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頁籤選單縮合
題名 | Focal glomerulosclerosis manifested with nephrotic syndrome=以腎病症候群為表徵之局部腎絲球硬化症 |
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作者姓名(中文) | 曹永魁; | 書刊名 | 中華民國小兒科醫學會雜誌 |
卷期 | 30:6 民78.11-12 |
頁次 | 頁401-407 |
分類號 | 417.574 |
關鍵詞 | 腎病症候群; 局部腎絲球硬化症; |
語文 | 英文(English) |
中文摘要 | 在過去十年中經腎臟切片檢查證實爲局部腎絲球硬化的腎病症候群孩童23位,接受1至13.5年(4.7±4.0年)的長期追踪。其中20位爲男生,3位為女生;平均腎病發生年齡爲7.2±4.0歲。病理的檢查20位元爲局部區斷性腎絲球硬化,3位元爲局部球性硬化。高血壓及血尿在臨床上常見,糖尿偶而可見,但較常見於腎衰竭的病人。13位病童爲一開始對類固醇有反應者,另10位(包括3位女童)則在發病時就是類固醇抗性者。至本文發表,6位病童進入腎衰竭階段,其中有兩位爲末期腎臟病,另有一位死於敗血症;8位仍有持續性蛋白尿;9位則處於緩解狀態;3位元局部球性硬化的病童均未發生腎衰竭。和其他作者比較,此病之臨床緩解似乎稍微好一點,(但在發生腎衰竭方面並無明顯的差異)。除了糖尿以外,本研究沒有發現任何臨床或病理表徵可預測病童是否會發生腎衰竭。 |
英文摘要 | To assess the long-term outcome for nephrotic children with focal glomerulosclerosis, 23 patients were studied. Twenty were male and three female; the mean age at onset was 7.2±4.0 years. Twenty of the 23 children had focal segmental glomerulosclerosis, and the other 3 showed focal global sclerosis in renal biopsy specimens. Hypertension (11/23) and hematuria (9/23) were frequent clinical features. Glycosuria (4/23) was occasionally noted. Of the patients studied 13 were initial steroid responders and 10, steroid nonresponders. The mean duration of follow-up for the entire group was 4.7±4.0 years (ranging from I to13.5 years). From the total study group, 13% had renal deaths; 13% had decreased creatinine clearance, but not end-stage renal disease; 35% had persistent proteinuria; and 39% were in remission. None of the three patients with focal global sclerosis developed chronic renal failure. The data suggest that for children with focal glomerulosclerosis, clinical outcome is not too pessimistic. Except for glycosuria, no clinical or morphologic features were predictive of the development of chronic renal failure, in this study. |
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