin testing to see if his reactivity decreases over time B. he is likely to outgrow his allergy if he strictly avoids peanuts for 3 to 5 years C. avoiding soy and other legumes will help him outgrow his peanut allergy D. he is unlikely to outgrow his peanut allergy by adolescence E. daily antihistamine therapy will help him outgrow his peanut allergy
D. he is unlikely to outgrow his peanut allergy by adolescence Peanut allergy is usually considered a lifelong food allergy (FA).
46
A 2-year-old-boy with sickle cell disease presents to the emergency department with abdominal pain. His mother reports that he has been less active and complaining of left-sided abdominal pain for the past 24 hours. There is no history of emesis, diarrhea, or cough. He was drinking well and his appetite was normal until this morning. He has been taking folic acid and prophylactic penicillin (125 mg orally twice a day). His temperature is 37.8°C, his pulse rate is 150 beats/min, his respiratory rate is 30 breaths/min, his oxygen saturation is 99% on room air, and his blood pressure is 90/40 mm Hg while sleeping on the examining table. His lips are pale, and the spleen edge is palpable 5 cm below the left costal margin. There is a 2/6 systolic murmur at the left sternal border with no gallop rhythm. The remaining physical examination findings are unremarkable. The following are the results of the boy’s complete blood cell count: * White blood cell count, 13,500/µL (13.5 × 109/L) * Hemoglobin, 6.0 g/dL (60 g/L) * Platelet count, 135 × 103/µL (135 × 109/L) Of the following, the MOST appropriate next step in management is to A. administer a bolus of 20 mL/kg of normal saline B. transfuse 10 mL/kg of packed red blood cells C. initiate continuous intravenous morphine D. administer ceftriaxone intravenously E. administer supplemental oxygen
B. transfuse 10 mL/kg of packed red blood cells The child in the vignette who has sickle cell disease presents with lethargy, pallor, and splenomegaly and is found to have a low hemoglobin level and thrombocytopenia. Accordingly, splenic sequestration must be presumed until proven otherwise. The most appropriate treatment for this patient would be a packed red blood cell transfusion to rapidly restore intravascular volume and oxygen-carrying capacity.
47
A 2-year-old girl presents after a prolonged focal-onset seizure. She was playing when she suddenly started crying and her right arm started jerking. Her whole body then jerked for 10 to 15 minutes, during which time she could not respond to her mother. The jerking was subsiding when the paramedics arrived, and the child slept en route to the emergency department. The girl was born at 26 weeks’ gestation and has developmental delay. She sits but does not yet walk. Of the following, the STRONGEST predictor of seizure recurrence in this child is ``` A. exposure to brother’s video games B. family history of febrile seizures C. minor trauma the day before the visit D. preexisting neurologic impairment E. stress associated with visiting the biological father ```
D. preexisting neurologic impairment The girl in the vignette has preexisting neurologic impairments that are likely related to complications of prematurity. A preexisting neurologic problem is the most powerful overall predictor of seizure recurrence after a first unprovoked seizure.
48
A 2-year-old boy you have followed up since birth presents to your office for a health supervision visit. He was born at term and has had normal growth and development. The mother recently noticed that he looks “cross-eyed” at times. There is no family history of eye abnormalities. On physical examination, his left eye is deviated nasally with a white pupillary reflex. There is no proptosis, conjunctival injection, or pain with eye movements. The remainder of results for the physical examination are normal. Results of the complete blood cell count are within normal limits. [Unilateral White Pupillary Reflex] Of the following, the MOST likely diagnosis for this patient is: ``` A. bilateral retinoblastoma B. congenital cataract C. leukemia with chloroma D. neuroblastoma E. unilateral retinoblastoma ```
E. unilateral retinoblastoma One of the most common causes of leukocoria (white pupillary reflex) in children is retinoblastoma (47% of cases); therefore, all children with a new finding of leukocoria should be referred immediately to an ophthalmologist who is experienced in examining children. Other causes include persistent fetal vasculature, retinopathy of prematurity, cataracts, optic disc abnormalities, uveitis, or vitreous hemorrhage. Retinoblastoma -Leukocoria and strabismus are the most common presenting findings. Other findings may include decreased vision, ocular inflammation, vitreous hemorrhage, hyphema, orbital cellulitis, proptosis, glaucoma, eye pain, fever, and a family history of retinoblastoma. The diagnosis of retinoblastoma is usually made on the basis of clinical examination, and the presence of calcification on computed tomography or ocular ultrasonography. In this vignette, the age of the child, the lack of findings in right eye, and the relatively higher frequency of unilateral disease over bilateral disease make unilateral retinoblastoma the most likely diagnosis in this patient.
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A 3-month-old infant is brought to your office because of rectal bleeding. His birth weight was 3,500 grams. He had been breastfed until 2 weeks ago when he was weaned to a cow milk protein-based formula. For the past week, his mother has noted streaks of blood and mucus in his stool that occurs 2 to 3 times per day. Although he has remained active, alert, and afebrile, he appears uncomfortable when passing a stool. On examination today, he weighs 6,900 grams; results from the remainder of the general physical examination are unremarkable except for a small anal fissure. If the following, you are MOST likely to recommend A. a radioallergosorbent test (RAST) for cow milk protein B. a skin prick test for cow milk protein C. adding 4 ounces of diluted prune juice daily to his feedings D. changing to a casein hydrolysate formula E. changing to a soy protein formula
D. changing to a casein hydrolysate formula The infant described has a 1-week history of hematochezia that occurred after weaning to a cow milk protein–based formula. Results of his physical examination are unremarkable except for a small anal fissure. These findings are most consistent with a diagnosis of protein-induced, allergic proctocolitis. Considering this infant’s excellent weight gain and healthy appearance, the most appropriate therapeutic recommendation is to institute feedings with a casein hydrolysate formula. Caseins account for about 80% of these milk proteins. The remaining 20%, including lactalbumin, lactoglobulin, and bovine serum albumin, are contained in the whey fraction. Caseins manifest a low level of immunogenicity, owing in part to their flexible, bulky (ie, noncompact) structure. By contrast, lactoglobulin from the whey fraction is considered the major allergen responsible for cow milk protein intolerance, although sensitization to several whey proteins is observed in most patients with documented allergies. Protein-induced, allergic proctocolitis usually occurs in the first few months after birth and is most frequently associated with ingestion of cow-milk or soy proteins. The most common sign of allergic proctocolitis is blood-streaked but otherwise normal-appearing stools, with or without passage of mucus. Small anal fissures have been reported in up to 50% of cases, and affected infants generally appear healthy and have normal weight gain.
50
A 17-year-old girl in your practice comes to your office for evaluation of a febrile illness. She returned from a 2-week community service project helping to build houses in Costa Rica. The fevers began the day before her departure and have continued with daily temperatures up to 39.1°C. She also complains of severe headaches, diffuse body aches, fatigue, and nausea. She noted a diffuse pruritic rash yesterday. On physical examination, she is alert and uncomfortable but not toxic in appearance. The girl’s temperature is 39.2°C, her pulse rate is 104/min, her blood pressure is 106/60 mm Hg, and her respiratory rate is 18 breaths/min. There is a diffuse maculopapular rash on her trunk and extremities, and examination of her head, eyes, ears, nose, and throat reveals bilateral conjunctival injection and a pharynx with diffuse erythema and no exudates. There is diffuse, nontender cervical lymphadenopathy; result of her cardiac examination is normal; her abdomen is soft, nontender, and without hepatosplenomegaly; there are no joint abnormalities; and result of her neurologic examination is within reference range. The following are the patient’s laboratory results: * White blood cell count, 2,600/µL (2.6 × 109/L), with 30% neutrophils, 4% band neutrophils, 58% lymphocytes, and 8% monocytes * Hemoglobin, 12.2 g/dL (120 g/L)• Hematocrit, 36.3% (.36) * Sodium, 134 mEq/L (134 mmol/L) * Potassium, 3.6 mEq/L (3/6 mmol/L) * Chloride 102 mEq/L (102 mmol/L) * Bicarbonate, 22 mEq/L (22 mmol/L) * Blood urea nitrogen, 4 mg/dL (1.4 mmol/L) * Creatinine, 0.6 mg/dL (53 µmol/L) * Aspartate aminotransferase, 96 U/L * Alanine aminotransferase, 112 U/L * Total bilirubin, 0.6 mg/dL (10.3 µmol/L) Of the following, the test that is MOST likely to reveal the cause of her illness is ``` A. Epstein-Barr serology panel B. dengue virus serology (IgG, IgM) C. malaria smear D. Mantoux skin test E. hepatitis A serology (IgM) ```
B. dengue virus serology (IgG, IgM) The classic description of dengue fever (also known as breakbone fever) includes the affected individual demonstrating severe muscle and joint pains, headache, and retro-orbital pain in association with an acute febrile illness. Nonspecific rash, nausea, vomiting, diarrhea, and respiratory symptoms may also occur. Laboratory findings seen in dengue fever include leukopenia, thrombocytopenia, and a modest elevation of liver function tests.
51
A 2-year-old girl who has developmental delay presents to urgent care after a cyanotic episode at daycare. She was noted to have a look of fear and then confusion associated with slow breathing, a choking sound, and diminished awareness. Afterward, she seemed “out of it” and unlike herself. The parents believe that this is similar to several previous episodes, although the duration today was longer. Physical examination reveals that the child has small growth parameters, including head circumference. She sits but does not stand, and she has no language and poor eye contact. She has truncal unsteadiness and brings her hands to her mouth frequently. Of the following, the MOST likely diagnosis is ``` A. acute cerebellar ataxia B. ataxia telangiectasia C. Joubert syndrome D. Niemann-Pick disease type C E. Rett syndrome ```
E. Rett syndrome The girl in the vignette presented for medical attention after experiencing a seizure. According to her parents, it appears that several previous, unprovoked seizures have occurred, which is consistent with a diagnosis of epilepsy. In girls who have small growth parameters, developmental delay, truncal unsteadiness, and stereotypic midline hand behaviors, Rett syndrome should be considered. Rett syndrome is an X-linked dominant disorder caused most often by a mutation in the MECP2 gene. The diagnosis of Rett syndrome is made clinically and is the entity most likely responsible for the symptoms and signs exhibited by the girl in the vignette.
52
A 3-month-old term infant is brought to the emergency department for increased work of breathing and wheezing that began earlier that day. There is no fever, but she is unable to breastfeed because of severe nasal congestion. Her past medical history is unremarkable and this is her first respiratory illness. Vital signs include a temperature of 36.7°C, a respiratory rate of 60 breaths/min, and a heart rate of 140 beats/min. Pulse oximetry is 98% on room air. Mild nasal flaring and congestion are noted, and lung examination reveals subcostal retractions with wheezes and coarse crackles bilaterally. Of the following, the MOST appropriate therapy to initiate at this time is ``` A. ceftriaxone B. intravenous fluids C. chest physiotherapy D. racemic epinephrine E. methylprednisolone ```
B. intravenous fluids viral bronchiolitis is the most common respiratory illness in infants and young children. Inflammation and subsequent edema of the bronchioles lead to wheezing, crackles, hypoxemia, and accessory muscle use. The most common cause of bronchiolitis is respiratory syncytial virus, but influenza, parainfluenza, human metapneumovirus, and adenovirus are also responsible. Children who have bronchiolitis often experience difficulty feeding because of significant nasal congestion and tachypnea. All children with bronchiolitis should have a careful assessment of their hydration status, and intravenous or nasogastric fluids should be administered if oral intake is poor. Other important treatment measures include supplemental oxygen for oxygen saturation below 90% and nasal saline and suctioning as needed.
53
A 3-year-old boy is brought to the emergency department at 8 am after his parents found him unresponsive in bed. The last time they had seen him awake was at 2 am when they found him playing in the living room as they were cleaning up after a dinner party. On physical examination, the child has diaphoresis and moans to painful stimuli. His vital signs include a temperature of 35.8°C, heart rate of 105 beats/min, respiratory rate of 18 breaths/min, blood pressure of 84/34 mm Hg, and oxygen saturation of 97% in room air. His pupils are midsized and sluggishly reactive.Of the following, the MOST appropriate next step is to A. begin a dopamine infusion B. administer naloxone C. perform endotracheal intubation D. perform a bedside glucose determination E. obtain computed tomography of the head
D. perform a bedside glucose determination The patient in the vignette is exhibiting signs and symptoms of acute ethanol intoxication, concern for which should be heightened by the parents’ report of unsupervised activity in an environment where ethanol was accessible. Signs of exposure to a sedative-hypnotic agent include hypothermia, hypotension, bradycardia, and coma. Other signs of acute alcohol toxicity include ataxia, respiratory depression, and, in young children, hypoglycemia.The approach to any pediatric patient who presents with acute alteration in mental status should first include assessment of airway patency, adequacy of oxygenation, ventilation, and perfusion and a bedside glucose determination. Stabilization of vital functions and correction of hypoglycemia should be performed as indicated.
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A 3-year-old boy enters the examination room. His mother calls his name and he does not respond. He grabs your hand and puts it on a bottle of bubbles. His mother hands him a toy car that he turns over and begins to spin the wheels while saying the sound “eeh.” He then begins to jump up and down while looking out the window. According to his past history, he is speaking 5 single words on an inconsistent basis. He will primarily repeat words in a nonfunctional manner. He began walking at age 12 months. Of the following, this boy is MOST likely to have a diagnosis of ``` A. attention-deficit/hyperactivity disorder B. autism spectrum disorder C. cerebral palsy D. epilepsy E. language delay ```
B. autism spectrum disorder The child described in the vignette has impairment in his social communication and social interactions and exhibits restricted and repetitive behaviors. These behaviors are central features of the diagnostic criteria for an autism spectrum disorder (ASD).
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A 3-week-old newborn infant who was born at term experiences shortness of breath with feeds. Her mother had no prenatal care. Physical examination is remarkable for a blood pressure of 100/60 mm Hg in the right arm, redundant skin overlying the posterior neck (Item Q72A), and edema of the lower extremities (Item Q72B). There is a gallop rhythm and a regurgitant murmur at the mid-precordium; her femoral pulses are diminished. There are subcostal retractions and rales on auscultation of the lungs.Of the following, the MOST appropriate next step in the evaluation of this infant is ``` A. 12-lead electrocardiogram B. complete metabolic profile C. serum B-type natriuretic peptide D. blood chromosomal analysis E. chest radiograph ```
E. chest radiograph In the infant in the vignette, the edema of the feet and redundant skin overlying the posterior neck raise suspicion for Turner syndrome. The blood pressure measurement in the right arm was significantly elevated for a 3-week-old. The most appropriate next step in evaluating this newborn is to obtain a chest radiograph to assess for cardiomegaly, atelectasis, or pulmonary infiltrates.
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A 4-year-old boy with acute lymphoblastic leukemia who is on maintenance chemotherapy and has a central venous catheter is admitted to the hospital for management of a temperature greater than 38.9°C and neutropenia. Physical examination findings are normal. The following are the results of the laboratory tests: - Sodium, 134 mEq/L (134 mmol/L) - Potassium, 3.4 mEq/L (3.4 mmol/L) - Chloride, 102 mEq/L (102 mmol/L) - Bicarbonate, 24 mEq/L (24 mmol/L) - Blood urea nitrogen, 14 mg/dL (5 mmol/L) - Creatinine, 0.3 mg/dL (26.5 µmol/L) He begins empiric treatment with intravenous piperacillin-tazobactam (1.2 grams every 8 hours) and intravenous gentamicin (60 mg every 8 hours). After 3 days of therapy, a gentamicin level of 5.2 µg/mL (target range,
C. increase the interval between doses of gentamicin to every 12 hours and measure the gentamicin level again after 3 more doses Nephrotoxicity and ototoxicity are the major adverse events associated with aminoglycosides. Monitoring trough levels (serum level of drug obtained just before the fourth or fifth dose) may be useful in minimizing these adverse events. Prolonging the interval between doses or decreasing the dose can be used to address elevated trough levels.
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A 4-year-old boy presents with right-sided flank pain. His mother says she noticed a tinge of red in his urine earlier this morning. He plays often with his 2 older brothers, but there is no history of obvious trauma. There is no history of fever, frequency, or urgency, although his mother mentions that at times her son appears uncomfortable when voiding. On physical examination, his temperature is 37.3°C, his heart rate is 80 beats/min, his respiratory rate is 18 breaths/min, and his blood pressure is 106/62 mm Hg. You note no costovertebral angle tenderness, suprapubic tenderness, or edema. Urinalysis reveals the following results: * Specific gravity, 1.020 * pH, 6 * 2+ blood * Trace protein * Leukocyte esterase, negative * Nitrite, negative * Significant for greater than 100 red blood cells/HPF * Fewer than 5 white blood cells/HPF * Occasional crystals/HPF Of the following, the MOST likely diagnosis in this patient is ``` A. papillary necrosis B. viral cystitis C. urolithiasis D. renal hematoma E. acute pyelonephritis ```
C. urolithiasis The young child in the vignette presents with symptoms of flank pain, gross hematuria, and questionable dysuria. The most probable cause of the patient’s dysuria is crystalluria associated with urolithiasis. he most common clinical symptoms, like those seen in adults, are renal colic and hematuria. The patient in the vignette lacks pyuria; therefore, disease processes associated with inflammation, such as acute pyelonephritis and acute cystitis (whether viral or bacterial), are unlikely in this patient. The absence of renal trauma makes renal hematoma unlikely. In addition, this patient’s history is not consistent with a disease process capable of presenting with papillary necrosis such as a sickle hemoglobinopathy.