Treatment approaches in children with steroid sensitive nephrotic syndrome

Öz

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.

Anahtar Kelimeler

• Childhood, steroid sensitive nephrotic syn­drome, treatment.

Steroide yanıtlı nefrotik sendromlu çocuklarda tedavi yaklaşımları

Öz

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.

Anahtar Kelimeler

• Çocukluk çağı, steroide yanıtlı nefro­tik sendrom, tedavi.

Kaynakça

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