Nephrotic syndrome in children: Challenges of treatment

Abstract

Minimal change disease is the most common cause of idiopathic childhood nephrotic syndrome, however, the incidence of focal segmental glomerulosclerosis appears to be rising in many countries. The main challenges in treating the nephrotic child include management of both the steroid-dependent and steroid-resistant patient. In the steroid-dependent child, evidence of serious steroid toxicity necessitates alternative immunosuppressive therapy such as alkylating agents or cyclosporine, with their potential for long-term side-effects. The child with steroid-resistant disease is at high risk of the complications of nephrotic syndrome, including intractable oedema, infections, thrombosis and the long-term consequences of hyperlipidemia. The role of alternative immunosuppressive agents such as cyclophosphamide and cyclosporine is limited, as response rates range from 10 to 30%. Non-immunological treatment with angiotensin-converting-enzyme inhibitors and non-steroidal anti-inflammatory agents have been advocated to reduce the proteinuria. Newer experimental modalities for the treatment of the 'difficult' nephrotic child with focal segmental glomerulosclerosis include the use of plasmapheresis for removal of a putative 'circulating factor' that appears to mediate proteinuria in these patients.