Clinical trials of vitamin B6 and proline supplementation for gyrate atrophy of the choroid and retina.

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Clinical trials of vitamin B6 and proline supplementation for gyrate atrophy of the choroid and retina.

Five patients with gyrate atrophy of the choroid and retina were examined ophthalmologically, especially ophthalmoscopically, to evaluate trials of vitamin B6 (pyridoxine) or supplementary proline. The oral administration of vitamin B6 was tried in two patients. The vitamin did not alter the serum ornithine level and the progression of chorioretinal atrophy in one patient (case 2). Despite a re...

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Gyrate Atrophy of the Choroid and Retina: A Case Report

Introduction Gyrate atrophy of the choroid and retina is a metabolic disorder, which is inherited in an autosomal recessive pattern. Although gyrate atrophy is rare, it is concerning as it results in blindness. It is characterized by hyperornithinemia, retinal atrophy, leads to progressive myopia and tunnel vision,...

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Gyrate atrophy of the choroid and retina with hyper-ornithinemia responsive to vitamin B6: a case report

BACKGROUND Gyrate atrophy of the retina and choroid is a rare autosomal recessive inherited disease, characterized by progressive chorioretinal atrophy that results in progressive deterioration of peripheral and night vision and leading to blindness. CASE PRESENTATION This report presents a case of a 28-year-old man consulting for a progressive fall of visual acuity with hemeralopia. Eye fund...

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Gyrate atrophy of the choroid and retina associated with hyperornithinaemia.

The inheritance of the autosomal recessive type of gyrate atrophy of the choroid and retina seems well documented (Botermans, I 972), but the aetiology of the disease is still unknown. Animal experiments and some disturbances of lipid, carbohydrate, and protein metabolism connected with chorio-retinal degenerations in man support the possibility of an enzymatic disorder as an aetiological facto...

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Gyrate atrophy of the choroid and retina in a cat.

An adult male, domestic short-haired cat with generalized retinal atrophy was found to have a 60-fold increase in plasma ornithine and ornithinuria. Ornithine-8-aminotransferase activity was undetectable in its tissues and in its cultured skin fibroblasts. This feline condition is thus analogous to gyrate atrophy of the choroid and retina in humans.

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ژورنال

عنوان ژورنال: British Journal of Ophthalmology

سال: 1985

ISSN: 0007-1161

DOI: 10.1136/bjo.69.4.283