NEFROTÝK SENDROMLU OLGULARIMIZIN KLÝNÝK SEYÝR VE TEDAVÝYE CEVAPLARININ DEÐERLENDÝRÝLMESÝ

Hastanemiz Büyük Çocuk Kliniðinde 1993-1996 yýllarý arasýnda Nefrotik Sendrom (NS) tanýsý alarak, tedavi edilen, 1-14 yaþlarýnda 22'si (%44.8) kýz, 27'si (%55.2) erkek 49 hastanýn klinik seyir ve tedaviye yanýtlarýný retrospektif olarak inceledik. Olgularýn 46'sý (%93.8) primer nefrotik sendrom (NS), 3'ü (%6.1) sekonder NS'lu idi. Primer NS'lu olgularýn da biri (%2) mezangioproliferatif glomerulonefrit, 45'i (%98) minimal lezyonlu nefrotik sendrom (MLNS)'du. MLNS'lu 17 (%37.7) olgu tedaviye yanýt vermiþ ve rölaps gözlenmemiþtir. 5 olguda (%11.1) az rölaps, 13 (%28.8) olguda sýk rölaps, iki (%4.4) olguda steroide baðýmlýlýk, 5 (%11.1) olguda steroide direnç tespit edilmiþtir. Üç olguda (%6.6) spontan iyileþme görülmüþtür. Sekonder NS'lu olgularýmýzýn ise biri (%33.3) amiloid nefroz, bir diðeri (%33.3) lupus nefriti, diðeri (%33.3) ise hepatit A nefritiydi. Lupus nefritli hastamýz yüksek doz steroid tedavisine yanýt vermiþ olup diðer amiloid nefroz ve hepatit A nefriti olgularýmýza anjiotensin converting enzim inhibitör (ACEI) uygulanmýþtýr. Amiloid nefroz dýþýndaki olgumuzda proteinüri azalmýþtýr.

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.