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Hyperornithinaemia Associated with Gyrate Atrophy of the Choroid and Retina: Two Cases from India

Hypcrornithinaemia associated with gyrate atrophy of the choroid and retina is a rare, autosomal recessive disorder resulting from a deficiency of the mitochondrial matrix enzyme ornithine c5-aminotransferase. This enzyme catalyses the pyridoxal phosphate-dependent transamination of ornithine and c-ketoglutarate to ~'-pyrroline 5-carboxylic acid and glutamic acid. Over 150 biochemically documented cases have been reported, of which one-third are Finnish.' There have been no published reports of the association of hyperornithinaemia with gyrate atrophy of the choroid and retina from India. We report two cases with this disorder, one of which demonstrated a biochemical response to moderate pharmacological doses of pyridoxine.