{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T13:46:09Z","timestamp":1791812769384,"version":"4.3.4"},"reference-count":9,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"Suppl 3","license":[{"start":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T00:00:00Z","timestamp":1786665600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Gyrate atrophy is a rare autosomal recessive chorioretinal dystrophy caused by ornithine aminotransferase deficiency, resulting in toxic accumulation of plasma ornithine and progressive retinal degeneration. We describe a 20-year-old male, born to consanguineous parents, who presented with progressive diminution of vision, nyctalopia, and peripheral visual field loss. Fundus examination revealed sharply demarcated, scalloped areas of midperipheral chorioretinal atrophy with relative macular sparing. Optical coherence tomography demonstrated cystoid macular edema, whereas plasma ornithine levels were markedly elevated. The patient was treated with dietary protein restriction and pyridoxine supplementation, with biochemical improvement and stabilization of visual function. Early recognition, metabolic monitoring, and evaluation of pyridoxine responsiveness may delay visual deterioration and improve visual prognosis in gyrate atrophy.<\/jats:p>","DOI":"10.4103\/aam.aam_218_26","type":"journal-article","created":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T14:09:19Z","timestamp":1786630159000},"page":"S494-S497","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Case report on Vitamin B6-Responsive Gyrate atrophy with Cystoid Macular Edema in a Consanguineous Young Male: A Treatable Metabolic Retinopathy"],"prefix":"10.4103","volume":"25","author":[{"given":"Rohan","family":"Jagadish","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nilesh","family":"Giri","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gautami Ramesh","family":"Laturkar","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Soumya","family":"Grover","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jay Kumar","family":"Trapasiya","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2026,8,14]]},"reference":[{"key":"R1-20261012","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1136\/bjo.58.1.3","article-title":"Gyrate atrophy of the choroid and retina associated with hyperornithinaemia","volume":"58","author":"Takki","year":"1974","journal-title":"Br J Ophthalmol"},{"key":"R2-20261012","first-page":"3","article-title":"Gyrate atrophy of the choroid and retina","volume":"11","author":"Weleber","year":"1990","journal-title":"Ophthalmic Genet"},{"key":"R3-20261012","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1016\/S0161-6420(81)35035-0","article-title":"Clinical trial of vitamin B6 for gyrate atrophy of the choroid and retina","volume":"88","author":"Weleber","year":"1981","journal-title":"Ophthalmol"},{"key":"R4-20261012","first-page":"112","article-title":"Pyridoxine-dependent reduction in plasma ornithine in gyrate atrophy of the choroid and retina","volume":"1","author":"Gibberd","year":"1985","journal-title":"Lancet"},{"key":"R5-20261012","first-page":"325","article-title":"Gyrate atrophy of the choroid and retina","volume":"27","author":"Ohba","year":"1982","journal-title":"Surv Ophthalmol"},{"key":"R7-20261012","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1001\/archopht.1991.01080110075039","article-title":"Gyrate atrophy of the choroid and retina: Long-term reduction of ornithine slows retinal degeneration","volume":"109","author":"Kaiser-Kupfer","year":"1991","journal-title":"Arch Ophthalmol"},{"key":"R8-20261012","first-page":"184","article-title":"Ocular findings and ornithine aminotransferase mutations in gyrate atrophy","volume":"39","author":"Ayyagari","year":"1998","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"R9-20261012","first-page":"1099","article-title":"Macular lesions and visual function in gyrate atrophy","volume":"99","author":"Kaiser-Kupfer","year":"1981","journal-title":"Arch Ophthalmol"},{"key":"R10-20261012","first-page":"181","article-title":"Gyrate atrophy of the choroid and retina","volume":"13","author":"Weleber","year":"1992","journal-title":"Ophthalmic Genet"}],"container-title":["Annals of African Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/aam.aam_218_26","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T13:01:38Z","timestamp":1791810098000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/aam.aam_218_26"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,14]]},"references-count":9,"journal-issue":{"issue":"Suppl 3","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.4103\/aam.aam_218_26","relation":{},"ISSN":["1596-3519","0975-5764"],"issn-type":[{"value":"1596-3519","type":"print"},{"value":"0975-5764","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,14]]},"assertion":[{"value":"2026-03-01","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-12","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}