New insights into treatment of nephrotic syndrome in children.

Primary nephrotic syndrome (PNS) is the most common glomerular disease seen in children. The pathogenic basis of PNS, in context of witnessed immunological dysfunction and its relationship with slit diaphragm and the podocyte cytoskeleton, has been explored for years. The outcome and management of PNS in children is determined by the response to corticosteroids and the frequency of relapses. The first episode of nephrotic syndrome should be treated adequately, both in terms of dose and duration of steroids, aiming at reducing the risk for subsequent relapses. While patients with steroid-sensitive nephrotic syndrome have a favorable long-term outcome, almost half of them relapse frequently and are at risk of adverse effects of steroids. Immunosuppressive agents are suggested for patients with frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome. Calcineurin inhibitors are strongly recommended for patients who show resistance to steroids. Rituximab is a new therapy that could be a potential area but still requires further trials. The aim of therapy is to induce and maintain remission of proteinuria while avoiding medication-related adverse effects.