{"orpha":179006,"name":"Primary immunodeficiency due to a defect in adaptive immunity","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":null,"note":"This term does not characterize a disease but a group of diseases. To learn about the diseases included under this term, you can consult the classifications.","url":"https://raremap.alftech.space/d/ORPHA-179006/","orphanet_url":"https://www.orpha.net/en/disease/detail/179006","names":{"fr":{"name":"Déficit immunitaire primaire par défaut de l'immunité adaptative","synonyms":["Immunodéficience primaire par défaut de l'immunité adaptative"]},"de":{"name":"Primärer Immundefekt durch Störung der adaptiven Immunität"},"es":{"name":"Inmunodeficiencia primaria por defecto en la inmunidad adaptativa"},"it":{"name":"Immunodeficienza primitiva da difetto dell'immunità adattativa"},"nl":{"name":"Primaire immuundeficiëntie door defect van verworven immuniteit"},"pt":{"name":"Deficiência na imunidade adaptativa"},"pl":{"name":"Pierwotny Niedobór odporności z powodu defektu odporności nabytej"},"cs":{"name":"Primární imunodeficience způsobená poruchou specifické imunity"},"uk":{"name":"Первинний імунодефіцит внаслідок дефекту адаптивного імунітету"},"zh":{"name":"适应性免疫缺陷所致原发性免疫缺陷"},"tr":{"name":"Adaptif bağışıklıktaki bir defekte bağlı primer immün yetmezlik"}},"xrefs":[{"source":"ICD-11","code":"4A01","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1169765917"},{"source":"MONDO","code":"15823","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680501","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":2284,"name":"OBSOLETE: Primary T cell immunodeficiency","status":"inactive"}],"preferential_parent":{"orpha":98004,"name":"Rare immune disease"},"parents":[{"orpha":101997,"name":"Primary immunodeficiency","classifications":[156,195]}],"children":[{"orpha":101972,"name":"Combined T and B cell immunodeficiency","type":"Clinical group"},{"orpha":169361,"name":"Immune dysregulation disease with immunodeficiency","type":"Category"},{"orpha":101977,"name":"Immunodeficiency due to predominantly antibodies defect","type":"Category"},{"orpha":331217,"name":"Syndrome with combined immunodeficiency","type":"Category"},{"orpha":83471,"name":"T-cell immunodeficiency with thymic aplasia","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}