The management of nephrotic syndrome in children

Abstract

Untreated nephrotic syndrome is associated with increased risks of life threatening infection, thromboembolism, lipid abnormalities, hypovolaemia and acute renal failure. The aims of management of a child with nephrotic syndrome are to induce and maintain complete remission with resolution of proteinuria and oedema without serious adverse effects of therapy and to prevent or treat the complications. The majority of children in Europe, the Americas and Asia have steroid sensitive nephrotic syndrome. Data from a meta-analysis of randomised controlled trials (RCTs) indicate that regimens of 1-2 months of daily prednisone (60 mg/m²/day) followed by 1.5-6 months of alternate day prednisone (starting at 40 mg/m²/ day and reducing by 5-10 mg/m²/day every 4 weeks) in the first episode of nephrotic syndrome reduce the risk for relapse. However about 80% relapse and require further courses of steroids and second line therapies if steroid toxicity ensues. Data from RCTs supports the use of alkylating agents (cyclophosphamide, chlorambucil), cyclosporin and levamisole in these children to achieve prolonged periods of remission after induction of remission with prednisone. Steroid resistant nephrotic syndrome is more common in Africa. Few therapies are effective. In such children, cyclosporin, alkylating agents and high dose intravenous methylprednisone may be used. In addition to specific therapies for nephrotic syndrome, supportive therapies are required to control oedema (loop diuretics, aldosterone antagonists, thiazide diuretics, albumin infusions, angiotensin-converting enzyme inhibitors), to reduce the risk of infection (penicillin, pneumococcal vaccination) and thromboembolism (aspirin) and to control hyperlipidaemia (HMG-CoA reductase inhibitors).

Clinics in Mother and Child Health Vol. 1(2) 2004: 101-112