Çocukluk Çağı Nefrotik Sendromu: 230 Olgunun Değerlendirilmesi

Abstract

Nephrotic Syndrome in Childhood: Retrospective evaluation of 230 cases In this study, we evaluated 230 children (0 to 17 years old) with nephrotic syndrome (NS) who admitted to the Department of Pediatrics of Ondokuz Mayis University between January 1986 to December 1998, retrospectively. The diagnostic criteria of International Study of Kidney Diseases of Children (ISKDC) were used to define the patients in this study. Patients were classified into two groups according to etiology as primary and secondary NS. Clinical and laboratory findings of all cases were examined. It is observed that 77.8% of the cases had primary and 17.4% had secondary NS, 4.8% of them could not be classified. 39.1% of the patients were female and 60.9% of them were male. Female/male ratio was 1/1.6. Steroid responsive cases consisted 77.8% of the cases with minimal change NS (MCNS). 51% of the patients with steroid responsive MCNS developed one or more relapses. Among the relapses, rare and frequent relapses were 29.4% and 10.7%, respectively. 3.9% of the patients with MCNS were steroid dependent. Amyloidosis was diagnosed in 27 patients and it was the most common cause of secondary NS. The most frequent complication was infection. In 230 patients, 57.4% went to remission, 5.6% died and 10.4% progressed to chronic renal failure. These findings were evaluated according to age, sex, etiology, response to treatment, prognosis and compared with national data and the literature. Bu calismada Ondokuz Mayis Universitesi Tip Fakultesi Cocuk Sagligi ve Hastaliklari Anabilimdali Pediatrik Nefroloji unitesinde Ocak 1986 ile Aralik 1998 yillan arasinda gecen 12 yillik sure icinde izlenen 0-17 yas grubunda nefrotik sendrom (NS) tanisi alan 230 hasta retrospektif olarak incelendi. Calismaya dahil edilen NS'lu hastalarda Uluslararasi Cocuk Bobrek Hastaliklari Calisma Grubunca (ISKDC) kabul edilen tani kriterleri kullanildi. Etiyolojik nedenlere gore primer (idiyopatik) ve sekonder NS olarak siniflandirilan olgularin klinik ve laboratuvar bulgulari incelendi. Olgularin %77.8'i primer, %17.4'u sekonder NS olarak belirlendi, %4.8 olguda ayrini yapilamadi. Olgularin %39.1'u kiz, %60.9'i erkekti ve kiz/erkek orani 1/1.6 olarak saptandi. Minimal lezyonlu NS (MLNS) dusunulerek kortikosteroid tedavisi verilen hastalarin (n: 127) %77.8'i steroide yanit verdi. Steroide yanit veren hastalarin %51'inde relaps gozlendi ve relaps olgularinin %29.4'u nadir relaps, %10.7'si sik relaps seklindeydi. Hastalarin %3.9'u ise steroide bagimli olarak saptandi. Amiloidozis 27 hastada saptandi ve sekonder NS'un en sik nedeni idi. Gelisen komplikasyonlar icinde en sik enfeksiyonlar goruldu. Toplam 230 NS'lu hastanin %57.4'u remisyona girdi, %5.6'i eksitus oldu ve %10.4'unde kronik bobrek yetmezligi (KBY) gelisti. Yas, cins, etiyolojik dagilim, tedaviye yanit ve prognoz yonunden incelenen nefrotik sendrom tanisi almis 230 olgunun bulgulari ulkemiz verileri ve literatur bulgulariyla karsilastirildi.