Evolution of Cellular Inclusions in Bietti&rsquo;s Crystalline Dystrophy | Zendy

Chi-Chao Chan | Zendy; Furusato | Zendy; Cameron | Zendy

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Evolution of Cellular Inclusions in Bietti’s Crystalline Dystrophy

Author(s) -

Chi-Chao Chan,

Furusato,

Cameron

Publication year - 2010

Publication title -

ophthalmology and eye diseases

Language(s) - English

Resource type - Journals

ISSN - 1179-1721

DOI - 10.4137/oed.s2821

Subject(s) - dystrophy , retinal pigment epithelium , ultrastructure , degeneration (medical) , atrophy , posterior pole , cornea , choroid , biology , anatomy , melanosome , ophthalmology , retina , retinal , medicine , genetics , melanin , neuroscience

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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