All issues > Volume 51(3); 2008

Original Article

Korean J Pediatr. 2008;51(3):293-298. Published online March 15, 2008.

Therapeutic response of cyclosporine and outcome in steroid resistant nephrotic syndrome

Hyung Soon HS Choi¹, Joo Hoon JH Lee¹, Young Seo YS Park¹

¹Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Correspondence Young Seo YS 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.

Keywords :Children, Steroid resistant nephrotic syndrome, Cyclosporine