Abstract
Gyrate Atrophy is an autosomal recessively inherited rare disease of the choroid and retina. Deficiency of ornithine aminotransferase enzyme (OAT) leads to 10 to 20 times ornithine levels in plasma which is thought to be the cause for the ocular findings. OAT gene is located on the tenth chromosome and when a mutation occurs OAT enzyme activity is decreased in the body. In the first decade, areas of chorioretinal atrophy are seen in the mid-peripheral retinal areas. In the progressing years, the macula may be involved and myopia is an accompanying feature]. Patients initially describe night vision problems and progressive visual field defects leading to central vision loss and blindness later on in the disease. Diagnosis is evident with clinical findings and elevated ornithine levels in plasma and is confirmed with OAT enzyme immune assay. Treatment is dietary modification and treatment of complications that may develop in the retina. GA is reported in diverse ethnic populations and in many sites around the World. Finland is the most reported country (1/50,000). It is very rare in Turkey.
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