NEFROTİK SENDROMLU OLGULARIMIZIN KLİNİK SEYİR VE TEDAVİYE CEVAPLARININ DEĞERLENDİRİLMESİ

Hastanemiz Büyük Çocuk Kliniğinde 1993-1996 yılları arasında Nefrotik Sendrom (NS) tanısı alarak, tedavi edilen, 1-14 yaşlarında 22'si (%44.8) kız, 27'si (%55.2) erkek 49 hastanın klinik seyir ve tedaviye yanıtlarını retrospektif olarak inceledik. Olguların 46'sı (%93.8) primer nefrotik sendrom (NS), 3'ü (%6.1) sekonder NS'lu idi. Primer NS'lu olguların da biri (%2) mezangioproliferatif glomerulonefrit, 45'i (%98) minimal lezyonlu nefrotik sendrom (MLNS)'du. MLNS'lu 17 (%37.7) olgu tedaviye yanıt vermiş ve rölaps gözlenmemiştir. 5 olguda (%11.1) az rölaps, 13 (%28.8) olguda sık rölaps, iki (%4.4) olguda steroide bağımlılık, 5 (%11.1) olguda steroide direnç tespit edilmiştir. Üç olguda (%6.6) spontan iyileşme görülmüştür. Sekonder NS'lu olgularımızın ise biri (%33.3) amiloid nefroz, bir diğeri (%33.3) lupus nefriti, diğeri (%33.3) ise hepatit A nefritiydi. Lupus nefritli hastamız yüksek doz steroid tedavisine yanıt vermiş olup diğer amiloid nefroz ve hepatit A nefriti olgularımıza anjiotensin converting enzim inhibitör (ACEI) uygulanmıştır. Amiloid nefroz dışındaki olgumuzda proteinüri azalmıştır.

CLINICAL COURSE AND TREATMENT RESPONSE IN PATIENTS WITH NEPHROTIC SYNDROME

We retrospectively reviewed the clinical course and treatment response of 49 patients who had a diagnosis of nephrotic syndrome and were treated in the Elder Child Clinics of our hospital between 1993 and 1996. The ages of the patients ranged from one to 14 years. Of 49 patients 22 (44.8%) were girls, and 27 (55.2%) were boys. Forty-six patients (93.8%) and three patients (6.1%) had primary and secondary nephrotic syndrome, respectively. Of patients with primary nephrotic syndrome, 45 (98%) had minimal-lesion nephrotic syndrome (MLNS). Among patients with MLNS, 17 (37.7%) patients responded well to treatment and did not develop recurrences, whereas five (11.1%) had minimal and 13 (28.9%) had frequent recurrences; two patients (4.4%) had steroid-dependent and five patients (11.1%) had steroid-resistant MLNS. Three (6.6%) had recovered without any medical therapy. Three patients with secondary nephrotic syndrome had amyloid nephrosis (33.3%), lupus nephritis (33.3%), and hepatitis A nephritis (33.3%), respectively. The patient with lupus nephritis responded to high-dose steroid therapy. The remaining two patients with amyloid nephrosis and hepatitis A nephritis were administered angiotensin-converting enzyme inhibitör (ACEI) therapy. Proteinuria decreased in patients with lupus nephritis and hepatitis A nephritis.