Answers and Explanations

1. The answer is D [XIV.A, XIV.E.1, and XIV.F]. Infants with urinary tract infections (UTIs) have
an increased risk of having underlying structural abnormalities, including vesicoureteral
reflux. A structural abnormality of the urinary tract, such as vesicoureteral reflux, predisposes
a child to developing a UTI. Before 6 months of age, UTIs are twice as common in boys, but
after 6 months of age, UTIs are more common in girls. Before 6 months of age, UTIs are 10
times more common in uncircumcised boys as compared with circumcised boys. In neonates
and infants, urine for culture must be collected by suprapubic aspiration of the urinary
bladder or by a sterile urethral catheterization. Bagged specimens obtained from an infant are
inappropriate for culture as they are very likely to be contaminated. Outpatient management
for older nontoxic children with suspected UTI may be appropriate. However, toxic-appearing
children, neonates, and patients who have significant dehydration should be hospitalized and
administered intravenous antibiotics initially.
2. The answer is D [Figure 11-1]. Patients may develop red-colored urine from the ingestion of
exogenous pigments, such as those found in beets, and from medications, such as phenytoin
and rifampin. Such patients have negative urine dipsticks for blood. Although 4–5% of school
children may have microscopic hematuria on a single voided urine sample, only 0.5–2% have
persistent microscopic hematuria on retesting. The diagnosis of microscopic hematuria may be
made if ≥6 red blood cells (RBCs) are noted per high-power field on three or more consecutive
urine samples. Patients with positive dipsticks for blood should have microscopic evaluations
of fresh urine specimens. RBCs that appear as normal biconcave disks usually originate in the
lower urinary tract, unlike dysmorphic RBCs, which are more likely to originate in the
glomerulus. False-negative results on dipstick for blood may occur with ascorbic acid (vitamin
C) ingestion.
3. The answer is E [I.D, I.E]. Oral rehydration therapy has been shown to be a safe and
inexpensive alternative to intravenous rehydration and effective even in the face of secretory
diarrhea, such as would be seen in cholera. Patients with secretory diarrhea still maintain their
ability to absorb fluid and electrolytes through an intact, coupled cotransport mechanism.
However, oral rehydration therapy should not be used for patients with severe life-
threatening dehydration, paralytic ileus, or gastrointestinal obstruction. In patients with these
problems, parenteral rehydration is more appropriate. The goal of the first phase of parenteral
rehydration (emergency phase) is to restore or maintain the intravascular volume to ensure
perfusion of vital organs, and this phase is the same for all patients (regardless of the patient’s
initial serum sodium level). Appropriate fluids for use in the emergency phase include
isotonic crystalloids, such as normal saline or lactated Ringer solutions in boluses of 20 mL/kg.
One quarter– or one half–normal saline is not an appropriate intravenous fluid for the
emergency phase. The subsequent repletion phase, or the more gradual correction of fluid and
electrolyte deficits, should occur over 24 hours for patients with isonatremic and
hyponatremic dehydration, and over 48 hours for patients with hypernatremic dehydration.
There is a risk of cerebral edema if deficit replacement occurs too quickly in patients with
hypernatremic dehydration. Ongoing losses should be replaced on a “milliliter for milliliter
basis” concurrent with the replacement of deficits.
4. The answer is E [V.F.1]. The most common form of acute glomerulonephritis in school-age
children is poststreptococcal glomerulonephritis. Patients usually present with hematuria,
proteinuria, and hypertension after an infection of the skin (sometimes up to 28 days after
impetigo) or pharynx (usually 10–14 days after pharyngitis) with a nephritogenic strain of
group A β-hemolytic streptococcus. The prognosis for children with poststreptococcal
glomerulonephritis is excellent, and affected children usually recover completely; renal failure

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 is rare. Laboratory features consistent with the diagnosis include transient low serum
complement levels. The antistreptolysin O titer is positive in 90% of children after a
respiratory infection but in only 50% of patients who have had skin infections. Antibiotic
treatment of streptococcal pharyngitis or impetigo does not reduce the risk of
poststreptococcal glomerulonephritis, although the risk of rheumatic fever is reduced.
5. The answer is D [VI.A, VI.B, VI.E, and VI.G.5]. Nephrotic syndrome in children is defined as
heavy proteinuria (>50 mg/kg/24 hours), hypoalbuminemia, hypercholesterolemia, and
edema. Patients with nephrotic syndrome are susceptible to infections with encapsulated
organisms, such as pneumococcal infections, and are at risk for developing peritonitis,
pneumonia, and overwhelming sepsis. Patients with nephrotic syndrome and fever should
therefore be treated empirically with antibiotics pending culture results. The most common
form of nephrotic syndrome in children is minimal change disease, which comprises 90% of all
cases. Primary glomerular disease (e.g., IgA nephropathy) and systemic diseases (e.g.,
systemic lupus erythematosus) are less common causes of nephrotic syndrome in children.
Most cases of childhood nephrotic syndrome (two-thirds) occur in children younger than
5 years of age. Renal biopsy to establish the diagnosis or to determine a management approach
is not indicated for most patients with nephrotic syndrome. However, it is indicated for
patients who have impaired creatinine clearance or for those who do not respond to initial
management with corticosteroids.
6. The answer is E [VII.C.3]. There are three subtypes of hemolytic uremic syndrome (HUS), a
Shiga toxin–associated form (the most common form in childhood), pneumococcal- associated
form, and an atypical form caused by medications or complement system dysregulation which
is often inherited. Shiga toxin–associated HUS occurs as a result of intestinal infection with a
toxin-producing bacterial strain, most commonly Escherichia coli 0157:H7. The toxin binds to
vascular endothelial cells, especially in the renal vasculature, causing platelet thrombi and
resultant renal ischemia. Patients with HUS have a microangiopathic hemolytic anemia, renal
impairment, and thrombocytopenia, which may result in visible petechiae on the skin. The
prognosis for patients with Shiga toxin–associated HUS is generally good, although poor
prognostic signs include elevated white blood cell count on admission and prolonged
oliguria. Antibiotic treatment of the E. coli hemorrhagic colitis is controversial and may
actually increase the likelihood that a patient will go on to develop HUS.
7. The answer is C [V.F.2]. This patient’s clinical presentation and ethnicity are consistent with
IgA nephropathy (Berger disease), the most common form of chronic glomerulonephritis in
the world. Patients with IgA nephropathy typically present in the second or third decade of
life with recurrent bouts of gross hematuria associated with respiratory infections. IgA
nephropathy is most common in Asia and Australia and in Native Americans.
Membranoproliferative nephritis, membranous nephropathy, and nephritis as a result of
systemic lupus erythematosus during childhood are all less common causes of
glomerulonephritis in children. This patient’s clinical presentation is not consistent with
Henoch–Schönlein purpura, which is characterized by abdominal pain, palpable purpura on
the buttocks and thighs, and joint symptoms.
8. The answer is D [XII.E]. Vesicoureteral reflux (VUR) is caused by abnormalities of the
ureterovesical junction, most commonly a shortened submucosal tunnel in which the ureter
inserts through the bladder wall. The inheritance pattern is most commonly autosomal
dominant with variable expression but autosomal recessive inheritance has also been
described. The majority of children with VUR eventually outgrow the reflux, although a
minority develop severe renal impairment owing to reflux nephropathy from severe VUR.
Patients with grade 4 or 5 VUR should be referred to a pediatric urologist for consideration of
ureteral reimplantation. Although previously recommended, low-dose prophylactic
antibiotics to decrease the risk of urinary tract infection in low risk patients is not indicated.

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 9. The answer is E [I.B and I.C]. Maintenance water and electrolyte calculations are designed to
balance the usual daily losses of water and salts as a result of normal daily metabolic activities.
These losses include both measurable forms (sensible losses), such as urinary losses, and less
readily measurable but still clinically significant forms (insensible losses), such as losses from
the skin, lungs, or gastrointestinal tract. The maintenance sodium requirement is
approximately 2–3 mEq/kg/day for infants and children. When calculating maintenance fluids
using the surface area method, the maintenance water requirement for children is
1500 mL/m2/day. Fever will result in increased insensible losses, and maintenance fluids
should be increased by about 10% for every degree of temperature above 38°C. Maintenance
fluid calculations should not be adjusted for increased ongoing losses, such as profuse watery
diarrhea. Instead, any increased stool losses should be replaced on a “milliliter per milliliter”
basis.
10. The answer is E [Figure 11-2 and Table 11-3]. This patient likely has a renal vein thrombosis,
which presents in infancy with the sudden onset of gross hematuria and unilateral or bilateral
flank masses. Acute renal failure may result. Infants of diabetic mothers have a greatly
increased risk of renal vein thrombosis. Maternal systemic lupus erythematosus (SLE) would
not result in hematuria in the infant; however, maternal SLE is associated with infant heart
block. Adenovirus infection is a cause of hemorrhagic cystitis, which often causes hematuria;
however, this would be very unusual in a neonate. Similarly, although sickle cell disease also
causes hematuria, it would be an uncommon presentation in a neonate. Hypercalciuria is a
common cause of hematuria; however, a flank mass would not be a presenting sign.
11. The answer is C [IX.E and Table 11-2]. The classic electrolyte presentation seen in renal
tubular acidosis (RTA) is a hyperchloremic metabolic acidosis with a normal serum anion gap.
Hypokalemia is not specifically associated with RTA; however, type IV RTA is associated with
hyperkalemia. Patients with Fanconi syndrome may present with proximal (type II) RTA with
glucosuria, aminoaciduria, and hyperphosphaturia (therefore low serum phosphorus levels,
not high serum phosphorus levels). Hypocalcemia is not a feature of RTA.
12. The answers are B, A, and D, respectively [VIII.D, VIII.E, and VIII.B]. Infantile polycystic
kidney disease (question 12) is an autosomal recessive disorder characterized by greatly
enlarged cystic kidneys, severe hypertension, and variable degrees of liver involvement.
Severe cases are associated with oligohydramnios, pulmonary hypoplasia, and early neonatal
death. Although the family history may be negative in recessive disorders, there is a 25% risk
of affected siblings in subsequent pregnancies. In contrast, adult polycystic kidney disease
(question 13) is inherited in an autosomal dominant pattern, and there is considerable
variability in its severity, ranging from mild microscopic hematuria to severe hypertension
and renal failure. Adult polycystic kidney disease may also be associated with cerebral
aneurysms and early death. Alport syndrome (question 14) is inherited in multiple patterns,
but the X-linked form is by far the most common. Alport syndrome is characterized by renal
manifestations, including hypertension and hematuria, as well as renal failure more
consistently in the males; hearing loss; and ocular abnormalities of the lens and retina.

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