Childhood nephrotic syndrome.
Corticosteroids remain the cornerstone of treatment for childhood idiopathic nephrotic syndrome, with steroid response the dominant prognostic factor; most children respond and retain normal kidney function, though relapses and steroid toxicity drive the need for steroid-sparing agents.
Background
Idiopathic nephrotic syndrome is the most common glomerular disease in children, characterized by massive proteinuria, hypoalbuminemia, and edema. Roughly 85-90% of children respond to an oral corticosteroid course and are classified as having steroid-sensitive disease, while those who do not respond within 4-6 weeks are defined as steroid-resistant. Steroid responsiveness is the main prognostic factor and guides subsequent therapy.
Key recommendations
Oral corticosteroids are first-line for the initial episode and relapses. Most steroid-resistant cases respond to second-line immunosuppression, mainly calcineurin inhibitors (tacrolimus or cyclosporine), with angiotensin-system blockade added for residual proteinuria and hypertension. For frequently relapsing or steroid-dependent disease, steroid-sparing agents including levamisole, mycophenolate mofetil, calcineurin inhibitors, cyclophosphamide, and the anti-CD20 antibody rituximab are used to minimize cumulative steroid exposure.
Clinical implications
Children with steroid-sensitive disease generally maintain normal kidney function, so the principal morbidity arises from the side effects of repeated steroid courses and the relapsing disease course rather than from progression to kidney failure. In contrast, multidrug-resistant disease, often focal segmental glomerulosclerosis with a substantial monogenic component, carries a high risk of kidney failure and recurrence after transplantation. Close monitoring and preventive measures are warranted to avoid acute complications such as hypovolemia, acute kidney injury, infection, and thrombosis.
Category
Research
Source
Lancet
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