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Scheie Syndrome and Macular Corneal DystrophyAn Ultrastructural Comparison of Conjunctiva and Skin
Harry A. Quigley;
Morton F. Goldberg, MD
Arch Ophthalmol. 1971;85(5):553-564.
Abstract
In the first electron microscope study of tissue from a patient with mucopolysaccharidosis type V (Scheie syndrome), multiple vacuolations were seen in fibroblasts from conjunctiva and skin and in conjunctival epithelial cells. Inclusions were single membrane-limited, contained a granulofibrillar material, and were associated with Golgi areas. Skin epithelium was normal. Skin and bulbar conjunctiva from the inferior fornix in four patients with macular corneal dystrophy were normal. In one macular dystrophy patient, a second conjunctival biopsy, taken 2 mm from the limbus, showed fibroblasts with dilated rough endoplasmic reticulum.
Author Affiliations
Baltimore; Chicago
From the Department of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore (Mr. Quigley), and the Department of Ophthalmology, University of Illinois Eye and Ear Infirmary, Chicago (Dr. Goldberg).
Footnotes
Submitted for publication Aug 3, 1970.
Read in part before the Section on Anatomy and Pathology at the Association for Research in Ophthalmology spring meeting, Sarasota, Fla, May 5, 1970, and in part before the 29th clinical meeting of the Wilmer Ophthalmological Institute Residents' Association, Baltimore, April 16, 1970.
Reprint requests to Woods 155, Wilmer Institute, Johns Hopkins Hospital, Baltimore 21205 (Mr. Quigley).
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