Turkish Abstract Abstract Article PDF Similar Articles

Gazi Üniversitesi Tıp Fakültesi Göz Hastalıkları Anabilim dalı Gyrate atrophy is an autosomal recessive chorioretinal dystrophy associated with hyperornithinemia and hyperornithinuria, secondary to the deficiency of ornithine aminotransferase activity. Low night vision, high myopia and peripheral visual field limitation are the earliest findings which occur in late childhood and usually shows slow progression. Macular involvement or severe vision field changes may cause loss of vision by the 5th or 6th decades. The main purpose in the treatment is to decrease the plasma ornithine level. Decreases in plasma ornithine levels slowed down or stopped the progression of retinal degeneration in some experimental models. In this study we described the clinical characteristics of gyrate atrophy and reviewed the results of different treatment modalities. Keywords : Gyrate atrophy, hyperornithinemia, clinical findings, treatment