{"orpha":476102,"name":"OBSOLETE: Hereditary pediatric Behçet-like disease","type":"Disease","group":"Disorder","status":"inactive","flags":["Obsolete entity"],"synonyms":["OBSOLETE: Behçet-like disease due to HA20","OBSOLETE: Behçet-like disease due to haploinsufficiency of A20"],"definition":null,"note":"This entity has been obsoleted from the Orphanet nomenclature of rare diseases.<br/><br/>Instead, consider using Unclassified autoinflammatory syndrome","url":"https://raremap.alftech.space/d/ORPHA-476102/","orphanet_url":"https://www.orpha.net/en/disease/detail/476102","names":{"fr":{"name":"OBSOLETE : Maladie de Behçet-like héréditaire de l'enfant","synonyms":["OBSOLETE : Maladie de Behçet-like par haploinsuffisance de A20"]},"de":{"name":"OBSOLET: Behçet-ähnliche Krankheit des Kindes, hereditäre","synonyms":["OBSOLET: Behçet-ähnliche Krankheit durch A20-Haploinsuffizienz","OBSOLET: Behçet-ähnliche Krankheit durch HA20"]},"es":{"name":"OBSOLETO: Enfermedad pediátrica hereditaria similar a Behçet","synonyms":["OBSOLETO: Enfermedad similar a Behçet por HA20","OBSOLETO: Enfermedad similar a Behçet por haploinsuficiencia de A20"]},"it":{"name":"OBSOLETO: Malattia pediatrica ereditaria simil-Behçet","synonyms":["OBSOLETO: Malattia simil-Behçet da HA20","OBSOLETO: Malattia simil-Behçet da aploinsufficienza di A20","OBSOLETO: Malattia simil-Behçet da HA20","OBSOLETO: Malattia simil-Behçet da aploinsufficienza di A20"]},"nl":{"name":"OBSOLEET: Hereditaire pediatrische Behçet-achtige ziekte","synonyms":["OBSOLEET: Behçet-achtige ziekte door A20-haploinsufficiëntie","OBSOLEET: Behçet-achtige ziekte door HA20"]},"pt":{"name":"OBSOLETO: Doença pediátrica hereditária Behçet-like","synonyms":["OBSOLETO: Doença Behçet-like devido a HA20","OBSOLETO: Doença Behçet-like devido a haploinsuficiência A20"]},"pl":{"name":"NIEAKTUALNE: Dziedziczna choroba Behþeta u dzieci i młodzieży","synonyms":["NIEAKTUALNE: Choroba podobna do choroby Behçeta spowodowana HA20","NIEAKTUALNE: Choroba podobna do choroby Behçeta spowodowana haploinsuficjencją A20"]},"cs":{"name":"NEAKTUÁLNÍ: Dědičné dětské onemocnění podobné Behçetově nemoci","synonyms":["NEAKTUÁLNÍ: Behçet-like disease due to HA20","NEAKTUÁLNÍ: Behçet-like disease due to haploinsufficiency of A20","NEAKTUÁLNÍ: Behçet-like onemocnění způsobené HA20","NEAKTUÁLNÍ: Behçet-like onemocnění způsobené haploinsuficiencí A20"]},"uk":{"name":"ЗАСТАРІЛО: Спадкова дитяча Бехчет-подібна хвороба","synonyms":["ЗАСТАРІЛО: Бехчет-подібна хвороба, спричинена HA20","ЗАСТАРІЛО: Бехчет-подібна хвороба, спричинена гаплонедостатністю А20"]},"zh":{"name":"遗传性儿科Behpet样疾病","synonyms":["A20单倍剂量不足导致的Behcet样疾病"],"definition":"一种罕见的常染色体显性遗传的自身炎症综合征，其特点是早期全身性炎症伴有自身免疫表现，更罕见的是体液免疫缺陷和循环促炎细胞因子的产生增加，多数表现为反复发作的口腔口疮性溃疡、生殖器溃疡、关节痛或关节炎、周期性发热、视网膜炎和严重的胃肠道受累（疼痛、腹泻、呕吐、直肠出血）。"},"tr":{"name":"Kalıtsal pediatrik Behçet-benzeri hastalık","synonyms":["HA20'ye bağlı Behçet benzeri hastalık","A20'nin haplo yetersizliğine bağlı Behçet benzeri hastalık"]}},"xrefs":[],"associations":[{"type":"Referred to","orpha":324936,"name":"Unclassified autoinflammatory syndrome"}],"incoming_associations":[],"preferential_parent":null,"parents":[],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}