Methylprednisolone Pulse Therapy for Focal Segmental Glomerulosclerosis in Children: with the Mendoza Protocol

Korean Journal of Nephrology 2011;30(1):18-25.

Eun Ae Yang, Hyo Jung Park, Min Hyun Cho, and Cheol Woo Ko

Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea

원저 : Methylprednisolone 충격요법을 시행한 국소성 분절성 사구체 경화증 환아들의 임상경과

양은애, 박효정, 조민현, 고철우

경북대학교 의학전문대학원 소아과학교실

Abstract

Purpose: The clinical characteristics and pathological findings of patients with focal segmental glomerulosclerosis (FSGS) receiving methylprednisolone pulse therapy (MP pulse therapy) according to the Mendoza protocol were studied to evaluate its therapeutic efficacy. Methods: The clinical course and pathological findings of 12 patients that were diagnosed with FSGS and treated according to the Mendoza protocol from 2001 to 2006 were reviewed retrospectively. Results: Only three patients among the total twelve patients finished MP pulse therapy, who were diagnosed with minimal change nephrotic syndrome on the first renal biopsy and have preserved renal function at the recent follow-up. Among them, one patient (8%) achieved complete remission, but relapsed 28-months after the end of therapy, and one patient (8%) had a partial remission. Eight patients progressed to end-stage renal disease (ESRD) and four regressed during therapy. All eight patients that were diagnosed with FSGS, on the first renal biopsy, progressed to ESRD and required hemodialysis or kidney transplantation. The frequency of ESRD in this group was statistically significant. Conclusion: MP pulse therapy according to the Mendoza protocol showed low therapeutic efficacy; it appeared effective in only 17% and most of the patients (67%) progressed to ESRD. There may be close correlation between the severity of glomerular sclerosis at biopsy and ESRD. These results suggest that the indications for MP pulse therapy according to the Mendoza protocol in nephrotic patients with FSGS requires further clarification.

Key Words: Focal segmental glomerulosclerosis, Methylprednisolone, End stage renal disease