Definition (Kidney Int 2004;66:1294)
• Constellation of edema, proteinuria, hypoalbuminemia, and ↑ plasma cholesterol levels
Etiology (Kidney Int 2004;66:1294)
• Heterogenous disorder with mutations in multiple slit diaphragm and podocyte proteins now identified (N Engl J Med 2006;354:1387)
• Minimal change dz used to account for ∼60–85% of nephrotic syndrome; followed by idiopathic focal segmental glomerulosclerosis (FSGS), mesangial prolif (MP),
membranoprolif glomerulonephritis (MPGN), and membranous nephropathy (MN)
• ↑ ing fraction attributable to FSGS in recent past, particularly w/ African-Americans
Evaluation (Kidney Int 2004;66:1294)
• Bx may be indicated for those p/w typical features of FSGS (HTN, hematuria, renal insuff) or for those failing to respond to steroids
• Adolesc may be biopsied before steroids as FSGS and MN are more common
Therapy (Pediatr Nephrol 2011;26:881)
• Initial rx prednisone 60 mg/m2/d divided doses. 12 wk course may ↓ rate of relapse
• Steroid responsiveness confers favorable prognosis; check PPD before Rx
• W/ failure to respond to steroids, may use cytotoxic agents, calcineurin inhibitors
(cyclosporine, tacrolimus; all require long-term tx), or mycophenolate mofetil and assoc
w/ worsening renal fxn over time; choice of subsequent therapy may depend on bx results