{"orpha":98981,"name":"Essential iris atrophy","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":[],"definition":"A clinical variant of iridocorneal endothelial (ICE) syndrome, characterized by progressive iris atrophy and holes present on the surface of the iris, corneal edema, corectopia, uveal ectropion and anterior synechiae. Secondary glaucoma is also a common complication of the disease.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98981/","orphanet_url":"https://www.orpha.net/en/disease/detail/98981","names":{"fr":{"name":"Atrophie essentielle de l'iris","definition":"L'atrophie essentielle de l'iris est une forme de syndrome irido-cornéo-endothélial (ICE) caractérisée par une atrophie irienne progressive et par la présence de trous sur la surface de l'iris, un oedème cornéen, une corectopie, un ectropion de l'uvée et la formation de synéchies antérieures. Le glaucome secondaire complique fréquemment la maladie."},"de":{"name":"Irisatrophie, essentielle","definition":"Die Essentielle Irisatrophie ist eine klinische Variante des Irido-kornealen (ICE) Syndroms (s. dort). Kennzeichen sind progrediente Irisatrophie mit Löchern auf der Irisoberfläche, Hornhautödem, Korektopie, Uvea-Ektropion und vordere Synechie. Sekundäres Glaukom ist eine häufige Komplikation der Krankheit."},"es":{"name":"Atrofia esencial de iris","definition":"Es una variante clínica del síndrome endotelial iridocorneal (ICE), que se caracteriza por una atrofia progresiva del iris y agujeros presentes en la superficie del iris, edema corneal, corectopia, ectropión uveal y sinequias anteriores. El glaucoma secundario es también una complicación frecuente de esta enfermedad."},"it":{"name":"Atrofia essenziale dell'iride","definition":"L'atrofia essenziale dell'iride è una variante clinica della sindrome endoteliale iridocorneale (ICE; si veda questo termine), caratterizzata da atrofia progressiva dell'iride e buchi sulla sua superficie, edema corneale, corectopia, ectropion uveale e sinechie anteriori. Il glaucoma secondario è una complicazione comune della malattia."},"nl":{"name":"Essentiële irisatrofie","definition":"Een klinische variant van iridocorneaal endotheliaal (ICE) syndroom, gekarakteriseerd door progressieve irisatrofie en aanwezigheid van gaatjes op het oppervlak van de iris, corneaoedeem, corectopie, ectropion van uvea, en anterieure synechieën. Secundair glaucoom is ook een gangbare complicatie van de ziekte."},"pt":{"name":"Atrofia da íris, essencial"},"pl":{"name":"Zanik tęczówki"},"cs":{"name":"Esenciální atrofie duhovky"},"uk":{"name":"Есенціальна атрофія райдужної оболонки","definition":"A clinical variant of iridocorneal endothelial (ICE) syndrome, characterized by progressive iris atrophy and holes present on the surface of the iris, corneal edema, corectopia, uveal ectropion and anterior synechiae. Secondary glaucoma is also a common complication of the disease."},"zh":{"name":"特发性虹膜萎缩症","definition":"该病是虹膜角膜内皮(ICE)综合征的一种临床亚型，其特征是虹膜进行性萎缩，虹膜表面出现孔洞，角膜水肿，瞳孔异位，葡萄膜外翻和前粘连，继发性青光眼也是该病的常见并发症。"},"tr":{"name":"Esansiyel iris atrofisi"}},"xrefs":[{"source":"ICD-10","code":"H21.2","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"9A90.2","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1473835703"},{"source":"MONDO","code":"0020371","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0271111","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":64734,"name":"Iridocorneal endothelial syndrome","classifications":[147,156,189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}