N EPH RO LO GY T EST
J UL Y, 20 13 Question 1:
A 10-yr-old girl (body surface area of 1.0 m 2) with chronic renal insufficiency is i s seen in your clinic and undergoes a 24-hr urine collection for measurement of creatinine clearance. The results are as follows: urine creatinine 144 mg/dL (12672 mcmol/l) ; serum creatinine 1.7 mg/dL (150 mcmol/l); urine volume 700 mL. Based on these measurements, this patient's standard creatinine clearance (mL/min/1.73 m2) is: 35 mL/min/1.73 m2 60 mL/min/1.73 m2 40 mL/min/1.73 m2 70 mL/min/1.73 m2 25 mL/min/1.73 m2
Question 2: A 3-yr-old boy presents to your office with sudden onset of cola-colored urine, progressive facial swelling over the past 3 days, and decreased urine volume over the past day. His examination is notable for blood pressure 130/80 mm Hg, periorbital edema, bibasilar rales, and ankle swelling. His urinalysis is remarkable for 3+ hematuria, 1+ proteinuria, 100 red blood cells per high-power field, and red blood cell casts. His serum electrolytes are normal and the serum albumin is i s 3.2 g/liter. This clinical presentation is most consistent with: Acute renal failure Acute pyelonephritis Nephrotic syndrome Acute glomerulonephritis Chronic renal failure
Question 3: In the case described in Question 2, which of the following laboratory studies would be the most helpful in determining the cause of this patient's hematuria and proteinuria? Complete blood cell count Serum cholesterol determination Antistreptolysin O antibody level 24-hr urine collection for measurement of protein and creatinine clearance 1
Complement C3 and C4,
Question 4: 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, which 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? This disease is more common in females The primary treatment is blood pressure control Progressive kidney disease occurs in a majority of children The complement C3 value is usually low Children with this disease rarely present with grosshematuria
Question 5: A 15-yr-old boy with a 12-yr history of microscopic hematuria is noted to have bilateral highfrequency sensorineural hearing loss, blood pressure of 140/90 mm Hg, serum creatinine of 1.5 mg/dl (132 mcmol/l), and urinary protein of 2,000 mg/24 hr. This patient's mother also has microscopic hematuria. The most likely mode of inheritance for this child's glomerular disease is: Autosomal dominant with incomplete penetrance X-linked dominant Autosomal recessive X-linked recessive Autosomal dominant
Question 6: A 5-yr-old girl presents with cola-colored urine, oliguria, and body edema 2 wk after being treated for group A-hemolytic streptococcal pharyngitis. Her complement C3 is noted to be very low at 15 mg/dL (0.15 g/l). When should this patient's complement C3 level be repeated in order to confirm your suspected diagnosis? In 1 week In 2 weeks In 3 weeks 2
In 4 weeks In 8 weeks
Question 7: The most common clinical presentation for membranous nephropathy in children is: Asymptomatic microscopic hematuria Acute nephritic syndrome Nephrotic syndrome Complete absence of symptoms with normal results on urinalysis Acute renal failure
Question 8: Which of the following statements about membranoproliferative glomerulonephritis in children is true? It occurs most commonly in the first decade of life Hypocomplementemia usually resolves within 2 mo of presentation Alternate-day glucocorticoid therapy may be beneficial in stabilizing the clinical course Progression to end-stage renal disease is rare It is a common cause of nephrotic syndrome in childhood
Question 9: A 14-yr-old girl presents to your clinic with a 1-mo history of fatigue. Over the past week, she has developed low-grade fevers with temperatures to 38 oC, bilateral knee pain, and chest pain with deep inspiration. On examination, she is seen to be a tired-appearing adolescent in no acute distress. Blood pressure is 130/80 mm Hg. Breath sounds are diminished over the right lung base. Cardiac examination findings are normal. Abdominal auscultation/palpation reveals no abnormalities. A urinalysis reveals 3+ hematuria and 3+ proteinuria. Which of the following laboratory studies is the most appropriate next step in confirming this patient's diagnosis? Sedimentation rate Rheumatoid factor Antinuclear antibody
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Anti-neutrophil cytoplasmic antibody Kidney biopsy
Question 10: The most appropriate initial treatment for the patient described in Question 9 is: Oral chorambucil Oral prednisone Intravenous monthly cyclophosphamide infusions Plasmapheresis Conservative management by continued follow-upevaluation in your clinic before initiation of furthertreatment
Question 11: A 3-yr-old boy presents to an urgent care clinic with a 3-day history of abdominal pain and difficulty walking. Abnormal findings include blood pressure of 120/80 mm Hg, diffuse abdominal tenderness, purpuric rash of the hands and ankles, and diffuse periarticular tenderness and swelling of the ankles. The most likely diagnosis is: Systemic lupus erythematosus Kawasaki's disease Juvenile rheumatoid arthritis Henoch-Schoenlein purpura Stevens-Johnson syndrome
Question 12: All of the following glomerular diseases often manifest with rapidly progressive glomerulonephritis except: Wegener's granulomatosis Systemic lupus erythematosus Membranoproliferative glomerulonephritis Goodpasture syndrome 4
Focal segmental glomerulosclerosis
Question 13: A 3-yr-old girl presents to your office with acute onset of lethargy and pallor. The child's mother reports that the child had bloody diarrhea for 5 days that cleared one day prior to presenting to your office. She also notes acute onset of cola-colored urine. On examination, the patient is pale and lethargic. Blood pressure is 120/80 mm Hg. The most appropriate next step in diagnosis would be: Urinalysis X-ray examination of the abdomen Urine culture Complete blood cell count Prothrombin time
Question 14: A 3-yr-old girl develops bloody diarrhea and pallor of acute onset. A stool culture reveals E. coli O157:H7. Laboratory values include Na+ 130 mg/dL, K+ 5.5 mEq/L, Cl-90 mg/dL, total CO2 18 mEq/L, BUN 100 mg/dL (urea 35 mmol/l), and creatinine 4.0 mg/dL (352 mcmol/l). All of the following are accepted treatments for this patient except: Antihypertensive pharmacotherapy to maintain blood pressure below the 90 th percentile for age and height Fluid replacement at rate to cover insensible losses plus urine output Institution of antibiotic treatment against E. coli bacteria Early institution of dialysis Aggressive nutrition
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Question 15: A full-term male newborn is noted to have gross hematuria of acute onset associated with new bilateral abdominal flank masses at 24 hr of life. All of the following may be contributing factors in this clinical scenario except: Dehydration Perinatal asphyxia Hypertension Sepsis Maternal diabetes
Question 16: An 8-yr-old girl presents with dysuria, abdominal pain, and intermittent red urine. A urinalysis reveals specific gravity of 1.020, pH of 6.0, 2+ blood, no protein, and Excess red blood cells per high-power field. A 24-hr urine specimen reveals 8 mg/kg body weight (0.2 mmol/kg) of calcium. Which of the following is an acceptable treatment for this patient's problem? Increased intake of sodium-containing fluids Hydrochlorthiazide Vitamin D supplementation Vitamin C supplementation
Question 17: A full-term newborn male is noted to have bilateral flank masses, hepatomegaly, and blood pressure of 120/80 mm Hg. A renal ultrasound study reveals enlarged hyperechoic kidneys bilaterally and an echogenic liver. Which of the following is correct regarding this patient's diagnosis? Hypertension is uncommon This condition is inherited in an autosomal recessive pattern This condition is associated with pancreatic cysts Cerebrovascular hemorrhage is often seen in these patients Respiratory distress is uncommon in the neonatal period
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Question 18: 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? Evaluate the patient for kidney transplantation Check the blood pressure Obtain a cystogram to evaluate for vesicoureteral reflux Start the patient on an antibiotic for prevention of urinary tract infection Obtain a renal ultrasound study of all siblings
Question 19: 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. All of the following organisms may cause infection leading to these symptoms except: Ureaplasma Chlamydia E. Coli Adenovirus Enterovirus
Question 20: All of the following statements are true except: In humans, formation of nephrons is complete at birth but functional maturation continues during the first decade of life The plasma filtered through the glomerular capillary walls is cell free but contains all the substances in the plasma Fetal kidney function is not necessary for normal intrauterine homeostasis After birth, the glomerular filtration (GF) increases until kidney growth ceases toward the end of the second decade of life Serum creatinine level does not rise above normal until the GF rate falls by 30-40%
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Question 21: All of the following statements are true except: Hematuria is defined as the presence of at least 5 red blood cells (RBCs) in HPF (or 10 RBCs/ul) Urethrorrhagia refers to urethral bleeding in the presence of urine False-negative results on Chemstrip testing may be due to a urine preservative such as formalin Screening urinalysis should be performed at well child visits at 5 yr of age
Question 22: All of the following statements are true except: IgA nephropathy commonly manifests with gross hematuria 1-2 days after the onset of an apparent viral upper respiratory tract infection Gross hematuria does not occur in patients with thin glomerular basement membrane disease Gross hematuria occurs in Alport syndrome The primary treatment of IgA nephropathy is proper blood pressure control The presence of anterior lenticonus is pathognomonic for Alport syndrome
Question 23: All of the following statements regarding poststreptococcal glomerulonephritis (PSGN) are true except: PSGN is common in children 2 to 5 yr of age, The acute phase of PSGN usually resolves in 6-8 wk Microscopic hematuria may persist for 1-2 yr following the initial presentation of PSGN The serum C3 level is usually reduced in the acute phase of PSGN The best single antibody titer to document cutaneous streptococcal infection is the deoxyribonuclease (DNase) B antigen
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Question 24: All of the following statements about hemolytic-uremic syndrome (HUS) are true except: HUS is the most common cause of acute renal failure in young children Verotoxin elaborated by Escherichia coli O157:H7 initiates endothelial cell injury in HUS HUS always presents after an enteritis with diarrhea The diagnosis of HUS requires microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure Mortality from HUS is less than 10
Question 25: All of the following statements regarding autosomal recessive and autosomal dominant polycystic kidney disease (ARPKD and ADPKD) are true except: ARPKD typically presents in the 4th or 5th decade of life ADPKD is a systemic disorder affecting many organ systems The treatment of ARPKD and ADPKD is primarily supportive The presentation of ADPKD in older children has a favorable prognosis In about 85% of patients with ADPKD, the trait maps to the PKD1 gene on the short arm of chromosome 16
Question 26: A 16-yr-old boy with focal segmental glomerulosclerosis has a serum creatinine of 1.9 mg/dL (167 mcmol/l) and a 24-hr urinary protein excretion of 1800 mg. All of the following strategies may theoretically help to slow the progression of chronic renal failure except: Careful control of systemic hypertension Reduction of proteinuria using ACE inhibitor therapy Administration of epidermal growth factor Normalization of serum calcium/phosphorus balance Treatment of metabolic acidosis with NaHCO3
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Question 27: A 12-yr-old boy presents with a long-standing history of polyuria and polydipsia, progressive fatigue, decreased appetite, morning nausea and emesis, weight loss, and impaired growth velocity. In addition, he has had no response to a 6-mo course of iron therapy for treatment of anemia. Initial laboratory evaluation reveals BUN of 125 mg/dL (43 mmol/l) and serum creatinine of 8.7 mg/dL (765 mcmol/l). Other expected laboratory features include all of the following except: Elevated parathyroid hormone level Increased anion gap metabolic acidosis Decreased levels of growth hormone Small, echogenic kidneys on ultrasonography Hypocalcemia
Question 28: A 4-mo-old boy is noted to have poor growth at a routine well child visit. Results of laboratory studies include serum sodium 140 mmol/L, potassium 3.5 mmol/L, chloride 116 mmol/L, and bicarbonate 13 mmol/L. All of the following should be considered in the differential diagnosis except: Distal renal tubular acidosis Chronic diarrhea Proximal renal tubular acidosis Lactic acidosis Renal Fanconi syndrome
Question 29: 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/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: Ingestion of high potassium-containing foods Distal renal tubular acidosis Acute pyelonephritis 10
Congenital adrenal hyperplasia
Question 30: A patient develops renal Fanconi syndrome after receiving ifosfamide for treatment of Wilms tumor. All of the following are features of this condition except: Phosphaturia Metabolic alkalosis Rickets Polyuria Growth retardation
Question 31: 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 (26 mcmol/l). 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: Unilateral renal agenesis Polycystic kidneys Nephrocalcinosis Small echogenic kidneys bilaterally Enlarged kidneys
Question 32: A 1-wk-old full-term male infant presents with irritability and low-grade fever. His parents report that his urine output has been very high, despite a decrease in oral intake. Physical examination reveals a moderately to severely dehydrated infant. Serum sodium is 170 mmol/L. Serum osmolarity is 340 mmol/kg. Urinalysis reveals a specific gravity of 1.000, with no protein, blood, or leukocytes. Urine osmolarity is 240 mmol/kg. The patient is given intravenous fluids. Massive polyuria is noted. Vasopressin is administered, but no change in urine output or urine osmolarity is seen. The genetic defects that cause this congenital condition result in: Inability to respond to aldosterone Inability to produce antidiuretic hormone 11
Overproduction of atrial natriuretic factor Tubular unresponsiveness to antidiuretic hormone Abnormal regulation of osmoreceptors in the hypothalamus
Question 33: A 16-yr-old girl has a 3-yr history of bipolar disorder and seizures. In the last several months she has noted new onset of polyuria and excessive thirst. Her current medications include lithium and valproic acid. Findings on physical examination are unremarkable. All of the following are potential causes of her recent symptoms except: Psychogenic polydipsia Lithium toxicity Central diabetes insipidus Congenital nephrogenic diabetes insipidus Diabetes mellitus
Question 34: A 1-mo-old infant presents with lethargy. Physical examination reveals a mildly to moderately dehydrated infant. Results of laboratory tests include serum sodium 137 mmol/L, potassium 3.2 mmol/L, chloride 90 mmol/L, and bicarbonate 38 mmol/L. All of the following are potential causes of this patient's laboratory findings except: Pyloric stenosis Chronic respiratory insufficiency Chronic diarrhea Bartter syndrome
Question 35: A 13-yr-old girl develops a sore throat and low-grade fever. A throat culture is positive for group A streptococcal infection, for which she is given oral penicillin. Seven days later, she develops a rash and fever. Urine output is normal. Her pulse is 90/min; blood pressure is 110/60 mm Hg. Serum creatinine is 2.4 mg/dL (211 mcmol/l). WBC count is 12,000 per mm3 with 60% neutrophils, 25% lymphocytes, and 15% eosinophils. C3 level is normal. Urinalysis demonstrates specific gravity of 1.010, small amount of blood, no protein, 5-10 WBCs per high-power field, 5-10 RBCs per high-power field, and no RBC casts. The most likely diagnosis is: Acute post streptococcal glomerulonephritis 12
Toxic shock syndrome Acute interstitial nephritis Minimal-change nephrotic syndrome Acute tubular necrosis
Question 36: A 12-yr-old boy presents with a 1-yr history of worsening polyuria and a 2-to 3-wk history of nausea, fatigue, and malaise. Serum creatinine is 4.0 mg/dL (352 mcmol/l); bicarbonate is 15 mg/dL. Urinalysis shows specific gravity of 1.004, trace leukocytes, trace blood, and no protein, with 3-5 WBCs per high-power field, 3-5 RBCs per high-power field, and no RBC casts. Which of the following is the most likely diagnosis? Acute poststreptococcal glomerulonephritis Chronic interstitial nephritis Minimal-change nephrotic syndrome Acute interstitial nephritis
Question 37: A 4-yr-old boy with chronic kidney disease is seen in your clinic. His lab shows a serum creatinine 2.2 mg/dL (194 mcmol/l). His body length is 100 cm. What is the estimated GFR according to Schwartz formula? 25 mL/min/1.73 m2 40 mL/min/1.73 m2 60 mL/min/1.73 m2 70 mL/min/1.73 m2 80 mL/min/1.73 m2
Question 38: A 2-yr-old girl has an acute afebrile diarrheal syndrome characterized by abdominal pain, vomiting, and grossly bloody stools. A stool culture suggests E. coli O157:H7; a fecal toxin electroimmunoassay suggests that a Shiga toxin is present. Appropriate care includes: Loperamide An oral antibiotic (choice based on susceptibility of the Shiga toxin-producing E. coli) 13
A parenteral antibiotic (choice based on susceptibility of the Shiga toxin-producing E. coli) Careful follow-up evaluation for development of thrombocytopenia, anemia, and/or renal failure
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Question 39: Hemolytic-uremic syndrome is typically a complication of infection with E. coli O157:H7 or other Shiga toxin-producing strain of E. coli. The same process, microangiopathic hemolytic anemia with renal failure, can also follow infection with: Salmonella typhi or Campylobacter jejuni Shigella fIexneri Shigella sonnei Shigella dysenteriae serotype 1 Any species or serotype of Shigella
Question 40: Urine dipstick testing of a specimen obtained from a febrile 4yr-old child with acute viral gastroenteritis shows specific gravity 1.030, pH 5.0, 2+ proteinuria, and no blood cells. The most likely cause of the patient's proteinuria is: Transient proteinuria Nephrotic syndrome Orthostatic proteinuria Acute glomerulonephritis Chronic glomerulonephritis
Question 41: A newborn boy is delivered by emergency cesarean section for severe fetal distress and placental abruption. Resuscitation is required in the delivery room. The infant develops gross hematuria and oliguric acute renal failure with a peak serum creatinine level of 8.0 mg/dL (704 mcmol/l) at 8 days of age. The most likely cause of the infant's acute renal failure is: Renal dysplasia Cortical necrosis Obstructive uropathy Acute glomerulonephritis Prerenal azotemia
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