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gyrate atrophy of choroid and retina


Definition: a slowly progressive atrophy of the choriocapillaris, pigmentary epithelium, and sensory retina, with irregular confluent atrophic areas and an associated ornithinuria; autosomal recessive inheritance; due to a deficiency of ornithine ΄-aminotransferase, caused by mutation in the ornithine ΄-aminotransferase gene (OAT) on chromosome 10q.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.