[Treatment of idiopathic nephrotic syndrome in children]

Area of Science:

• Pediatric Nephrology
• Internal Medicine
• Renal Histopathology

Context:

• Idiopathic nephrotic syndrome (INS) is a primary kidney disease in children, with an annual incidence of 1-3 per 100,000.
• Characterized by proteinuria and hypoalbuminemia, INS presents with varied, non-specific renal histological findings.
• Initial management involves corticosteroid therapy, with renal biopsy typically deferred.

Purpose:

• To review current, evidence-based recommendations for managing pediatric idiopathic nephrotic syndrome.
• To outline treatment strategies for steroid-responsive and steroid-resistant cases of INS.
• To provide an update on therapeutic options for children with difficult-to-treat nephrotic syndrome.

Summary:

• The majority of children (80-90%) with INS respond to initial steroid treatment.
• Children with focal and segmental glomerulosclerosis who are steroid-resistant often require immunosuppressive agents.
• Advanced therapies include calcineurin inhibitors (cyclosporine, tacrolimus), mycophenolate mofetil, and rituximab, alongside renin-angiotensin system blockade.

Impact:

• Informs clinical practice for pediatric nephrologists and pediatricians treating INS.
• Aims to improve treatment outcomes for children with idiopathic nephrotic syndrome.
• Highlights the evolving therapeutic landscape for steroid-resistant nephrotic syndrome in pediatric populations.