{"orpha":488239,"name":"Acute macular neuroretinopathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["AMNR"],"definition":"A rare, acquired retinal disorder characterised by transient or permanent visual impairment accompanied by the presence of reddish-brown, wedge-shaped lesions in the macula, the apices of which tend to point towards the fovea. The lesions usually appear in a petaloid or tear-drop configuration. Patients tend to be young, Caucasian, and female.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-488239/","orphanet_url":"https://www.orpha.net/en/disease/detail/488239","names":{"fr":{"name":"Neurorétinopathie aiguë maculaire","synonyms":["AMNR"],"definition":"Maladie rétinienne rare et acquise, caractérisée par une déficience visuelle transitoire ou permanente accompagnée de lésions arciformes de couleur rouge-brunâtre dans la macula, dont les extrémités vont progressivement s'étendre vers la fovéa. Les lésions sont généralement disposées en forme de pétales. Les patients sont majoritairement des femmes, jeunes, de type caucasien."},"de":{"name":"Neuroretinopathie, akute makuläre","synonyms":["AMNR"],"definition":"Eine seltene erworbene Netzhauterkrankung, die durch eine vorübergehende oder dauerhafte Sehbehinderung gekennzeichnet ist, die mit rötlich-braunen, keilförmigen Läsionen in der Makula einhergeht, deren Spitzen zur Fovea zeigen. Die Läsionen haben gewöhnlich eine petaloid- oder tropfenförmige Gestalt. Die Patienten sind in der Regel jung, weiblich und gehören der weißen Bevölkerung an."},"es":{"name":"Neurorretinopatía macular aguda","synonyms":["AMNR"],"definition":"Es un trastorno retiniano adquirido poco frecuente que se caracteriza por discapacidad visual transitoria o permanente acompañada de la presencia de lesiones maculares de color marrón rojizo en forma de cuña cuyos ápices tienden a apuntar hacia la fóvea. Las lesiones suelen adoptar una configuración petaloide o en forma de lágrima. Los pacientes tienden a ser mujeres jóvenes, y de etnia caucásica."},"it":{"name":"Neuroretinopatia maculare acuta","definition":"Si tratta di una retinopatia acquisita rara, caratterizzata da una compromissione transitoria o permanente della vista, associata alla presenza di lesioni cuneiformi rossastre-marroni sulla macula, i cui apici sono orientati verso la fovea. In genere la disposizione delle lesioni ricorda la forma di un petalo o di una lacrima. La malattia colpisce soprattutto le giovani donne caucasiche."},"nl":{"name":"Acute maculaire neuroretinopathie","synonyms":["AMNR"],"definition":"Een zeldzame, verworven aandoening van retina, gekarakteriseerd door transiënt of permanent verlies van gezichtsvermogen, vergezeld van aanwezigheid van rood-bruine, wigvormige laesies in macula, waarvan de apices doorgaans in de richting van fovea wijzen. De laesies verschijnen meestal in een petaloïde of traanvormige configuratie. Patiënten zijn eerder jong, Kaukasisch, en vrouwelijk."},"pt":{"name":"Neurorretinopatia macular aguda"},"pl":{"name":"Ostra neuroretinopatia plamki żółtej","synonyms":["AMNR"]},"cs":{"name":"Akutní makulární neuroretinopatie","synonyms":["AMNR"]},"uk":{"name":"Гостра макулярна нейроретинопатія","synonyms":["AMNR"],"definition":"A rare, acquired retinal disorder characterised by transient or permanent visual impairment accompanied by the presence of reddish-brown, wedge-shaped lesions in the macula, the apices of which tend to point towards the fovea. The lesions usually appear in a petalloid or tear-drop configuration. Patients tend to be young, Caucasian, and female."},"zh":{"name":"急性黄斑神经视网膜病变","synonyms":["AMNR"],"definition":"一种罕见的获得性视网膜疾病，其特征是短暂或永久性的视力障碍，伴有黄斑部的赤褐色楔形病变，其尖端往往指向凹陷。病变通常呈花瓣状或泪滴状。患者多为年轻的高加索女性。"},"tr":{"name":"Akut maküler nöroretinopati","synonyms":["AMNR"]}},"xrefs":[{"source":"ICD-10","code":"H35.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9B65.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1081365759"},{"source":"MONDO","code":"0044627","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5200735","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10086444","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":716419,"name":"Rare isolated non-progressive predominantly macular disorder","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":101.0,"area":"Worldwide","status":"Validated","source":"26973287[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_26973287[PMID]"}],"phenotypes":null}