{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T15:05:28Z","timestamp":1785942328381,"version":"3.56.0"},"reference-count":81,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2021,12,13]],"date-time":"2021-12-13T00:00:00Z","timestamp":1639353600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["European Journal of Ophthalmology"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:p>Gyrate atrophy (GA) of the choroid and retina is a rare autosomal recessive genetic condition characterized by elevation of the plasma level of the amino acid ornithine due to deficiency of the enzyme ornithine ketoacid aminotransferase. Accumulation of ornithine occurs in various body tissues but leads primarily to characteristic ophthalmic manifestations including myopia, cataract, progressive chorioretinal atrophy, and macular changes. Patients usually present with night blindness that starts in the first decade of life followed by visual field constriction and eventually diminution of the central visual acuity and blindness. The condition has been reported worldwide and its differential diagnosis is broad and includes choroideremia and retinitis pigmentosa. Treatment currently depends on life-long dietary modifications including restriction of the amino acid arginine in diet. This article describes in detail the pathogenesis, clinical features, multimodal imaging findings, and treatment options for GA of the choroid and retina and its complications.<\/jats:p>","DOI":"10.1177\/11206721211067333","type":"journal-article","created":{"date-parts":[[2021,12,13]],"date-time":"2021-12-13T07:04:59Z","timestamp":1639379099000},"page":"1314-1323","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":24,"title":["Gyrate Atrophy of the Choroid and Retina: A Review"],"prefix":"10.1177","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5148-316X","authenticated-orcid":false,"given":"Ayman G.","family":"Elnahry","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, Faculty of Medicine, Cairo University, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5738-5915","authenticated-orcid":false,"given":"Gehad A.","family":"Elnahry","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Faculty of Medicine, Cairo University, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2021,12,13]]},"reference":[{"key":"bibr1-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.58.1.3"},{"key":"bibr2-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(73)90667-3"},{"key":"bibr3-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9394(14)75948-3"},{"key":"bibr4-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/0888-7543(92)90258-T"},{"key":"bibr5-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(81)35031-3"},{"key":"bibr6-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1080\/13816810.2018.1536220"},{"key":"bibr7-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.70.8.612"},{"key":"bibr8-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1111\/j.1755-3768.1986.tb06937.x"},{"key":"bibr9-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.1991.01080110075039"},{"key":"bibr10-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(81)35035-0"},{"key":"bibr11-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(81)35033-7"},{"key":"bibr12-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.74.11.5159"},{"key":"bibr13-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1097\/00004397-200010000-00008"},{"key":"bibr14-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)50731-1"},{"key":"bibr15-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.97.3.1224"},{"key":"bibr16-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1038\/eye.1994.58"},{"key":"bibr17-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(81)35028-3"},{"key":"bibr18-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199804000-00002"},{"key":"bibr19-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9394(14)70397-6"},{"key":"bibr20-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1007\/s10633-012-9335-0"},{"key":"bibr21-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2016-217556"},{"key":"bibr22-11206721211067333","doi-asserted-by":"publisher","DOI":"10.4103\/0974-620X.184529"},{"key":"bibr23-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1136\/bjophthalmol-2018-312347"},{"key":"bibr24-11206721211067333","doi-asserted-by":"publisher","DOI":"10.2147\/OPTH.S64248"},{"key":"bibr25-11206721211067333","first-page":"513","volume":"24","author":"Angioi-Duprez K","year":"2001","journal-title":"J Fr Ophtalmol"},{"key":"bibr26-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1186\/s12886-018-0755-9"},{"key":"bibr27-11206721211067333","doi-asserted-by":"publisher","DOI":"10.3341\/kjo.2013.27.5.388"},{"key":"bibr28-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1590\/S0004-27492007000500024"},{"key":"bibr29-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1136\/bcr.04.2010.2951"},{"key":"bibr30-11206721211067333","doi-asserted-by":"publisher","DOI":"10.22336\/rjo.2018.37"},{"key":"bibr31-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0042-6989(98)00073-X"},{"key":"bibr32-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9394(80)90114-2"},{"key":"bibr33-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1172\/JCI118730"},{"key":"bibr34-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/0305-0491(87)90075-7"},{"key":"bibr35-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1007\/BF00964382"},{"key":"bibr36-11206721211067333","first-page":"1135","volume":"25","author":"Korte GE","year":"1984","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bibr37-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1172\/JCI109680"},{"key":"bibr38-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005602405349"},{"key":"bibr39-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.29.7.996"},{"key":"bibr40-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9394(14)76892-8"},{"key":"bibr41-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799162"},{"key":"bibr42-11206721211067333","first-page":"251","volume":"20","author":"Valle DL","year":"1981","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"bibr43-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajo.2004.12.083"},{"key":"bibr44-11206721211067333","doi-asserted-by":"publisher","DOI":"10.4103\/ijo.IJO_984_19"},{"key":"bibr45-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1007\/s10792-017-0579-2"},{"key":"bibr46-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.59.5.735"},{"key":"bibr47-11206721211067333","first-page":"344","volume":"44","author":"Kennaway NG","year":"1989","journal-title":"Am J Hum Genet"},{"key":"bibr48-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2008.12.010"},{"key":"bibr49-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1111\/j.1755-3768.1987.tb08499.x"},{"key":"bibr50-11206721211067333","doi-asserted-by":"publisher","DOI":"10.5301\/ejo.5000660"},{"key":"bibr51-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.1996.01100140495035"},{"key":"bibr52-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1007\/s10792-015-0085-3"},{"key":"bibr53-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.74.6.363"},{"key":"bibr54-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1007\/BF00139577"},{"key":"bibr55-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.120.2.146"},{"key":"bibr56-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2011.09.017"},{"key":"bibr57-11206721211067333","doi-asserted-by":"publisher","DOI":"10.3928\/01913913-20200813-01"},{"key":"bibr58-11206721211067333","first-page":"112067212110080","author":"Elnahry AG","journal-title":"Eur J Ophthalmol"},{"key":"bibr59-11206721211067333","doi-asserted-by":"publisher","DOI":"10.3928\/23258160-20190703-03"},{"key":"bibr60-11206721211067333","author":"Mansour AM","journal-title":"Eye (Lond)"},{"key":"bibr61-11206721211067333","doi-asserted-by":"publisher","DOI":"10.2147\/OPTH.S195564"},{"key":"bibr62-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(96)30590-3"},{"key":"bibr63-11206721211067333","doi-asserted-by":"publisher","DOI":"10.3892\/etm.2014.1648"},{"key":"bibr64-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1080\/02713683.2019.1606252"},{"key":"bibr65-11206721211067333","first-page":"226","volume":"78","author":"McInnes RR","year":"1980","journal-title":"Trans Am Ophthalmol Soc"},{"key":"bibr66-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1076\/opge.20.4.219.2271"},{"key":"bibr67-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1023\/B:BOLI.0000028779.29966.05"},{"key":"bibr68-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(87)33269-5"},{"key":"bibr69-11206721211067333","first-page":"616","volume":"56","author":"Michaud J","journal-title":"Am J Hum Genet"},{"key":"bibr70-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198104093041503"},{"key":"bibr71-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(85)34098-8"},{"key":"bibr72-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaapos.2017.12.016"},{"key":"bibr73-11206721211067333","author":"\u00c7avdarl\u0131 C","year":"2020","journal-title":"Arq Bras Oftalmol"},{"key":"bibr74-11206721211067333","doi-asserted-by":"publisher","DOI":"10.5301\/ejo.5001010"},{"key":"bibr75-11206721211067333","doi-asserted-by":"publisher","DOI":"10.22336\/rjo.2018.50"},{"key":"bibr76-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/j.jfo.2018.03.029"},{"key":"bibr77-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcrs.2003.10.033"},{"key":"bibr78-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1590\/S0004-27492012000100012"},{"key":"bibr79-11206721211067333","first-page":"170","volume":"29","author":"Takahashi O","year":"1985","journal-title":"Jpn J Ophthalmol"},{"key":"bibr80-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1111\/j.1444-0938.2005.tb06717.x"},{"key":"bibr81-11206721211067333","doi-asserted-by":"publisher","DOI":"10.1186\/s12886-021-01862-1"}],"container-title":["European Journal of Ophthalmology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/11206721211067333","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/11206721211067333","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/11206721211067333","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T19:45:30Z","timestamp":1777405530000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/11206721211067333"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12,13]]},"references-count":81,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2022,5]]}},"alternative-id":["10.1177\/11206721211067333"],"URL":"https:\/\/doi.org\/10.1177\/11206721211067333","relation":{},"ISSN":["1120-6721","1724-6016"],"issn-type":[{"value":"1120-6721","type":"print"},{"value":"1724-6016","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,12,13]]}}}