Evolution of Cellular Inclusions in Bietti’s Crystalline Dystrophy
Emiko Furusato1,2, J. Douglas Cameron2 and Chi-Chao Chan1
1Immunopathology Section, Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, MD. 2Neuropathology and Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC.
Abstract
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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