ABSTRACT
Hyperornithinemia 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 d-aminotransferase [OAT]. Highest rate of gyrate atrophy is reported in Finland. We report for the first time 3 cases [siblings] in one Omani family in the Gulf, presenting with the classical clinical features and biochemical abnormality of this condition. The available forms of treatments which include treatment with pyridoxine, dietary restriction of arginine and supplementation with creatinine andproline, are known to slow the progress of disease process and help to delay the patient s morbidity, but no form of therapy is found to be unequivocally effective