Bietti结晶性营养不良中细胞包涵体的演化

Ophthalmology and eye diseases Pub Date : 2010-01-01 DOI:10.1177/117917211000200003

E. Furusato, J. D. Cameron, C. Chan

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摘要

Bietti结晶性营养不良(BCD)包括视网膜后极小的黄白色晶状体，视网膜色素上皮(RPE)萎缩的绒毡视网膜变性和脉络膜“硬化”;此外，在许多患者的角膜浅缘也发现了黄色晶体。BCD是一种常染色体隐性遗传性状(4q35-tel)，通常在生命的第三个十年发病。本文就BCD细胞晶体和脂质包涵体的超微结构作一综述。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Evolution of Cellular Inclusions in Bietti's Crystalline Dystrophy

Bietti's crystalline dystrophy (BCD) consists of small, yellow-white, glistening intraretinal crystals in the posterior pole, tapetoretinal degeneration with atrophy of the retinal pigment epithelium (RPE) and “sclerosis” of the choroid; in addition, sparking yellow crystals in the superficial marginal cornea are also found in many patients. BCD is inherited as an autosomal-recessive trait (4q35-tel) and usually has its onset in the third decade of life. This review focuses on the ultrastructure of cellular crystals and lipid inclusions of BCD.

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