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肾病综合征及肾功能不全与淀粉样变性的关联:结构与功能之间的相关性

Nephrotic syndrome and renal insufficiency in association with amyloidosis: a correlation between structure and function.

作者信息

von Gise H, von Gise V, Stark B, Bohle A

出版信息

Klin Wochenschr. 1981 Jan 15;59(2):75-82. doi: 10.1007/BF01477286.

Abstract

The following results were obtained by correlating light and electron microscopic findings from 85 cases of glomerular renal amyloidosis with clinical parameters: 1. Amyloid masses deposited in the glomeruli do not represent an effective filtration barrier, i.e., the formation of primary urine is not significantly influenced even by extensive amyloid masses in the glomeruli; protein retention is first observed when the glomerular capillaries are almost totally obliterated. 2. Once the nephrotic syndrome has developed in association with glomerular renal amyloidosis, it shows no tendency for remittance, despite progressing renal insufficiency. 3. The reason for this persistance of the nephrotic syndrome, despite increasing renal insufficiency, is a progressive reduction in the capacity of the tubules for protein reabsorption in the presence of increasing interstitial fibrosis of the kidney. 4. Interstitial fibrosis of the kidney cortex leads to increasing impairment in the oxygen and energy supply to the tubule cells together with considerable functional deterioration which, in addition to other metabolic disturbances, also results in a reduced capacity for protein reabsorption.

摘要

通过将85例肾小球肾淀粉样变性的光镜和电镜检查结果与临床参数进行关联,得出以下结果:1. 沉积在肾小球中的淀粉样物质团块并不构成有效的滤过屏障,即即使肾小球中有大量淀粉样物质团块,原尿的形成也不会受到显著影响;当肾小球毛细血管几乎完全闭塞时,才首次观察到蛋白质潴留。2. 一旦肾病综合征与肾小球肾淀粉样变性相关联而发展,尽管肾功能不全不断进展,但它并无缓解的趋势。3. 尽管肾功能不全不断加重,但肾病综合征持续存在的原因是,在肾脏间质纤维化不断加重的情况下,肾小管对蛋白质的重吸收能力逐渐降低。4. 肾皮质的间质纤维化导致肾小管细胞的氧气和能量供应受损加剧,同时功能严重恶化,这除了其他代谢紊乱外,还导致蛋白质重吸收能力降低。

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