{"orpha":2298,"name":"Insulin-resistance syndrome type B","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare genetic disease that belongs to the group of extreme insulin-resistance syndromes and is due to autoantibodies directed against insulin receptor.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2298/","orphanet_url":"https://www.orpha.net/en/disease/detail/2298","names":{"fr":{"name":"Syndrome d'insulino-résistance type B","definition":"Maladie génétique rare appartenant au groupe des syndromes d'insulinorésistance extrême due à la présence des auto-anticorps dirigés contre le récepteur de l'insuline."},"de":{"name":"Insulinresistenz-Syndrom Typ B","definition":"Eine seltene genetische Erkrankung, die zur Gruppe der extrem insulin-resistenten Syndrome gehört und auf Autoantikörper gegen den Insulinrezeptor zurückzuführen ist."},"es":{"name":"Síndrome de resistencia a la insulina tipo B","definition":"Es una enfermedad de base genética poco frecuente, perteneciente al grupo de síndromes de resistencia extrema a la insulina, debida a la presencia de autoanticuerpos dirigidos contra el receptor de insulina."},"it":{"name":"Sindrome da insulino-resistenza, tipo B","definition":"Si tratta di una malattia genetica rara che appartiene al gruppo delle sindromi da insulino-resistenza estrema. È causata da autoanticorpi diretti contro il recettore dell'insulina."},"nl":{"name":"Insulineresistentiesyndroom type B","definition":"Een zeldzame, genetische ziekte die behoort tot de groep van extreme insulineresistentiesyndromen en het gevolg is van auto-antilichamen gericht tegen insulinereceptor."},"pt":{"name":"Síndrome de resistência à insulina tipo B","definition":"Doença genética rara que pertence ao grupo das síndromes extremas de resistência à insulina e resulta da presença de autoanticorpos dirigidos contra o recetor de insulina."},"pl":{"name":"Zespół oporności na insulinę typu B"},"cs":{"name":"Syndrom inzulinové rezistence typ B"},"uk":{"name":"Синдром інсулінорезистентності, тип В","definition":"A rare genetic disease that belongs to the group of extreme insulin-resistance syndromes and is due to autoantibodies directed against insulin receptor."},"zh":{"name":"胰岛素抵抗综合征B型"},"tr":{"name":"İnsülin direnci sendromu tip B"}},"xrefs":[{"source":"ICD-10","code":"E34.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"5A44","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"408487090"},{"source":"MONDO","code":"0016464","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0342337","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"3009","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":181368,"name":"Rare insulin-resistance syndrome","classifications":[156,193]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class 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