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Article
March 1967

Alterations of Pigment Epithelium in Cystinosis

Author Affiliations

Bethesda, Md
From the Ophthalmology Branch, National Institute of Neurological Diseases and Blindness, National Institutes of Health, Public Health Service, US Department of Health, Education, and Welfare, and the Arthritis and Rheumatism Branch, National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethesda (Drs. Lietman and Seegmiller). Dr. Lietman is presently at the Johns Hopkins Hospital, Department of Pharmacology, Baltimore.

Arch Ophthalmol. 1967;77(3):361-369. doi:10.1001/archopht.1967.00980020363014
Abstract

A previously undescribed pigmentary disturbance of the peripheral fundus was seen in eleven patients with childhood cystinosis. Histologic observations in two of these children demonstrated that extensive degeneration and loss of retinal pigment epithelium was associated with the retinopathy. The peripheral fundus abnormalities may be of special diagnostic value since it can precede the classical corneal deposits of cystine crystals as suggested by the present study. The existence of this retinopathy appears to be peculiar to childhood cystinosis as it was found to be absent in the adult form of the disease.

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