Histopathologic Diagnosis and Outcome of Renal Biopsied Pediatric Nephrotic Syndrome

Chung, Hye-Jeon; Youn, Suk; Kim, Sung-Do; Cho, Byoung-Soo

J Korean Soc Pediatr Nephrol > Volume 9(2); 2005 > Article

J Korean Soc Pediatr Nephrol 2005;9(2): 149-158.

신생검을 받은 소아 신증후군에서 조직병리 소견과 치료 성과

정혜전, 윤석, 김성도, 조병수

¹경희대학교 의과대학 소아과학교실
²경희대학교 의과대학 소아과학교실
³경희대학교 의과대학 동서신장병연구소, 소아과학교실
⁴경희대학교 의과대학 동서신장병연구소, 소아과학교실

Histopathologic Diagnosis and Outcome of Renal Biopsied Pediatric Nephrotic Syndrome

Hye-Jeon Chung, Suk Youn, Sung-Do Kim, Byoung-Soo Cho

¹Disease Research Institute, Department of Pediatrics, College of Medicine, Kyung-Hee University
²Disease Research Institute, Department of Pediatrics, College of Medicine, Kyung-Hee University
³East-West Kidney Disease Research Institute, Department of Pediatrics, College of Medicine, Kyung-Hee University
⁴East-West Kidney Disease Research Institute, Department of Pediatrics, College of Medicine, Kyung-Hee University

ABSTRACT

PURPOSE: To determine the histological findings and treatment outcome in cases of childhood nephrotic syndrome which required renal biopsy. METHODS: We retrospectively reviewed the clinical, laboratory, pathologic findings and therapeutic outcomes of 169 nephrotic children who received a renal biopsy at the Department of Pediatrics, Kyunghee Medical University Hospital, Seoul from 1984 to 2004 over a period of 21 years. The renal biopsy was performed in nephrotic children who showed atypical features at presentation, or needed cytotoxic therapy because of frequent-relapsing, steroid-dependent, or steroid-resistant nephrotic syndrome(SRNS). RESULTS: Minimal change disease(MCD) was found in 52.1% of the patients, followed by diffuse mesangial proliferation(33.1%), focal segmental gomerulosclerosis(5.3%), membranoproliferative glomerulonephritis(2.4%), membranous nephropathy(2.4%), and IgA nephropathy(1.8 %). In MCD children, 14.8% had hematuria, 22.7% had hypertension, 5.7% showed decreased renal function, and no patient was found to have an abnormal complement level. Among patients diagnosed with diseases other than MCD, 43.2% had hematuria, 21.0% was found to be hypertensive, 7.4% of children showed decreased renal function and only 3(3.7%) had decreased complement level; the rates of hematuria and SRNS were found to be significantly higher than MCD patients. Among 37 SRNS patients, 30(81.0%) showed a final remission state with long-term steroid therapy, including methylprednisolone pulse therapy, over 4 months, with or without cytotoxic therapy. CONCLUSION: Almost half of the cases of childhood nephrotic syndrome requiring renal biopsy were not diagnosed with MCD. Among atypical features, hematuria and steroid-resistance would be the most probable indicators for a diagnosis other than MCD. Even in patients with SRNS, long-term methylprednisolone pulse therapy may result in a good remission rate.

Key words: Nephrotic syndrome | Renal biopsy | Methylprednisolone pulse therapy