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岩藻糖苷贮积症:一名成年人眼部的超微结构研究

Fucosidosis: ultrastructural study of the eye in an adult.

作者信息

Hoshino M, O'Brien T P, McDonnell J M, de la Cruz Z C, Green W R

机构信息

Eye Pathology Laboratory, Wilmer Ophthalmological Institute, Johns Hopkins Medical Institutions, Baltimore, Md.

出版信息

Graefes Arch Clin Exp Ophthalmol. 1989;227(2):162-71. doi: 10.1007/BF02169791.

Abstract

The ocular histopathological and ultrastructural features of fucosidosis in a man who survived to the age of 25 years are reported. Virtually all of the cells of the eye contained cytoplasmic, membrane-bound, and confluent areas of fibrillogranular and multilaminated material. The most striking accumulations were present within the endothelial cells lining blood vessels and corneal endothelium, and the least amount was present in the uveal melanocytes.

摘要

报告了一名活到25岁的岩藻糖苷贮积症患者眼部的组织病理学和超微结构特征。几乎眼部所有细胞都含有细胞质内、膜结合的、融合的纤维颗粒状和多层状物质区域。最显著的蓄积物存在于血管内皮细胞和角膜内皮细胞内,而葡萄膜黑素细胞中的含量最少。

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