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Gyrate atrophy of the choroid and retina. Early findings
Authors:M I Kaiser-Kupfer  I H Ludwig  F M de Monasterio  D Valle  I Krieger
Affiliation:2. Howard Hughes Medical Institute, Laboratory and Department of Pediatrics, The Johns Hopkins School of Medicine, Baltimore, Maryland;3. The Childrens Hospital of Michigan, Wayne State University School of Medicine, Department of Pediatrics, Detroit, Michigan
Abstract:Examination of two sisters ages 2 years 10 months and 6 years four months with gyrate atrophy of the choroid and retina provided an opportunity for detailed clinical investigation. Although the chorioretinal lesions were confined to the peripheral retina in the older case and were quite minimal in the younger case, there was electroretinographic evidence of marked involvement of the cone and rod systems. These cases offer an opportunity to assess an arginine restricted diet in preventing the progress of the disease.
Keywords:early findings  gyrate atrophy of the choroid and retina  psychophysical and electrodiagnostic testing
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