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
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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)
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