Treatment of gyrate atrophy of the choroid and retina with low arginine diet

In gyrate atrophy the blood ornithine is grossly elevated, due to deficiency of ornithine ketoacid transaminase, which converts ornithine towards glutamic acid. Two patients with gyrate atrophy have been treated with a low arginine diet and their blood ornithine levels have been reduced to near norm...

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Bibliographic Details
Published in:Transactions of the American Ophthalmological Society 1980, Vol.78, p.226-242
Main Authors: McInnes, R R, Arshinoff, S A, Bell, L, McCulloch, C
Format: Article
Language:English
Subjects:
Adolescent
Adult
Arginine - administration & dosage
Atrophy - diet therapy
Atrophy - enzymology
Choroid - pathology
Dark Adaptation
Humans
Male
Ornithine - blood
Ornithine-Oxo-Acid Transaminase - deficiency
Retina - pathology
Visual Acuity
Visual Fields
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Summary:In gyrate atrophy the blood ornithine is grossly elevated, due to deficiency of ornithine ketoacid transaminase, which converts ornithine towards glutamic acid. Two patients with gyrate atrophy have been treated with a low arginine diet and their blood ornithine levels have been reduced to near normal. At this level hyperammonemia may result from overtreatment, but this can be quickly cleared by a small dose of arginine. There has also been some improvement in vision, but no clearing of the gyrate areas. Future care with this regimen seems possible and improvements in handling of these patients are likely.
ISSN:0065-9533
1545-6110