Korean Journal of Pediatrics 2008;51(3):293-298.
Published online March 15, 2008.
Therapeutic response of cyclosporine and outcome in steroid resistant nephrotic syndrome
Hyung Soon Choi, Joo Hoon Lee, Young Seo Park
Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
스테로이드 저항성 신증후군에서 cyclosporine 치료 반응 및 결과
최형순, 이주훈, 박영서
울산대학교 의과대학 서울아산병원 소아과학교실
Correspondence: 
Young Seo Park, Email: yspark@amc.seoul.kr
Abstract
Purpose
: The aim of our study was to evaluate the therapeutic response to cyclosporine, time to remission and side effects in steroid resistant nephrotic syndrome (SRNS).
Methods
: This study included 22 children with idiopathic SRNS who were treated with cyclosporine between June 1989 and August 2006. Medical records were reviewed retrospectively.
Results
: The mean age of patients at diagnosis was 5.2?.3 years. The male to female ratio was 1.2:1. Pre-treatment renal biopsies showed minimal change (MCD) in 12 (54.5%), focal segmental glomerulosclerosis (FSGS) in 8 (36.4%), membranous nephropathy (MGN) in one (4.5%) and mesangioproliferative glomerulonephritis in one (4.5%). 15 (68.2%) patients responded to cyclosporine, of whom 11 (91.6%) patients were MCD, 3 (37.5%) patients FSGS, and 1 patient MGN (MCD vs FSGS, P<0.05). The time to remission in patients who responded to cyclosporine was 31.5?5.2 days. Four of the 15 cyclosporine responders maintained complete remission even after cessation of the medication Seven still received cyclosporine, 2 were intermittently treated with steroids after discontinuation of cyclosporine, and two were treated with cyclosporine and steroids. The mean duration of cyclosporine therapy was 546.5?46.2, 1,392.9?39.7, 439.5?4.1, and 433.5?4.2 days, respectively. We performed post-treatment biopsies in 8 patients and partial interstitial fibrosis and tubular atrophy were found in two.
Conclusion
: The thrapeutic response of cyclosporine is good in steroid resistant nephrotic syndrome, especially in minimal change. But, there is a problem of long term cyclosporine dependency.
Key Words: Children, Steroid resistant nephrotic syndrome, Cyclosporine


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