Chapters 500-600

A 1 yr old girl has a history of fever, anemia, severe skin eruption, generalized lymphadenopathy, and pulmonary infiltrates. A skull radiograph shows multiple punched-out lesions. The most likely diagnosis is:

A. Osteosarcoma

B. Osteochondritis

C. Langerhans cell histiocytosis

D. Leukemia

E. Ewing sarcoma

6. An asymptomatic adolescent male is noted to have hematuria on a clean catch urinalysis as part of a routine pre-participation physical for sports. The most appropriate strategy is:

A. Repeat urinalysis in 2 wk

B. Urine culture

C. Spot urine calcium

D. Urinalysis of 1st-degree relatives

E. Renal and bladder ultrasound

8. A 10 yr old boy is noted to have hematuria and proteinuria on a routine physical examination. He is without complaints, and examination findings are normal. Results of blood chemistry studies are also normal, but analysis of the 24-hr urine specimen reveals 2 g of protein and a normal creatinine clearance. A renal biopsy is performed, that reveals mesangial proliferative glomerulonephritis with very bright immunoglobulin A deposits in the mesangium on immunofluorescence. Which of the following statements is true regarding this child's form of glomerulonephritis?

A. This disease is more common in females

B. The primary treatment is blood pressure control

C. Progressive kidney disease occurs in a majority of children

D. The complement C3 value is usually low

E. Children with this disease rarely present with gross hematuria

13. A 12 yr old girl presents with edema and gross hematuria. Her evaluation reveals serum creatinine 1. 4 mg/dL, serum albumin 2. 3 mg/dL, 24-hr urine protein excretion 5. 5 g, and C3 12 mg/dL. The most likely cause of her nephrotic syndrome is:

A. Membranoproliferative glomerulonephritis

B. Minimal-change disease

C. IgA nephropathy

D. Membranous nephropathy

E. Focal segmental glomerulosclerosis

24. An 8 yr old girl presents with dysuria, abdominal pain, and intermittent pink urine. A urinalysis reveals specific gravity of 1. 020, pH of 6. 0, 2+ hematuria, no protein, and 50 red blood cells per high-power field. A 24-hr urine specimen reveals 6 mg/kg body weight of calcium. Which of the following is an acceptable treatment for this patient's problem?

A. Increased intake of sodium-containing fluids

B. Dietary calcium restriction

C. Single daily dose of hydrochlorothiazide

D. Vitamin D supplementation

E. Vitamin C supplementation

26. All of the following statements regarding autosomal recessive and autosomal dominant polycystic kidney disease (ARPKD and ADPKD) are true EXCEPT:

A. ARPKD typically presents in the 4th or 5th decade of life

B. ADPKD is a systemic disorder affecting many organ systems

C. The treatment of ARPKD and ADPKD is primarily supportive

D. The presentation of ADPKD in older children has a favorable prognosis

E. In about 85% of patients with ADPKD, the trait maps to the PKD1 gene on the short arm of chromosome 16

27. A 15 yr old boy is noted to have enlarged kidneys with macrocysts seen bilaterally on a renal ultrasound study after developing gross hematuria while playing hockey. His mother is 40 yr of age and demonstrates similar findings on renal ultrasound examination. What is the most appropriate next step in this patient's management?

A. Evaluate the patient for kidney transplantation

B. Check the blood pressure

C. Obtain a cystogram to evaluate for vesicoureteral reflux

D. Start the patient on an antibiotic for prevention of urinary tract infection

E. Obtain a renal ultrasound study of all siblings

28. A 15 yr old boy is seen in your office for dysuria and is noted to have 4+ hematuria and > 100 red blood cells per high-power field on urinalysis. A urine culture is negative. Which organism may cause this clinical scenario?

A. Epstein-Barr virus

B. Staphylococcus species

C. Adenovirus

D. Coxsackievirus

43. A 3 mo old patient hospitalized for bronchiolitis is noted on admission laboratory studies to have a serum bicarbonate of 14 mmol/L. Of the following, which is the most appropriate next step in the patient's management?

A. Perform renal ultrasonography

B. Perform ammonium chloride loading test

C. Repeat serum electrolyte determination via venipuncture

D. Measure urine electrolytes, with calculation of urine anion gap

E. Initiate treatment with oral bicarbonate supplementation

45. A 9 mo old male infant is noted to have failure to thrive. At birth, his height and weight were at the 25th percentile, but by 9 mo of age, height and weight are at less than the 5th percentile. Laboratory tests reveal a non–anion gap metabolic acidosis, with a serum potassium of 3. 5 mmol/L. Serum creatinine is 0. 3 mg/dL. Urinalysis shows a urine pH of 8. 0, with no abnormalities. A diagnosis of distal renal tubular acidosis is made. Renal ultrasonography is most likely to show:

A. Unilateral renal agenesis

B. Polycystic kidneys

C. Nephrocalcinosis

D. Small echogenic kidneys bilaterally

E. Enlarged kidneys

51. A 17 yr old girl presents with headache, vomiting, and focal seizure. Head CT is negative. You suspect meningoencephalitis. Which of the following conditions will not increase the risk of developing nephrotoxicity from administration of acyclovir?

A. Concomitant treatment with nafcillin and gentamicin

B. Dehydration

C. Concomitant treatment with ketorolac

D. Use of contrast agents for CT

E. Concomitant administration of fosphenytoin

55. A 3 yr old girl with congenital heart disease develops acute renal failure during a prolonged stay in an intensive care unit. Which of the following are potential contributory factors?

A. Use of diuretics for pulmonary edema

B. Nephrotoxic antibiotics

C. Hypotensive episodes

D. Contrast agent used for cardiac catheterization

E. Congestive heart failure

F. All of the above

56. A 16 yr old boy with focal segmental glomerulosclerosis has a serum creatinine of 1. 9 mg/dL and a 24-hr urinary protein excretion of 1800 mg. All of the following strategies are recommended to slow the progression of chronic renal failure EXCEPT:

A. Careful control of systemic hypertension

B. Reduction of proteinuria using ACE inhibitor therapy

C. Administration of erythropoietin

D. Normalization of serum calcium/phosphorus balance

E. Low-protein diet

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

A. Peritoneal dialysis

B. Intermittent hemodialysis

C. Continuous venovenous hemofiltration

D. A or B

63. A 5 yr old boy with end-stage renal disease has a rising serum creatinine of 4. 5 mg/dL. The most appropriate statement regarding patient management is:

A. At least a short period of dialysis is necessary before the possibility of renal transplantation is considered

B. Transplantation offers the best quality of life and survival rate for children with ESRD, as compared with dialysis

C. Kidney transplant has a higher mortality rate than prolonged hemodialysis

D. He is likely not large enough to accommodate an adult kidney allograft

E. Dialysis or transplantation should be considered only when he becomes symptomatic from ESRD (fatigue, decline in school performance, anorexia, and pruritus)

66. The most common abdominal mass in a neonate is:

A. Multicystic dysplastic kidney

B. Wilms tumor

C. Neuroblastoma

D. Meckel diverticulum

E. Ovarian teratoma

68. Clinical pyelonephritis may be diagnosed with a positive urine culture and which of the following:

A. Urgency

B. Flank pain

C. Fever

D. Vomiting

E. Any of B, C, or D

71. A 15 yr old girl is admitted with a 5-day history of worsening fever, left flank pain, and vomiting. Physical examination reveals an ill-appearing, dehydrated adolescent. Pulse rate is 110 beats/min; temperature is 40°C. Left costovertebral angle tenderness is present. Serum sodium is 131 mmol/L, potassium 6. 7 mmol/L, chloride 108 mmol/L, and bicarbonate 15 mmol/L. The most likely diagnosis is:

A. Addison disease

B. Ingestion of high potassium-containing foods

C. Distal renal tubular acidosis

D. Acute pyelonephritis

E. Congenital adrenal hiperplasia

73. A 7 mo old white male infant presents with failure to thrive and a BUN of 75 mg/dL. He has a walnut-sized mass palpable above the pubic symphysis. The most likely diagnosis is:

A. Renal artery stenosis

B. Renal hypoplasia

C. Urogenic bladder

D. Posterior urethral valves

E. Nephrolithiasis

74. A 6 yr old girl has a long history of urinary frequency and urgency. She also has nocturnal enuresis. In addition, she has urge incontinence. A urine culture is negative. The most likely diagnosis is:

A. Overactive bladder

B. Ureteral ectopia

C. Labial adhesion

D. Chronic cystitis

E. Nephrolithiasis

75. A 7 yr old boy has nocturnal enuresis. Which of the following is the most appropriate 1st-line approach to treatment?

A. Motivational chart

B. Moisture sensor alarm

C. Desmopressin

D. Imipramine

E. Oxybutynin

F. Either A or B

80. A 2 wk old infant girl has bilateral swelling of the breasts, with a milky discharge from one nipple. The best course of action is:

A. Perform ultrasonography on the affected breast to rule out abscess

B. Begin antistaphylococcal antibiotics

C. Reassure the parents that the swelling is due to maternal estrogen and should soon subside

D. Obtain blood culture and CBC

E. A, B, and D

81. A 3 wk old infant girl has unilateral swelling of the right breast, with overlying erythema. The best course of action is:

A. Ultrasonography of the chest to rule out abscess

B. Begin antistaphylococcal antibiotics

C. Reassure the parents that the swelling is due to maternal estrogen and should soon subside

D. Obtain blood culture and CBC

E. A, B, and D

82. A 17 yr old Tanner stage 2 girl presents with a history of bilateral spontaneous milky discharge from her breasts for 2 mo. Menarche was at age 12 yr, and her periods had been regular until 4 mo before this visit to your office. In addition, she complains of headache on awakening for the past 2 wk. A urine pregnancy test is negative. What test is indicated?

A. Serum IGF-1

B. Serum prolactin level

C. Serum estrogen level

D. Serum luteinizing hormone level

E. Serum vitamin D level

83. A 16 yr old girl complains of a smaller left breast. On your examination, you note slight asymmetry. Which of the following is the approach for this issue?

A. Reassurance that her examination normal and that most women have some degree of asymmetry

B. Referral to a plastic surgeon for possible left breast augmentation

C. Referral to a plastic surgeon for possible right breast reduction

D. Advise monthly breast self exams

E. A, B, and D

84. You diagnose an adolescent girl with polycystic ovary syndrome (PCOS). This condition has an increased risk of what disease?

A. Metabolic syndrome

B. Infertility

C. Diabetes mellitus

D. Endometrial cancer

E. All of the above

85. A 17 yr old girl with normal weight has irregular menses and acne. Ultrasound of the ovaries shows multiple small cysts. First-line therapy for this condition is which of the following?

A. Metformin

B. Combined estrogen and progesterone oral contraceptive pill

C. Low-fat diet

D. Spironolactone

86. A 17 yr old girl has abdominal pain. A plain film shows a calcification in the right lower quadrant. Ultrasound shows an ovarian mass. The most likely diagnosis is:

A. Mature cystic teratoma (dermoid cyst)

B. Malignant teratoma

C. Choriocarcinoma

D. Embryonal carcinoma

E. Dysgerminoma

87 Vaccination against HPV 16 and 18 is shown to prevent what percent of all cervical cancers?

A. 10%

B. 30%

C. 50%

D. 70%

E. 100%

88 What is the most common ovarian neoplasm in adolescents?

A. Mature cystic teratoma (dermoid cyst)

B. Malignant teratoma

C. Choriocarcinoma

D. Embryonal carcinoma

E. Dysgerminoma

91. A 7 day old male infant presents with a seizure. Serum glucose is 17 mg/dL. Examination is normal with exception of jaundice and microphallus. The most likely diagnosis is:

A. Congenital toxoplasmosis

B. Congenital hepatitis

C. Congenital hypopituitarism

D. Nonketotic hypoglycemia

E. Infantile spasms

92. Constitutional growth delay is characterized by all of the following EXCEPT:

A. Is an inherited trait

B. Length and weight are normal at birth

C. Growth is normal in infancy

D. The pubertal growth spurt is delayed

E. Is usually treated with recombinant growth hormone

114. A 10 yr old boy is under medical management for adrenal insufficiency. His mother reports he has complained that he is not as good at playing sports as previously and has trouble reading. The 1st step in the care of this boy is to:

A. Measure very long-chain fatty acids

B. Measure thyroid hormone levels

C. Increase his replacement dose of hydrocortisone

D. Order an MRI study of his brain and pituitary

E. Refer him to an ophthalmologis

117. A 2 wk old male infant is eating poorly, has repeated emesis, and has not regained his birth weight. On physical examination he appears dehydrated and is noted to have increased pigmentation of skin creases and genitalia. In addition to measuring serum electrolytes, assay of which of the following hormones would be most informative?

A. 17-Hydroxyprogesterone

B. Cortisol

C. 17-Hydroxypregnenolone

D. Renin

E. Aldosterone

118. A 5 yr old boy presents with pubic hair development. He is tall and has increased pigmentation of his genitalia and phallic enlargement. Blood pressure is 130/90 mm Hg. Measurement of which of the following hormones would be most likely to be diagnostic?

A. Testosterone

B. 17-Hydroxyprogesterone

C. 11-Deoxycortisol

D. Aldosterone

E. Deoxycorticosterone

119 A male newborn infant is noted to have increased pigmentation of his skin and genitalia, perineal hypospadias with bifid scrotum, and a 2-cm phallus with chordee. Gonads are palpable in the inguinal canal, and no uterus is visualized on ultrasound examination. The most likely diagnosis is:

A. Incomplete testicular feminization

B. 21-Hydroxylase deficiency

C. 3β -HSD deficiency

D. Lipoid adrenal hyperplasia

E. 11-Hydroxylase deficiency

121.. Carney complex includes which of the following components?

A. Testicular cell tumors

B. Cardiac myxomas

C. Primary pigmented adrenocortical disease

D. Autosomal dominant transmission

E. All of the above

123. Pheochromocytomas may be associated with:

A. Neurofibromatosis

B. Von Hippel-Lindau disease

C. MEN IIA and MEN IIB

D. Sturge-Weber syndrome

E. All of the above

124. Presenting manifestations of pheochromocytoma may include:

A. Sustained hypertension

B. Abdominal pain

C. Polyuria and polydipsia

D. Weight loss

E. All of the above

137. A 10 yr old girl with epilepsy is started on valproic acid at 1 5mg/kg/day (dose range 20-40 mg/kg/day). She continues to have regular seizures. She has no significant side effects from the medication. The appropriate next step in management is:

A. Start lamotrigine in addition to valproic acid

B. Stop valproic acid and replace with phenytoin

C. Start lamotrigine in addition to carbamazepine

D. Increase the dose of valproic acid

142. A 5 yr old girl is evaluated for progressive difficulty in walking, which seems to worsen during the day; her walking is much better after a good night's sleep. Examination during the afternoon shows that she has rigidity in the leg muscles and dystonic twisting of her feet. This kind of movement disorder often responds dramatically to which of the following medications?

A. Sodium valproate

B. Lorazepam

C. Levodopa

D. Gabapentin

E. Lithium

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

A. Nemaline rod myopathy

B. X-linked myotubular myopathy

C. Duchenne muscular dystrophy

D. Myotonic dystrophy, neonatal form

E. Congenital muscular dystrophy with merosin deficiency

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