Gyrate atrophy of the choroid and retina. Early findings

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 t...

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Published inOphthalmology (Rochester, Minn.) Vol. 92; no. 3; p. 394
Main Authors Kaiser-Kupfer, M I, Ludwig, I H, de Monasterio, F M, Valle, D, Krieger, I
Format Journal Article
LanguageEnglish
Published United States 01.03.1985
Subjects
Atrophy
Child
Child, Preschool
Choroid - pathology
Electrooculography
Electroretinography
Female
Fluorescein Angiography
Follow-Up Studies
Humans
Ornithine - blood
Ornithine-Oxo-Acid Transaminase - deficiency
Retina - pathology
Transaminases - deficiency
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Summary: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.
ISSN:0161-6420
DOI:10.1016/S0161-6420(85)34022-8