2. DISEASES PRESENTING PRIMARILY WITH
HEMATURIA
Acute Post-streptococcal Glomerulonephritis
A 10-year-old boy presents with Coca-Cola–colored
urine and edema of his lower extremities. On
physical examination, the patient has a blood
pressure of 185/100 mm Hg. He does not appear
to be in any distress. His lungs are clear to
auscultation, and his heart has a regular rate and
rhythm without any murmurs, gallops, or rubs. His
past medical history is remarkable for a sore
throat that was presumed viral by his physician 2
weeks before.
3. Etiology
Follows infection with nephrogenic strains of
group A beta-hemolytic streptococci of the
throat (mostly in cold weather) or skin (in warm
weather)
Diffuse mesangial cell proliferation and lumpy-
bumpy deposits of immunoglobulin (Ig) and
complement on glomerular basement membrane
and in mesangium
4. Clinical presentation
Most 5–12 years old (corresponds with typical age for
strep throat)
1–2 weeks after strep pharyngitis or 3–6 weeks
after skin infection (impetigo)
Ranges from asymptomatic microscopic hematuria to
acute renal failure
Edema, hypertension, hematuria (classic triad)
Constitutional symptoms—malaise, lethargy, fever,
abdominal or flank pain
5. Diagnosis
Urinalysis — RBCs, RBC casts, protein 1–2 +, poly-
morphonuclear cells
Mild normochromic anemia
Low C3 (returns to normal in 6–8 weeks)
Need positive throat culture or increasing antibody
titer to streptococcal antigens; best single test is
the anti-DNase antigen
Consider biopsy only in presence of acute renal
failure, nephrotic syndrome, absence of streptococcal
or normal complement; or if present >2 months after
onset
7. Treatment (in-patient, if severe)
Antibiotics for 10 days (penicillin)
Sodium restriction, diuresis
Fluid and electrolyte management
Control hypertension (calcium channel blocker,
vasodilator, or angiotensin- converting enzyme
inhibitor)
Complete recovery in >95%
8. IgA nephropathy (Berger
disease)
Most common chronic glomerular disease
worldwide
Clinical presentation
Most commonly presents with gross hematuria in
association with upper respiratory infection or
gastrointestinal infection
Then mild proteinuria, mild to moderate hypertension
Normal C3
Most important primary treatment is blood
pressure control.
9. Alport syndrome
The school nurse refers a 7-year-old boy because
he failed his hearing test at school. The men in this
patient’s family have a history of renal problems,
and a few of his maternal uncles are deaf. A
urinalysis is obtained from the patient, which shows
microscopic hematuria.
10. Alport syndrome
Hereditary nephritis (X-linked dominant); renal
biopsy shows foam cells
Asymptomatic hematuria and intermittent gross
hematuria 1–2 days after upper respiratory
infection
Hearing deficits (bilateral sensorineural) females
have subclinical hearing loss
Ocular abnormalities
11. Hemolytic uremic syndrome (HUS)
A 3-year-old child presents to the emergency center
with history of bloody diarrhea and decreased
urination. The mother states that the child’s
symptoms began 5 days ago after the family ate at a
fast-food restaurant. At that time the patient
developed fever, vomiting, abdominal pain, and
diarrhea. On physical examination, the patient
appears ill. He is pale and lethargic.
12. Most common cause of acute renal failure in
young children
Micro-angiopathic hemolytic anemia,
thrombocytopenia, and uremia
Most from E. coli O157:H7 (shiga toxin–
producing)
Most from undercooked meat or unpasteurized milk;
spinach
Also from Shigella, Salmonella, Campylobacter,
viruses, drugs, idiopathic
13. Pathophysiology
Capillary and arteriolar endothelial injury → localized
clotting
Mechanical damage to RBCs as they pass through
vessels
Intrarenal platelet adhesion and damage (abnormal
RBCs and platelets then removed by liver and spleen)
14. Clinical presentation
Most common <4 years old
Bloody diarrhea
5–10 days after infection, sudden pallor, irritability,
weakness, oliguria occur; mild renal insufficiency
to acute renal failure (ARF)
Labs—hemoglobin 5–9 mg/dL, fragmented
cells, white blood cells up to 30,000/mm3,
Coombs negative, platelets usually 20,000–
100,000/mm3, low-grade microscopic hematuria
and proteinuria
Many complications, including seizures, colitis,
heart disease, death
15. Treatment
Meticulous attention to fluids and electrolytes
Treat hypertension
Aggressive nutrition (total parenteral nutrition [TPN])
Early peritoneal dialysis
No antibiotics if E. coli O157:H7 is
suspected—treatment increases risk of
developing HUS
Fresh frozen plasma—may be beneficial
Prognosis—more than 90% survive acute stage;
small number develop ESRD (end-stage renal
disease)
16. POLYCYSTIC KIDNEY DISEASE
Autosomal-Recessive Type (Infantile)
Both kidneys greatly enlarged with many cysts
through cortex and medulla
Also liver disease — bile duct proliferation and
ectasia with hepatic fibrosis
Clinical presentation
Bilateral flank masses in neonate or early infancy
Hypertension, oliguria, acute renal failure
About half have liver disease in newborn period
17. Diagnosis
Bilateral flank masses in infant with pulmonary
hypoplasia (if severe)
Oliguria and hypertension in newborn with absence of renal
disease in parents
Ultrasound–prenatal and postnatal (numerous small cysts
throughout)
Treatment and prognosis
Symptomatic
Now more than 80% with 10-year survival
End-stage renal failure in more than half
Need dialysis and transplant
18.
19. Autosomal-Dominant Type (Adults)
Most common hereditary human kidney disease
Both kidneys enlarged with cortical and medullary cysts
Most present in fourth to fifth decade, but may present in
children and neonates
Renal ultrasound shows bilateral macrocysts
Also systemic cysts — liver, pancreas, spleen, ovaries;
intracranial (Berry) aneurysm (rarely reported in children)
Diagnosis — presence of enlarged kidneys with bilateral macro-
cysts with affected first-degree relative
Treatment
control of blood pressure (disease progression
correlates with degree of hypertension); presentation in
older children with favorable prognosis
20. DISEASES PRESENTING WITH
PROTEINURIA
Nephrotic Syndrome
A 3-year-old child presents to the physician with a
chief complaint of puffy eyes. On physical
examination, there is no erythema or evidence of
trauma, insect bite, cellulitis conjunctival injection, or
discharge.
21. Steroid-sensitive minimal change disease is the most
common nephrotic syndrome seen in children.
Features
Proteinuria (>40 mg/m2/hour)
Hypoalbuminemia (<2.5 g/dL)
Edema
Hyperlipidemia (reactive to loss of protein)
22. Minimal change disease
Clinical presentation
Most common between 2 and 6 years of age
May follow minor infections
Edema—localized initially around eyes and lower
extremities;
Common—diarrhea, abdominal pain, anorexia
Uncommon—hypertension, gross hematuria
23. Diagnosis
Urinalysis shows proteinuria (3–4 +)
Some with microscopic hematuria
24-hour urine protein—40 mg/m2/hour in children but
now preferred initial test is a spot urine for
protein/creatinine ratio >2
Serum creatinine usually normal but may be
increased slightly
Serum albumin <2.5 g/dL
Elevated serum cholesterol and triglycerides
C3 and C4 normal
24. Treatment
Mild — outpatient management; if severe—hospitalize
Start prednisone for 4–6 weeks, then taper 2–3 months
without initial biopsy
Consider biopsy with hematuria, hypertension, heart
failure, or if no response after 8 weeks of prednisone
(steroid resistant)
Sodium restriction
If severe—fluid restriction, plus intravenous 25%
albumin infusion, followed by diuretic to mobilize
and eliminate interstitial fluid
Re-treat relapses (may become steroid-dependent
or resistant); may use alternate agents
(cyclophosphamide, cyclosporine, high-dose pulsed
methylprednisolone); renal biopsy with evidence of
steroid dependency
25. Complications
Infection is the major complication; make sure
immunized against Pneumococcus and Varicella
Most frequent is spontaneous bacterial peritonitis
(S. pneumoniae most common)
26. Undescended Testes
Most common disorder of sexual differentiation in
boys (more in preterm)
Testes should be descended by 4 months of age or will
remain undescended
Usually in inguinal canal, but some are ectopic
Prognosis
Treated: bilateral (50–65% remain fertile), unilateral (85% remain
fertile)
Untreated or delay in treatment: increased risk for malignancy
(seminoma most common)
Surgery (orchiopexy) at 9–15 months
27.
28. Testicular Torsion
Most common cause of testicular pain age >12 years
Clinical presentation—acute pain and swelling;
tenderness to palpitation
Testicle in transverse lie and retracted, no cremasteric
reflex
Diagnosis—Doppler color flow ultrasound
Treatment
emergent surgery (scrotal orchiopexy); if within 6 hours
and <360-degree rotation, >90% of testes survive
29.
30. Epididymitis
Ascending, retrograde urethral infection → acute
scrotal pain and swelling (rare before puberty)
Main cause of acute painful scrotal swelling in a
young, sexually active male
Urinalysis shows pyuria (can be N. gonorrhoeae or
Chlamydia, but organisms mostly undetermined)
Treatment—bedrest and antibiotics
