Bietti结晶性营养不良中细胞包涵体的演化

E. Furusato, J. D. Cameron, C. Chan
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引用次数: 0

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。
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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