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 |
|
|
Share :
|
ABSTRACT |
Purpose : To determine the histological findings and treatment outcome in cases of child hood nephrotic syndrome which required renal biopsy. Methods : We retrospectively reviewed the clinical, laboratory, pathologic findings and therapeutic outcomes of 159 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. (J Korean Soc Pediatr Nephrol 2005;9:149-158) |
Key words:
Nephrotic syndrome | Renal biopsy | Methylprednisolone pulse therapy |
|