Pocket Pediatrics: The Massachusetts General Hospital for Children Handbook of Pediatrics (Pocket Notebook Series), 2 Ed.

HEMATURIA, NEPHRITIC SYNDROMES

Definition (Pediatr Clin North Am 2001;48:1519)

• Macroscopic hematuria is visible to the naked eye (Pediatr Clin North Am 1997;44:1191)

• Microscopic hematuria definitions vary by study, >5–10 RBCs/high-power, midstream collection (Adolesc Med Clin 2005;16:229)

Epidemiology (Pediatrics 1977;59:557; J Pediatr 1979;95:676)

• Macroscopic hematuria has an estimated incidence of 1.3 per 1,000

• Prevalence of microscopic hematuria 0.5–2% depending on population—incidence of microscopic hematuria 0.32% in girls and 0.14% of boys

Etiology (Urol Clin North Am 2004;31:559)

• In 342 children w/ asymptomatic microscopic hematuria: 81% idiopathic, 16% hyperCa, 1% PSGN; of 228 children w/ gross hematuria: 38% idiopathic, 22% hyperCa, 15% IgA nephropathy, 4% structural abnormalities (Arch Pediatr Adolesc Med 2005;159:353)

• Mimics include meds (chloroquine, iron, isoniazid, rifampin), foods (beets, blackberry),

pigments/toxins (bile, hemoglobinuria, myoglobinuria, lead, phenol, porphyria, urates)

Glomerular dz: Recurrent gross hematuria (IgA nephropathy, benign familial

hematuria, Alport syndrome), acute postinfectious GN (PIGN), membranoproliferative GN (MPGN), SLE, membranous nephropathy (MN), rapidly progressive GN (RPGN), Henoch–Schönlein purpura, Goodpasture dz

Interstitial and tubular Dz: Acute pyelonephritis, acute interstitial nephritis, TB, hematologic (sickle-cell dz, coagulopathies including von Willebrand dz, renal vein thrombosis, thrombocytopenia), meds (AGs, NSAID, amitriptyline, anticonvulsants, chlorpromazine, coumadin, cyclophosphamide, diuretics, PCN, thorazine)

Urinary tract: Bacterial or viral (adenovirus) infxn related, urolithiasis and hypercalciuria, structural anomalies (including polycystic kidney disease), trauma, tumor, exercise

Evaluation (Urol Clin North Am 2004;31:559)

• Careful history and physical examination, including family history

Macroscopic hematuria w/o significant proteinuria or RBC casts: Likely nonglomerular bleeding; requires urine cx (exclude infxn), renal & bladder U/S (exclude malig or cystic renal dz), CT (urolithiasis), &/or cystoscopy (exclude malig, other process)

Hematuria plus any of the following is suggestive of glomerulonephritis: Edema, HTN, significant proteinuria. Initial eval: CBC (HUS), CMP, sediment, throat

cx, streptozyme panel, & complement levels (PIGN)

• See Proteinuria section for eval and Rx of disorders, for which this is a primary feature

Dx & Management of Specific Disorders (Urol Clin North Am 2004;31:559)

Postinfectious GN: Up to 7–21 d after inciting infxn, often skin or soft tissue

• Infxn w/ Group A Strep or other infections; w/ supportive care, excellent prognosis

• Dark, tea-colored urine is common

• Pts can be asymptomatic, or have malaise, fatigue, HTN, edema, or oliguria

• C3 levels low during illness, improve over 6–8 wk

• Antistreptolysin O titers may be neg at first, streptozyme pos w/i 10 d

Henoch–Schönlein purpura: (See Rheumatology chapter) w/ renal involvement ∼50% cases, extent variable (proteinuria, hematuria, nephrotic syndr, glomerulonephritis, AKI)

• Relapses can occur, long-term prognosis depends on degree of renal compromise

IgA nephropathy common cause hematuria, path w/ mesangial deposition of IgA

• Poor prognostic indicators: HTN, nephrotic range proteinuria, renal insufficiency

• Controversy exists regarding utility of biopsy

Rapidly progressive glomerulonephritis: AKI, glomerular crescents on biopsy

EMERGENCY: Need prompt Rx w/ pulse steroids, can still progress to ESRD w/i wks. Aggressively pursue/address underlying condition



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