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 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.
D000293:Adolescent; D000328:Adult; D001120:Arginine / Q000008:administration & dosage*; D001284:Atrophy / Q000178:diet therapy / Q000201:enzymology; D002829:Choroid / Q000473:pathology*; D003623:Dark Adaptation; D006801:Humans; D008297:Male; D009952:Ornithine / Q000097:blood; D009953:Ornithine-Oxo-Acid Transaminase / Q000172:deficiency; D012160:Retina / Q000473:pathology*; D014792:Visual Acuity; D014794:Visual Fields
D001120:Arginine; D009952:Ornithine; D009953:Ornithine-Oxo-Acid Transaminase