Cyclosporine A (Cipol®) Therapy in Children with Idiopathic Nephrotic Syndrome

Hong, Ihn Hee; Ko, Cheol Woo; Koo, Ja Hoon; Kim, Ji-Hong; Kim, Pyung-Kil; Cho, Byoung Soo

J Korean Soc Pediatr Nephrol > Volume 3(1); 1999 > Article

J Korean Soc Pediatr Nephrol 1999;3(1): 48-56.

소아 특발성 신증후군에서의 cyclosporine A(Cipol®)의 치료 효과

홍인희, 고철우, 구자훈, 김지홍, 김병길, 조병수

¹경북대학교 의과대학 소아과학교실
²연세대학교 의과대학 소아과학교실
³경희대학교 의과대학 소아과학교실
⁴경희대학교 의과대학 소아과학교실
⁵경희대학교 의과대학 소아과학교실
⁶경희대학교 의과대학 소아과학교실

Cyclosporine A (Cipol®) Therapy in Children with Idiopathic Nephrotic Syndrome

Ihn Hee Hong, Cheol Woo Ko, Ja Hoon Koo, Ji-Hong Kim, Pyung-Kil Kim, Byoung Soo Cho

¹Department of Pediatrics, Kyungpook Uniyersity, College of Medicine
²Department of Pediatrics, Yonsei University, College of Medicine
³Department of Pediatrics, Kyunghee University, College of Medicine
⁴Department of Pediatrics, Kyunghee University, College of Medicine
⁵Department of Pediatrics, Kyunghee University, College of Medicine
⁶Department of Pediatrics, Kyunghee University, College of Medicine

ABSTRACT

Purpose : This multicenter collaboratory study was conducted to see the therapeutic efficacy and side effect of cyclosporine A (Cipol-$N^{(R)}$, Chong Kun Dang) on children with idiopathic nephrotic syndrome who experienced frequently relapsing (FR), steroid dependent (SD), or steroid resistant (SR) pattern. Patients and methods : Thirty-nine children with SD/FR NS and 3 children with SR NS were enrolled in the study. After induction of remission (SD/FR NS) with steroid or after 4 weeks of steroid therapy (SR NS), cyclosporine A was started in a dose of 4-5 mg/Kg/day in two divided dose and steroid (prednisolone or equivalent dose of deflazacort) was tapered slowly. During 16 weeks of study period, monthly check up of physical examination and various laboratory tests including BUN, creatinine, Ccr and cyclosporine blood level were done.
Results : Out of 39 children with SD/FR NS, 35($89.7%$) maintained sustained remission and at 4 weeks after therapy, values of serum protein, albumin, cholesterol, and 24 hours urinary protein excretion showed normal values. Two out of 3 children with SR NS showed and sustained remission with cyclosporine A therapy. Side reaction to cyclosporine A therapy showed hypertrichosis in 8 cases and hyperuricemia in 5 cases. However, other laboratory tests including CBC, liver profile, BUN, creatinine and GFR (creatinine clearance utilizing 24 hour urine) did not show any abnormalities during the 16 weeks of study period.
Conclusion : Cyclosporine A (Cipoi-$N^{(R)}$ Chong Kun Dang) can be utilized quite effectively on children with SD/FR or SR NS and further trial of cyclosporine A on long-term basis (1-2 year period) is needed to determine it's efficacy and side effect (especially nephrotoxicity) of long-term administration of cyclosporine A.

Key words: Idiopathic nephrotic syndrome | Cyclosporine A