Treatment approaches in children with steroid sensitive nephrotic syndrome

Abstract

Pediatric nephrotic syndrome (NS) is a chronic glomeru­lar disorder, and if untreated, is associated with increased risk of life-threatening infectious, thromboembolism, lipid abnormalities, and malnutrition. The aim of the manage­ment of NS in children is to induce and maintain com­plete remission with resolution of proteinuria and edema without encountering serious adverse effects of therapy. Over 90% of cases in children with idiopathic NS and a majority of them will respond to steroid therapy. However, a substantial number of patients relapse frequently and become steroid dependent. The occurrence of frequent relapses necessitates clear therapeutic strategies in or­der to maintain sustained remission and minimize steroid toxicity. Numerous therapeutic regimens have been pro­posed utilizing steroid sparing agents such as alkylating agents, principally, cyclophosphamide and chlorambucil, calcineurin inhibitors namely cyclosporin A and an immu­nomodulatory drug, levamisole, with variable success and associated side-effects. Recently, mycophenolate mofetil (MMF), tacrolimus, the anti-CD20 antibody, rituximab, have emerged as new therapeutic options for the man­agement of steroid dependent NS in a few uncontrolled clinical trials. It is therefore important that the benefits and risks of these agents are weighed before considering their use in the treatment of patients with NS.

Keywords

• Childhood, steroid sensitive nephrotic syn­drome, treatment.

Steroide yanıtlı nefrotik sendromlu çocuklarda tedavi yaklaşımları

Abstract

Çocukluk çağı nefrotik sendromu kronik bir glomerül has­talığıdır. Eğer tedavi edilmezse, yaşamı tehdit eden en­feksiyonlar, tromboembolizm, lipid anormallikleri ve mal­nutrisyon gibi komplikasyonları vardır. Tedavinin amacı proteinüriyi ve ödemi ciddi yan etkiler ortaya çıkmadan düzeltmektir. İdiopatik nefrotik sendromun (İNS) %90\'dan fazlası steroid tedavisine yanıt verir. Bununla birlikte, önemli sayıda hasta sık relaps gösterir ve steroide bağım­lı hale gelir. Sık relapsların ortaya çıkışı remisyon sağla­yıcı ve steroid toksisitesini azaltıcı yeni tedavi stratejilerini gerektirir Steroid yan etkilerinden koruyucu tedavi rejim­leri olarak alkilleyici ajanlar (siklofosfamid ve klorombusil), kalsinörin inhibitörleri (siklosporin A), immunomodulatör ajanlar (levamizol) önerilmiştir. Yakın zamanlarda, mikofe­nolat mofetil (MMF), tacrolimus, anti-CD20 antikoru olan rituximab steroid bağımlı nefrotik sendromda (SBNS) az sayıda kontrolsuz çalışma ile yeni tedavi seçenekleri ola­rak ortaya çıkmıştır. Bu nedenle nefrotik sendromlu ço­cuklarda bu ilaç tedavilerinin, kullanılmadan önce yarar ve risklerinin çok iyi değerlendirilmesinin önemi ortaya çıkmaktadır.

Keywords

• Çocukluk çağı, steroide yanıtlı nefro­tik sendrom, tedavi.

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