(B) CORRECT. This is the definition of nephrotic syndrome.

MCD produces a
nephrotic syndrome, with significant albuminuria. A single urine specimen will
not suffice for the definition of nephrotic syndrome (though it could be
extrapolated, given the daunting task of 24 hour urine collection in children).

Question 7

A clinical study is performed with pediatric subjects who had a diagnosis of

minimal change disease. These patients were observed to have prominent
periorbital edema at diagnosis. Laboratory test findings from serum and urine
tests were analyzed. Which of the following urinalysis test findings is most
likely to have been consistently present in these subjects?.

A Nitrite positive

B Proteinuria >3.5 gm/24 hours

C Hematuria with >10 RBC/hpf

D Calcium oxalate crystsls

E Renal tubular epithelial cells and casts

(C) CORRECT. Hereditary nephritis (Alport syndrome) is not associated with

immune complexes, but with a genetic defect of type IV collagen production.
In most families, it is inherited in an X-linked dominant pattern. Symptoms
usually appear at ages 5 to 20, with overt renal failure between ages 20 to 50.

Question 8

A 12-year-old boy is a member of a family with a history of renal disease, with

males more severely affected than females. He is found to have auditory
nerve deafness, corneal dystrophy, and ocular lens dislocation. A urinalysis
shows microscopic hematuria. A renal biopsy is performed. Microscopically,
the glomeruli show glomerular capillaries with irregular basement membrane
thickening and attenuation with splitting of the lamina densa. The mesangial
matrix is increased and epithelial cells may appear foamy. Which of the
following is the most likely diagnosis?

A Goodpasture syndrome
B IgA nephropathy

C Alport syndrome

D Dominant polycystic kidney disease

E Diabetes mellitus, type I

D) CORRECT. Renal concentrating ability is reflected by the specific gravity.

In a person with normal concentrating capacity, less water intake is reflected
by an increased specific gravity.

Question 9

A clinical study is performed of laboratory findings in subjects with renal

diseases. Loss of physiologic function accompanies many diseases. One
renal physiologic function affects thirst. Loss of which of the following renal
functions is most likely to be identified by laboratory measurement of the urine
specific gravity following water deprivation?

A Filtration

B Reabsorption

C Secretion

D Concentration

E Blood flow

(E) CORRECT. She has findings of hyperplastic arteriolosclerosis with

hypertensive emergency ('malignant' hypertension) when systolic pressure is
≥180 and/or diastolic pressure ≥120 mmHg along with signs of acute or
ongoing end-organ damage. Renal disease is likely to complicate diffuse
scleroderma, but not the more limited form of scleroderma sometimes called
'CREST' syndrome.

Question 10
A 45-year-old woman has had increasing malaise for the past year. On
physical examination her blood pressure is 265/150 mm Hg. Laboratory
studies show a plasma renin activity of 9 ng/mL/hr. She then suffers a 'stroke'
with a right basal ganglia hemorrhage and dies. At autopsy the kidneys are
bilaterally small with granular surfaces. Microscopically they show hyperplastic
arteriolosclerosis with fibrinoid necrosis, petechial hemorrhages, and
microinfarcts in the cortices. Which of the following conditions is most likely to
be her underlying cause of death?

A Diabetes mellitus, type II

B Fibromuscular dysplasia

C Factor V Leiden mutation

D Analgesic abuse

E Systemic sclerosis

(B) CORRECT. This is minimal change disease, the most common cause for
nephrotic syndrome in children, and fusion of podocyte foot processes is the
only pathologic finding present (on electron microscopy). Most patients
respond to corticosteroid therapy.

Question 12

A 5-year-old boy is noted to have increased puffiness around his eyes for the
past week, and he has been less active than normal. On physical examination
he has periorbital edema. Vital signs include T 37°C, P 75/minute, RR
22/minute, and BP 140/90 mm Hg. A urinalysis reveals sp. gr. 1.010, pH 6.5,
no glucose, 4+ protein, no blood, no casts, and no ketones. Microscopic
urinalysis reveals oval fat bodies, but no WBC's or RBC's. He improves
following a course of corticosteroid therapy. Which of the following renal
lesions is most likely to have been present in this boy?

A Glomerular crescent formation

B Podocyte foot process effacement

C Patchy acute tubular necrosis

D Hyperplastic arteriolosclerosis

E Mesangial immune complex deposition

(D) CORRECT. Proteinuria may be present with nephritic syndromes

(characterized by RBCs spilled into the urine from the glomeruli), but it is
usually not marked, as in nephrotic syndrome. Membranous nephropathy
typically produces nephrotic syndrome that is predominantly albuminuria.
Diseases with crescents, such as membranoproliferative glomerulonephritis,
lead to nephritic syndrome.

Question 13

A clinical study is performed involving subjects with glomerulonephritis. One

group of subjects has a diagnosis of crescentic glomerulonephritis and
another group has membranous nephropathy. Which one of the following
laboratory findings is most likely to be found in the absence of other findings in
subjects with membranous nephropathy?

A Rapid onset

B Red blood cell casts

C Oliguria

D Albuminuria

E Hypertension

(A) CORRECT. He has findings of ischemic acute tubular necrosis (ATN) from
heart failure with hypotension. A clue is the >20:1 ratio of urea nitrogen to
creatinine, which occurs early in the course, from prerenal azotemia. As the
disease progresses, the ratio begins to approach 10:1, typical for intrinsic
renal diseases. ATN may also be produced by toxins such as ethylene glycol
in antifreeze, but the tubular necrosis is more diffuse.

Question 14
A 51-year-old man is hospitalized for acute myocardial infarction. He has
decreased cardiac output with hypotension requiring multiple pressor agents.
His urine output drops over the next 3 days. His serum urea nitrogen
increases to 59 mg/dL, with creatinine of 2.9 mg/dL. Urinalysis reveals no
protein or glucose, a trace blood, and numerous hyaline casts. Five days later,
he develops polyuria and his serum urea nitrogen declines. Which of the
following pathologic findings in his kidneys is most likely to have caused his
azotemia?

A Patchy tubular necrosis

B Podocyte foot process effacement

C Glomerular crescent formation

D Hyperplastic arteriolosclerosis

E Mesangial immune complex deposition

(E) CORRECT. Therapy may depend upon determination of the severity and
nature of the renal disease with SLE.

Question 15

A clinical study is performed to determine the value of percutaneous renal

biopsy. The medical records of subjects with renal diseases are analyzed to
note the circumstances in which the results of a renal biopsy facilitated choice
of therapy that improved prognosis. In which of the following situations is a
percutaneous needle biopsy of the kidney most useful?

A Fever and flank pain with suspected acute pyelonephritis

B Prostatic hyperplasia with suspected hydronephrosis

C Premature neonate with suspected polycystic kidney disease

D Abdominal pain with suspected renal cyst

E Acute renal failure with suspected systemic lupus erythematosus

(C) CORRECT. Hypertension leads to cardiac enlargement, then dilation, and
eventual heart failure. This is the most common outcome with untreated
hypertension.

Question 17

A 43-year-old man goes to his physician for a routine check of his health
status. He is found to have a blood pressure of 150/95 mm Hg. His urinalysis
shows pH 6.5, specific gravity 1.015, no glucose, blood, or protein, and no
casts. His serum creatinine is 1.4 mg/dL. If he is not treated, which of the
following conditions will most likely cause his death?

A Intracerebral hemorrhage (stroke)

B Aortic aneurysm rupture

C Congestive heart failure

D Chronic renal failure

E Intracranial aneurysm rupture

(A) CORRECT. Crescents are characteristic for a rapidly progressive

glomerulonephritis (RPGN); they form when there is leakage of fibrinogen into
Bowman's space, with proliferation of epithelial cells to form the crescent.
RPGN is a form of nephritic syndrome, with RBCs and RBC casts in the urine
due to severe glomerular damage.

Question 18

A 20-year-old previously healthy man has been feeling tired for the past 5
days. He then passes dark-colored urine. On physical examination his blood
pressure is 160/90 mm Hg. Laboratory studies show his serum creatinine is
4.4 mg/dL and BUN 40 mg/dL. A urinalysis reveals pH 6, specific gravity
1.011, 3+ blood, 1+ protein, no glucose, and no ketones. On urine
microscopic examination there are numerous RBC casts. Which of the
following pathologic findings on renal biopsy is most likely to be present in this
man?

A Glomerular crescents
B Widened proximal tubules

C Neutrophilic infiltrates

D Basememt membrane thickening

E IgA deposited in glomerular capillaries

(A) CORRECT. Membranous nephropathy is the most common cause for

nephrotic syndrome in adults. Some cases are associated with underlying
infections or malignancies, but in most cases the cause is unknown. There is
diffuse thickening of the glomerular capillary basement membrane from
immune deposits.

Question 19

A 43-year-old man has had increasing malaise for the past 3 weeks. On
physical examination he has a blood pressure of 150/95 mm Hg and 1+ pitting
edema of the lower extremities to the knees. Dipstick urinalysis shows no
glucose, blood, ketones, nitrite, or urobilinogen, and the microscopic urinalysis
reveals no RBC/hpf and only 1 WBC/hpf. Additional laboratory studies show a
24 hour urine protein of 4.1 gm. His serum creatinine is 3.5 mg/dL with urea
nitrogen of 38 mg/dL. His hepatitis B surface antigen is positive. Which of the
following is the most likely diagnosis?

A Membranous nephropathy

B Systemic lupus erythematosus

C Acute tubular necrosis

D Diabetic nephropathy

E Post-streptococcal glomerulonephritis

(B) CORRECT. Ischemia, typically in hypotensive hospitalized patients, is the

most frequent antecedent to acute tubular necrosis (ATN), and ischemic
heasrt disease with coronary syndromes including MI's are common. This is a
pre-renal form of azotemia, and note the BUN:Cr ratio more than 20:1.
Question 20

A 60-year-old woman is admitted with sudden onset of chest pain and is

diagnosed with an acute myocardial infarction. There is difficulty maintaining
adequate blood pressure and tissue perfusion for 3 days. Her serum lactate
becomes elevated. Her serum urea nitrogen increases to 44 mg/dL and
creatinine to 2.2 mg/dL. Granular and hyaline casts are present on
microscopic urinalysis. Which of the following renal lesions is most likely to be
present in this situation?

A Chronic pyelonephritis

B Acute tubular necrosis

C Nodular glomerulosclerosis

D Renal vein thrombosis

E Minimal change disease

(A) CORRECT. Nodular glomerulosclerosis is a typical complication of long-

standing diabetes mellitus. Microalbuminuria may preceed development of
other abnormalities.

Question 21

A 50-year-old man was diagnosed at age 15 with type 1 diabetes mellitus. His
disease has been poorly controlled, as evidenced by elevated hemoglobin
A1C levels. He develops a non-healing ulcer of his foot at age 35. At age 45,
he has an increasing serum urea nitrogen and a urinalysis shows sp gr 1.012,
pH 6.5, 1+ protein, no blood, 1+ glucose, negative leukocyte esterase,
negative nitrite, and no ketones. Which of the following renal diseases is he
most likely to have?

A Nodular glomerulosclerosis

B Hyperplastic arteriolosclerosis

C Papillary necrosis
D Crescentic glomerulonephritis

E Pyelonephritis

(D) CORRECT. The WBC casts are most characteristic for an acute
interstitital nephritis (acute pyelonephritis). The casts can only come from
distal tubules / collecting ducts. The positive leukocyte esterase and nitrite are
typical for acute inflammation with bacterial infection of the urinary tract.

Question 23

A 70-year-old woman has had a fever for the past 3 days. She has burning
dysuria. On physical examination her temperature is 37.8°C and there is dull
pain on palpation of her left lower back. Laboratory studies show Hgb 13.3
g/dL, Hct 40.2%, and WBC count 12,300/microliter with differential count 72
segs, 9 bands, 13 lymphs, 5 monos, and 1 eosinophil. A urine dipstick
analysis shows sp gr. 1.017, pH 6, leukocyte esterase positive, nitrite positive,
protein negative, glucose negative, and blood negative. Which of the following
microscopic urinalysis findings would be most diagnostic for her renal
disease?

A Dysmorphic red blood cells

B Oval fat bodies

C Renal tubular epithelial cells

D White blood cell casts

E Triple phosphate crystals

(A) CORRECT. She has analgesic abuse nephropathy which leads to

papillary necrosis and chronic interstitial nephritis with tubular atrophy (though
the renal columns are spared). There is also risk for development of urothelial
carcinoma. The analgesics implicated may include combinations of
phenacetin, acetaminophen, and aspirin.

Question 24
A 53-year-old woman has had chronic arthritis pain for the past 3 years. She
has taken 2 gm of phenacetin and acetaminophen a day for her pain over that
time. She now has increasing fatigue. There are no abnormal findings on
physical examination. Laboratory studies show her serum urea nitrogen is 52
mg/dL and creatinine 5.4 mg/dL. Which of the following pathologic findings is
most likely to occur in her kidneys?

A Papillary necrosis

B Focal segmental glomerulosclerosis

C Nephrocalcinosis

D Acute interstitial nephritis

E Arteriolosclerosis

(C) CORRECT. This allergic response can occur following drug therapy with
such antibiotic agents as methicillin, as well as some diuretics and NSAIDs.
This allergic response with acute interstitial nephritis may be unrelated to the
amount of drug and duration of therapy. This condition is treated by stopping
the drug.

Question 25

A 25-year-old woman has been hospitalized for treatment of a Staphylococcus

aureus abscess of her left thigh complicating a puncture wound. The wound is
incised and drained and she receives antibiotic therapy. She is improving and
discharged home a week later, but the next day she develops a fever. On
physical examination her temperature is 38.1°C and there is a diffuse
erythematous skin rash of her trunk and extremities. A urinalysis shows sp gr
1.020, pH 6.5, 1+ blood, 1+ protein, no glucose, and no ketones. There are
10-20 WBCs/hpf and 1-5 RBCs/hpf, and a few eosinophils are noted on urine
microscopic examination. Which of the following is the most likely diagnosis?

A Acute tubular necrosis

B Septicemia with pyelonephritis

C Drug-induced interstitial nephritis

D Hemolytic-uremic syndrome

E Post-infectious glomerulonephritis

F Urinary tract infection

(A) CORRECT. The findings point to focal segmental glomerulosclerosis

(FSGS), which leads to chronic renal failure in half of cases. The lack of
resolution with corticosteroid therapy and the progression to chronic renal
failure is what sets FSGS apart from minimal change disease. FSGS is
idiopathic, so there is unlikely to be an underying condition.

Question 27

A 15-year-old girl has had increasing lethargy following a bout of the 'flu' 3
weeks ago. On physical examination there are no abnormal findings. Her
condition does not improve after 3 weeks on corticosteroid therapy, so a renal
biopsy is performed and microscopic examination shows segmental sclerosis
of 3 of 10 glomeruli. Immunofluorescence studies and electron microscopy do
not show immune deposits. Which of the following is the most likely outcome
for this girl's condition?

A Progression to chronic renal failure

B Improvement with additional corticosteroid therapy

C Development of restrictive lung disease

D Discovery of an underlying malignancy

E Remission following dietary change

(A) CORRECT. Most cases of membranous nephropathy are idiopathic, but in

some patients there is a history of an infection or a malignancy (usually lung)
with antigenemia that drives the immune deposition in the glomerular capillary
basement membranes. Membranous nephropathy produces primarily
proteinuria. Note the BUN:Cr ratio here close to 10:1 that suggests intrinsic
renal disease.

Question 30

A 60-year-old man was diagnosed last year with adenocarcinoma of the lung,
and he underwent right lower lobectomy. For the past 3 weeks he has had
increasing malaise. On physical examination he has pitting edema to his
knees and presacral edema. Abdominal and chest CT scans show scattered
hepatic mass lesions and hilar lymphadenopathy. A urinalysis reveals 4+
proteinuria, and his 24 hour urine protein is 2.7 gm. His serum urea nitrogen is
55 mg/dL with creatinine of 6.1 mg/dL. A renal biopsy is performed, and there
is focal deposition of IgG and C3 with a granular pattern. Which of the
following forms of glomerular disease is he most likely to have?

A Membranous nephropathy

B Rapidly progressive glomerulonephritis

C Nodular glomerulosclerosis

D Chronic glomerulonephritis

E Dense deposit disease

(B) CORRECT. These findings are typical for a drug-induced acute interstitial
nephritis. The eosinophilia is seen with allergic phenomena (as in a drug
allergy).

Question 35

A 49-year-old woman has been hospitalized for the past 10 days for treatment
of bronchopneumonia. She has developed chills and fever over the past 2
days. On physical examination her temperature is 38.8°C and she has a
diffuse erythematous skin rash. Laboratory studies show serum creatinine 2.2
mg/dL and glucose 73 mg/dL. A peripheral blood smear reveals eosinophilia.
On urinalysis she has 2+ proteinuria but no blood, glucose, or ketones. Which
of the following is the most likely diagnosis?

A Post-streptococcal glomerulonephritis
B Drug-induced interstitial nephritis

C IgA nephropathy

D Acute tubular necrosis

E Acute serum sickness

(E) CORRECT. Immunologic diseases such as systemic lupus erythematosus

may involve the kidney and may produce glomerulonephritis, and the renal
damage often leads to hypertension. Pharmacologic treatment is aimed at
stopping or masking the immunologic process.

Question 36

A clinical study is performed with subjects diagnosed with hypertension who

underwent an extensive workup to determine possible treatable causes for
their hypertension. It is observed that some causes for hypertension are
surgically correctable, while other causes are amenable to pharmacologic
therapy. Laboratory findings in the subjects are analyzed. Which of the
following laboratory test findings is most likely to be present in subjects with
hypertension treated by drugs, rather than by surgery?

A Hyperaldosteronemia

B Hyperreninemia

C Increased catecholamines

D Hypercalcemia

E Autoantibodies

(D) CORRECT. Classic polyarteritis nodosa (PAN) often affects multiple

organs, not just the kidney. The classic form of polyarteritis affects medium to
small sized arteries.

Question 37
A 30-year-old man has had increasing malaise with fever, abdominal pain,
and weight loss of 3 kg over the past 3 weeks. On physical examination his
blood pressure is 165/110 mm Hg. He has a stool positive for occult blood. A
urinalysis reveals hematuria but no proteinuria or glucosuria. He has no serum
anti-neutrophil cytoplasmic autoantibodies and his antinuclear antibody test is
negative. Aneurysmal arterial dilations and occlusions are seen in the medium
sized renal and mesenteric arteries with angiography. He improves with
corticosteroid therapy. Which of the following is the most likely diagnosis?

A Benign nephrosclerosis

B Fibromuscular dysplasia

C Nodular glomerulosclerosis

D Classic polyarteritis nodosa

E Systemic lupus erythematosus

F Thrombotic microangiopathy

G ANCA-associated granulomatous vasculitis

(C) CORRECT. Oval fat bodies appear in association with pronounced

proteinuria and lipiduria. These bodies represent degenerating, sloughed
tubular cells that are filled with lipid. The tubular cells try to reabsorb the
spilled protein and lipid and get overwhelmed.

Question 40

A 30-year-old man has noted puffiness around his eyes and swelling of his
feet for the past 2 weeks. On physical examination his blood pressure is
155/95 mm Hg. Urine microscopic examination reveals oval fat bodies. Which
of the following conditions is he most likely to have?

A Ascending pyelonephritis

B Nephritic syndrome
C Nephrotic syndrome

D Obstructive uropathy

E Renal infarction

F Papillary necrosis

(A) CORRECT. This is the typical (and only) pathologic finding for minimal
change disease, the most common cause for nephrotic syndrome in children,
but it can be seen at older ages, too.

Question 43

A 5-year-old child has been noted by his mother to be lethargic for 2 weeks.
On physical examination he has periorbital edema. He is afebrile. Dipstick
urinalysis reveals no glucose, ketones, or blood, but he has 4+ proteinuria
present. Microscopic urinalysis reveals no casts, but oval fat bodies are seen.
He is treated with corticosteroid therapy and his condition improves. Which of
the following renal electron micrographic findings is most characteristic for this
child's disease?

A Fusion of podocyte foot processes

B Subepithelial electron dense deposits

C Duplication of glomerular capillary basement membranes

D Irregular thickening of the glomerular basement membranes

E Mesangial cell proliferation

(E) CORRECT. This hypercellular glomerulus has many neutrophils,

characteristic for a post-infectious glomerulonephritis, for which a
nephritogenic strain of group A Streptococcus is a likely etiology. Other
serologic test findings could include elevated hyaluronidase and elevated anti-
DNase. Use of all three increases the sensitivity of detection.

Question 45
An 11-year-old girl has increasing lethargy and has passed dark-coloured
urine for the past week. She had a sore throat two weeks ago. On physical
examination she is afebrile with blood pressure 140/90 mm Hg. Laboratory
studies show her serum creatinine is 2.8 mg/dL and urea nitrogen 24 mg/dL.
Urinalysis shows 2+ blood, 2+ protein, no glucose, and no ketones.
Microscopic urinalysis shows dysmorphic RBC's. A renal biopsy is performed
and on microscopic examination shows glomerular hypercellularity, with
PMNs present. Electron microscopy shows subepithelial electron dense
'humps'. Which of the following laboratory test findings is most likely to be
present in this girl?

A Elevated serum glucose

B Antibody to double stranded DNA

C Antiglomerular basement membrane antibody

D Positive C3 nephritogenic factor

E Elevated antistreptolysin O titer