Q1
A
12-year-old girl is admitted to the hospital with left flank pain and weight
loss over a 2-month period. She has no history of fever, vomiting, or diarrhea.
Urinalysis is negative. Serum electrolytes are: sodium, 132 mEq/L (132 mmol/L);
potassium, 2.6 mEq/L (2.6 mmol/L); chloride, 92 mEq/L (92 mmol/L); bicarbonate,
30 mEq/L (30 mmol/L); creatinine, 0.7 mg/dL (61.9 mcmol/L); and blood urea
nitrogen, 15 mg/dL (5.4 mmol/L of urea). She admits to taking her grandmother's
diuretics to lose weight.
Of
the following, the MOST likely cause of the flank pain is:
A. acute
pyelonephritis
B. autosomal
dominant polycystic kidney disease
C. renal
abscess
D. simple
renal cyst
E. ureteropelvic
junction obstruction
Answer
E
Answer
E
Patients
who have UPJ obstruction may be asymptomatic or they may experience intermittent
abdominal or flank pain, abdominal fullness, gross hematuria, and dysuria due
to infection. Occasionally, mild or moderate UPJ obstruction may be undetected
until there is trauma to the kidney or an abnormal flow of urine into the
obstructed area. Such insults can occur after copious intake of fluids or the
use of diuretics, as seen in the girl described in the vignette.
Furosemide
is a loop diuretic that induces an increase in renal excretion of salt and
water, inhibiting the Na-K-2Cl cotransporter in the thick ascending limb of
Henle loop. Patients who are taking chronic diuretic therapy or abusing
diuretics, such as the girl described in the vignette, typically exhibit
hypokalemia and alkalosis on routine electrolyte evaluation. Patients who have
acute pyelonephritis usually experience flank pain, but the urinalysis reveals
white blood cells, nitrites, and bacteria.
Autosomal dominant polycystic kidney
disease (ADPKD) generally is seen in adults and is manifested by hematuria,
urinary tract infection, abdominal pain, and hypertension. Findings on
urinalysis in patients who have early ADPKD frequently are unremarkable.
Electrolyte analysis eventually may reveal azotemia, but hypokalemia and
alkalosis are not seen. A patient who has a renal abscess will experience
abdominal or flank pain, but also will develop a fever.
Furthermore, findings
on the urinalysis are similar to those seen in acute pyelonephritis. Simple
renal cysts are not uncommon and may, if large, cause abdominal discomfort, but
results of electrolyte analysis usually are normal in most patients
------------------------------
Q 2
Answer:
C
-----
Q 3
Answer
C
Nephrotic syndrome (NS) is characterized by proteinuria, hypoalbuminemia, edema, and hypercholesterolemia.
--------------
------------------------------
Q 2
Of
the following, the patient MOST likely to develop chronic renal failure is a:
A. 3-year-old
boy who has minimal-change nephrotic syndrome
B. 3-year-old
girl who has unilateral grade II vesicoureteral reflux
C. 4-year-old
boy who has a history of Henoch-Schönlein purpura and persistent nephrotic syndrome
D. 9-year-old
boy who has unilateral multicystic kidney dysplasia
E. 10-year-old girl who has immunoglobulin A
nephropathy and persistent microscopic Answer:
C
-----
Q 3
In
addition to vascular thrombosis, the MOST likely complications observed in
children who have minimal-change nephrotic syndrome are:
A. acute
renal failure
B. chronic
renal failure
C. hypercholesterolemia
D. hypernatremia
E. hyponatremia
Answer
C
Nephrotic syndrome (NS) is characterized by proteinuria, hypoalbuminemia, edema, and hypercholesterolemia.
--------------
A
child presents at age 6 months with failure to thrive and rickets. Urinalysis
shows 1+ protein and glucosuria. Your differential diagnosis includes nephropathic
cystinosis.
Of
the following, the test that would BEST confirm the diagnosis is:
A. leukocyte
cystine content
B. liver
biopsy
C. ophthalmologic
examination revealing cystine deposits
D. serum
level of cystine
E. urinary
excretion of cystine
Answer
A
The diagnosis of cystinosis depends upon recognition of the constellation of symptoms such as polyuria and polydipsia, anorexia, failure to thrive, dehydration, and hyperchloremic metabolic acidosis. Renal wasting of bicarbonate and phosphate and the presence of glucosuria should alert the physician to the likelihood of Fanconi syndrome. The gold standard for diagnosis of cystinosis is the demonstration of cystine accumulation in the tissues and in leukocytes. Liver biopsy may reveal cystine deposits in some but not all patients. A prenatal diagnosis can be made by measuring cystine content in amniotic cells. The finding of needle-shaped opacities in the cornea by slitlamp examination confirms the diagnosis. Serum levels of cystine are generally normal in nephropathic cystinosis. Urinary excretion of cystine may be elevated, but this finding is not specific for the condition.
--------------------------------
Q 4:
Answer
A
The diagnosis of cystinosis depends upon recognition of the constellation of symptoms such as polyuria and polydipsia, anorexia, failure to thrive, dehydration, and hyperchloremic metabolic acidosis. Renal wasting of bicarbonate and phosphate and the presence of glucosuria should alert the physician to the likelihood of Fanconi syndrome. The gold standard for diagnosis of cystinosis is the demonstration of cystine accumulation in the tissues and in leukocytes. Liver biopsy may reveal cystine deposits in some but not all patients. A prenatal diagnosis can be made by measuring cystine content in amniotic cells. The finding of needle-shaped opacities in the cornea by slitlamp examination confirms the diagnosis. Serum levels of cystine are generally normal in nephropathic cystinosis. Urinary excretion of cystine may be elevated, but this finding is not specific for the condition.
--------------------------------
Q 4:
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 at higher
than usual risk for dehydration in the setting of gastroenteritis
C.
this infant is likely to require dialysis by adolescence
D.
this infant will require annual ultrasonography to screen for renal
malignancy
E. toilet
training may be more challenging in the future for this infant
Answer
A
Answer
A
The
vignette describes a 1-month-old infant with a single kidney and a serum
creatinine of 0.5 mg/dL. This infant has unilateral renal agenesis, which has
an estimated incidence of 1 in 2,900 newborns. Infants with unilateral renal
agenesis have 50% of the expected nephron number. Because of a compensatory
increase in the single nephron GFR (SNGFR), the serum creatinine should
continue to fall to normal levels for the child’s age over the first 1 to 2
years of life until reaching an expected level of 0.3 mg/dL. Moreover, this
single kidney is fully functional and has normal urine concentrating abilities;
therefore, an infant with gastroenteritis would not be expected to be at high
risk of dehydration in this setting. It is unusual for the doubling of the
SNGFR to result in a hyperfiltration injury to the nephron mass and result in
proteinuria. As the single kidney should be structurally and functionally
normal, screening for hypertension should be done beginning at age 3 years
during health maintenance visits. Periodic urinalysis also should be considered
to screen for the development of proteinuria. 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.
Also, newborns with renal agenesis have an approximately 30% risk of
vesicoureteral reflux (VUR) into their kidneys; therefore, performance of a
voiding cystourethrogram to evaluate for VUR is recommended in these patients.
-------------------
Q 5:
-------------------
Q 5:
A
5-year-old otherwise healthy girl, who was fully toilet
trained at age 3, begins to wet her bed. She complains of some dysuria, but has
normal findings on physical examination Of
the following, the MOST likely etiology for this child's enuresis is:
A. constipation
with rectal distension
B. diabetes
mellitus
C. nocturnal
enuresis
D. sickle
cell trait
E. urinary
tract infection
Answer
E
Answer
E
Children
who have inflamed bladders due to infection (cystitis) often present with
urinary frequency. They also may have urgency, dysuria, and hematuria. Some
children develop secondary enuresis, as described for the child in the
vignette. Findings on physical examination of children who have cystitis are
normal, with the possible exception of suprapubic tenderness. The urinalysis
may reveal blood and protein, positive leukocyte esterase, and positive
nitrates. Microscopic examination may reveal white blood cells and bacteria.
Cystitis is diagnosed when a urine culture reveals greater than 100,000 cfu/mL
of a single organism. In infants and young children, sterile catheterization or
suprapubic aspiration is preferable for the collection of urine for culture.
Primary
enuresis refers to involuntary discharge of urine when continence never has
been achieved; secondary enuresis is incontinence that develops after at least
1 year of continence. Enuresis may be nocturnal or diurnal. Approximately 10%
to 15% of 5-year-old children and 1% of adults have nocturnal enuresis that
occurs at least once a month. When findings on physical examination and
urinalysis are normal, further testing usually is not helpful in determining an
etiology.
Severe
constipation with rectal distension can cause enuresis, but it generally is
associated with marked abdominal distension. Polyuria, polydipsia, and
polyphagia are expected in a child who has diabetes mellitus. Enuresis has been
associated with disorders marked by renal concentrating defects, such as sickle
cell disease or trait. However, children who have enuresis due to sickle cell
trait are asymptomatic and do not have dysuria.
------------
Q 6
You are evaluating a 10-year-old girl for a health supervision visit. Her weight and height are at
the 50th percentile for age, her blood pressure is 108/64 mm Hg, and there are no unusual
findings on physical examination. A screening urinalysis shows a specific gravity of 1.030, pH of
6.5, 2+ blood, and no protein. Urine microscopy reveals 5 to 10 red blood cells/high-power field.
Of the following, the MOST appropriate next step is
A. abdominal computed tomography scan
B. antinuclear antibody and complement measurement
C. blood urea nitrogen and creatinine measurements
D. referral for cystoscopy
E. repeat urinalysis in 2 weeks
Answer:
E
The child who has asymptomatic, isolated microscopic hematuria is seen frequently in the
ambulatory setting. Results of a urine dipstick test in patients who have hematuria are positive
for blood, indicating the presence of hemoglobin or myoglobin. A microscopic evaluation that
reveals more than 5 red blood cells/high-power field, as described for the girl in the vignette,
confirms the presence of hematuria. Because isolated microscopic hematuria has been found in
4% of children on at least one of four tested samples, evaluation is not recommended unless
hematuria is present on at least two of three urine samples. Accordingly, the girl in the vignette
should undergo repeat urinalysis in 2 weeks.
------
Q 7:
You are seeing an 8-month-old infant for a health supervision visit. He was born at 28 weeks’
gestation, weighed 1,200 g at birth, and has bronchopulmonary dysplasia. His only medication is
furosemide, which he has been receiving for several months.
Of the following, the MOST likely expected abnormality in this infant is
A. hypercalciuria
B. hypermagnesemia
C. hypocalcemia
D. hyponatremic dehydration
E. metabolic acidosis
Answer
A
Useful information about Furosemide
Furosemide is a loop diuretic that acts at the ascending limb of the loop of Henle (LOH).
Normally, approximately 20% to 25% of filtered sodium is reclaimed at this site, so blockade of
sodium reabsorption results in a brisk diuresis, with urinary losses of sodium, potassium, and
chloride. The blocked transporter is the Na+-K+-2Cl- channel.
In addition to direct effects on the electrolytes of this channel, loop diuretics influence
calcium and magnesium transport. Calcium and magnesium are reabsorbed passively in the
ascending limb of the LOH through paracellular transport as a result a gradient derived from
normal sodium chloride transport. The passive reabsorption of calcium is believed to occur
through a paracellular pathway that is facilitated by paracellin-1, a tight junction protein.
Interruption of sodium chloride reabsorption results in impaired calcium reabsorption and
consequent hypercalciuria and increased risk for nephrocalcinosis and kidney stones.
Furosemide also has an effect on the composition of endolymph in the inner ear because
the Na+-K+-2Cl- transporter that is in the ascending limb of the LOH also can be found in the
marginal cells of the cochlear duct of the inner ear. Furosemide induces changes in ion transport
in these cells, reducing endocochlear potentials in the cells, which results in hearing loss.
Ototoxicity is more likely when loop diuretics are used in high doses or are used in conjunction
with other ototoxic agents such as aminoglycosides. Furosemide-induced ototoxicity usually is
temporary, but can be permanent in some cases.
Patients receiving loop diuretics also may develop hypokalemia, metabolic alkalosis, and
hypomagnesemia (from increased urinary losses). Abnormalities in serum calcium are not
typical, despite hypercalciuria. Hypercalciuria can lead to nephrocalcinosis, which can be
longstanding, even after discontinuation of furosemide. Patients receiving furosemide are at risk
for dehydration, but this is typically hypernatremic dehydration; hyponatremic dehydration is
more common with thiazide diuretics.
------------
Q 6
You are evaluating a 10-year-old girl for a health supervision visit. Her weight and height are at
the 50th percentile for age, her blood pressure is 108/64 mm Hg, and there are no unusual
findings on physical examination. A screening urinalysis shows a specific gravity of 1.030, pH of
6.5, 2+ blood, and no protein. Urine microscopy reveals 5 to 10 red blood cells/high-power field.
Of the following, the MOST appropriate next step is
A. abdominal computed tomography scan
B. antinuclear antibody and complement measurement
C. blood urea nitrogen and creatinine measurements
D. referral for cystoscopy
E. repeat urinalysis in 2 weeks
Answer:
E
The child who has asymptomatic, isolated microscopic hematuria is seen frequently in the
ambulatory setting. Results of a urine dipstick test in patients who have hematuria are positive
for blood, indicating the presence of hemoglobin or myoglobin. A microscopic evaluation that
reveals more than 5 red blood cells/high-power field, as described for the girl in the vignette,
confirms the presence of hematuria. Because isolated microscopic hematuria has been found in
4% of children on at least one of four tested samples, evaluation is not recommended unless
hematuria is present on at least two of three urine samples. Accordingly, the girl in the vignette
should undergo repeat urinalysis in 2 weeks.
------
Q 7:
You are seeing an 8-month-old infant for a health supervision visit. He was born at 28 weeks’
gestation, weighed 1,200 g at birth, and has bronchopulmonary dysplasia. His only medication is
furosemide, which he has been receiving for several months.
Of the following, the MOST likely expected abnormality in this infant is
A. hypercalciuria
B. hypermagnesemia
C. hypocalcemia
D. hyponatremic dehydration
E. metabolic acidosis
Answer
A
Useful information about Furosemide
Furosemide is a loop diuretic that acts at the ascending limb of the loop of Henle (LOH).
Normally, approximately 20% to 25% of filtered sodium is reclaimed at this site, so blockade of
sodium reabsorption results in a brisk diuresis, with urinary losses of sodium, potassium, and
chloride. The blocked transporter is the Na+-K+-2Cl- channel.
In addition to direct effects on the electrolytes of this channel, loop diuretics influence
calcium and magnesium transport. Calcium and magnesium are reabsorbed passively in the
ascending limb of the LOH through paracellular transport as a result a gradient derived from
normal sodium chloride transport. The passive reabsorption of calcium is believed to occur
through a paracellular pathway that is facilitated by paracellin-1, a tight junction protein.
Interruption of sodium chloride reabsorption results in impaired calcium reabsorption and
consequent hypercalciuria and increased risk for nephrocalcinosis and kidney stones.
Furosemide also has an effect on the composition of endolymph in the inner ear because
the Na+-K+-2Cl- transporter that is in the ascending limb of the LOH also can be found in the
marginal cells of the cochlear duct of the inner ear. Furosemide induces changes in ion transport
in these cells, reducing endocochlear potentials in the cells, which results in hearing loss.
Ototoxicity is more likely when loop diuretics are used in high doses or are used in conjunction
with other ototoxic agents such as aminoglycosides. Furosemide-induced ototoxicity usually is
temporary, but can be permanent in some cases.
Patients receiving loop diuretics also may develop hypokalemia, metabolic alkalosis, and
hypomagnesemia (from increased urinary losses). Abnormalities in serum calcium are not
typical, despite hypercalciuria. Hypercalciuria can lead to nephrocalcinosis, which can be
longstanding, even after discontinuation of furosemide. Patients receiving furosemide are at risk
for dehydration, but this is typically hypernatremic dehydration; hyponatremic dehydration is
more common with thiazide diuretics.
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