{"orpha":353351,"name":"Idiopathic macular telangiectasia type 3","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Occlusive idiopathic juxtafoveolar retinal telangiectasis"],"definition":"Idiopathic macular telangiectasia type 3 is a rare, acquired, eye disease characterized by progressive visual loss, due to bilateral juxtafoveolar capillary occlusions, capillary telangiectasia, and minimal exudation. It is associated with systemic or cerebral vascular occlusive disease.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-353351/","orphanet_url":"https://www.orpha.net/en/disease/detail/353351","names":{"fr":{"name":"Télangiectasie maculaire idiopathique type 3","synonyms":["Télangiectasie rétinienne juxtafovéolaire idiopathique occlusive"],"definition":"Maladie oculaire rare et acquise caractérisée par une perte progressive de vision, due à des occlusions bilatérales des capillaires juxtafovéolaires, à des télangiectasies capillaires et à une exsudation minime. Elle est associée à une maladie vasculaire occlusive systémique ou cérébrale."},"de":{"name":"Idiopathische makuläre Teleangiektasie Typ 3","synonyms":["Occlusive idiopathic juxtafoveolar retinal telangiectasis"]},"es":{"name":"Telangiectasia macular idiopática tipo 3","synonyms":["Telangiectasia retiniana oclusiva yuxtafoveolar idiopática"],"definition":"La telangiectasia macular idiopática tipo 3 es una enfermedad ocular adquirida y poco frecuente caracterizada por pérdida visual progresiva debida a oclusiones capilares yuxtafoveolares bilaterales, telangiectasia capilar y exudación mínima. Se asocia a enfermedad oclusiva vascular cerebral o sistémica."},"it":{"name":"Telangectasia maculare idiopatica, tipo 3","synonyms":["Telangectasia retinica iuxtafoveale idiopatica occlusiva"],"definition":"La telangectasia maculare idiopatica tipo 3 è una malattia acquisita rara dell'occhio, caratterizzata dalla perdita progressiva della vista, causata da occlusioni bilaterali dei capillari in prossimità della fovea, telangectasia dei capillari e minima essudazione. Si associa all'occlusione sistemica dei vasi o solo di quelli cerebrali."},"nl":{"name":"Idiopathische maculaire teleangiëctasie type 3","synonyms":["Occlusieve idiopathische juxtafoveolaire retinale teleangiëctasie"],"definition":"Idiopathische maculaire teleangiëctasie type 3 is een zeldzame, verworven oogziekte die gekarakteriseerd wordt door progressief verlies van gezichtsvermogen als gevolg van bilaterale juxtafoveolaire capillaire occlusies, capillaire teleangiëctasie, en minimale exsudatie. Het is geassocieerd met systemische of cerebrale vasculaire occlusieve ziekte."},"pt":{"name":"Telangiectasia idiopática macular, tipo 3"},"pl":{"name":"Idiopatyczna teleangiektazja plamki typu 3","synonyms":["Zamykająca idiopatyczna teleangiektazja okołodołeczkowa siatkówki"],"definition":"*Idiopatyczna teleangiektazja plamki typu 3 jest rzadką, nabytą chorobą oczu, która charakteryzuje się postępującą utratą wzroku spowodowaną obustronnym zamknięciem naczyń włosowatych wokół dołka środkowego siatkówki oka, teleangiektazją naczyń włosowatych i minimalnym wysiękiem. Towarzyszy choroba zarostowa naczyń układowych lub mózgowych."},"cs":{"name":"Idiopatická makulární teleangiektázie, typ 3","synonyms":["Okluzivní idiopatické juxtafoveolární releangiektázie sítnice"]},"uk":{"name":"Ідіопатична макулярна телеангіектазія, тип 3","synonyms":["Оклюзійний ідіопатичний юкстафовеолярний телеангіоектаз сітківки"],"definition":"Idiopathic macular telangiectasia type 3 is a rare, acquired, eye disease characterized by progressive visual loss, due to bilateral juxtafoveolar capillary occlusions, capillary telangiectasia, and minimal exudation. It is associated with systemic or cerebral vascular occlusive disease."},"zh":{"name":"特发性黄斑毛细血管扩张症3型","synonyms":["阻塞性特发性黄斑旁视网膜毛细血管扩张症"],"definition":"特发性黄斑毛细血管扩张症3型是一种罕见的获得性眼病，其特征是进行性视力丧失，原因是双侧黄斑中心凹旁毛细血管闭塞、毛细血管扩张和少量渗出。本病与全身或脑血管闭塞性疾病有关。"},"tr":{"name":"İdiyopatik maküler telenjiektazi tip 3","synonyms":["Oklüzif idiyopatik jukstafoveolar retinal telenjiektazi"]}},"xrefs":[{"source":"ICD-10","code":"H35.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9B75.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018147","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4751436","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":482092,"name":"Rare idiopathic macular telangiectasia","classifications":[181,189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":9.0,"area":"Worldwide","status":"Validated","source":"22219035[PMID]_ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"22219035[PMID]_ORPHANET"}],"phenotypes":null}