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肾淀粉样变性中肾病综合征病因的研究。关于电子显微镜检查结果的讨论。

Investigations on the cause of the nephrotic syndrome in renal amyloidosis. A discussion of electron microscopic findings.

作者信息

Gise H, Mikeler E, Gruber M, Christ H, Bohle A

出版信息

Virchows Arch A Pathol Anat Histol. 1978 Aug 22;379(2):131-41. doi: 10.1007/BF00432482.

Abstract

Systematic electron microscopic investigation of glomeruli of 35 patients with renal amyloidosis (grade I-III), among them 26 with nephrotic syndrome, reveals the following: 1. The extent of the area of basement membrane denuded of its epithelial covering is correlated significantly with the reduction of plasma protein concentration at the time of renal biopsy. 2. In amyloid free regions of the glomerular capillary loops, the foot processes of the epithelial cells remain intact despite the presence of the nephrotic syndrome. From these findings we conclude that the high glomerular protein losses in amyloidosis occur in areas of the basement membrane which are penetrated by amyloid and denuded of their epithelial covering. With increasing number of these lesions per unit area, the permeability of the capillary network for protein increases to a degree which is significantly correlated with the reduced plasma protein concentration at the time of biopsy.

摘要

对35例肾淀粉样变性(I - III级)患者的肾小球进行系统电子显微镜检查,其中26例患有肾病综合征,结果如下:1. 基底膜上皮覆盖物剥脱区域的范围与肾活检时血浆蛋白浓度的降低显著相关。2. 在肾小球毛细血管袢无淀粉样物质的区域,尽管存在肾病综合征,上皮细胞的足突仍保持完整。从这些发现我们得出结论,淀粉样变性中高肾小球蛋白丢失发生在基底膜被淀粉样物质穿透并剥脱上皮覆盖物的区域。随着单位面积内这些病变数量的增加,毛细血管网络对蛋白质的通透性增加到与活检时血浆蛋白浓度降低显著相关的程度。

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