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威尔逊病中的凯泽-弗莱舍尔环及相关白内障。

Kayser-Fleischer ring and associated cataract in Wilson's disease.

作者信息

Tso M O, Fine B S, Thorpe H E

出版信息

Am J Ophthalmol. 1975 Mar;79(3):479-88. doi: 10.1016/0002-9394(75)90625-x.

Abstract

A 22-year-old woman with hepatolenticular degeneration, or Wilson's disease, was note clinically to have Kayser-Fleischer rings and associated cataracts. Histopathologic, histochemical, and electron microscopic study of the cornea showed deposition of copper in the peripheral Descemet's membrane, with excessive accumulation in Hassall-Henel warts. Copper deposits were also noted in the anterior and posterior lens capsule without the presence of degenerative changes in the epithelial or cortical cells of the lens. We propose that cellular activity is required for the depostion of copper material into the thick basement membranes of the cornea and lens, in contrast to the concept of simple diffusion.

摘要

一名22岁患有肝豆状核变性(即威尔逊氏病)的女性,临床检查发现有凯泽-弗莱舍尔环及相关白内障。对角膜进行的组织病理学、组织化学和电子显微镜研究显示,铜沉积于周边的后弹力层,在哈索尔-亨勒疣中有过多积聚。在前、后晶状体囊膜中也发现了铜沉积,而晶状体上皮或皮质细胞未出现退行性改变。我们提出,与简单扩散的概念相反,铜物质沉积到角膜和晶状体的厚基底膜需要细胞活动。

相似文献

3
Kayser-Fleischer ring. A pathological study.凯泽-弗莱舍尔环。一项病理学研究。
Br J Ophthalmol. 1970 Dec;54(12):794-800. doi: 10.1136/bjo.54.12.794.
4
Ultrastructure of the Kayser-Fleischer ring.凯-弗环的超微结构。
Am J Ophthalmol. 1973 Oct;76(4):455-61. doi: 10.1016/0002-9394(73)90730-7.
5
The Kayser Fleischer ring.凯泽-弗莱舍尔环。
Trans Ophthalmol Soc U K (1962). 1970;90:191-3.

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