{"orpha":101988,"name":"Primary immunodeficiency due to a defect in innate 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-101988/","orphanet_url":"https://www.orpha.net/en/disease/detail/101988","names":{"fr":{"name":"Déficit immunitaire primaire par défaut de l'immunité innée","synonyms":["Immunodéficience primaire par défaut de l'immunité innée"]},"de":{"name":"Primärer Immundefekt durch Anomalie des angeborenen Immunsystems"},"es":{"name":"Inmunodeficiencia primaria por un defecto en la inmunidad innata"},"it":{"name":"Immunodeficienza primitiva da difetto dell'immunità innata"},"nl":{"name":"Primaire immuundeficiëntie door defect van aangeboren immuniteit"},"pt":{"name":"Imunodeficiência primária devida a anomalia da imunidade inata"},"pl":{"name":"Pierwotny Niedobór odporności z powodu defektu odporności wrodzonej"},"cs":{"name":"Primární imunodeficience způsobená defektem přirozené imunity"},"uk":{"name":"Первинний імунодефіцит внаслідок дефекту вродженого імунітету"},"zh":{"name":"先天性免疫缺陷所致原发性免疫缺陷"},"tr":{"name":"Doğumsal bağışıklıktaki bir defekte bağlı primer immün yetmezlik"}},"xrefs":[{"source":"ICD-11","code":"4A00","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"551037838"},{"source":"MONDO","code":"15135","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681778","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19810","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98004,"name":"Rare immune disease"},"parents":[{"orpha":101997,"name":"Primary immunodeficiency","classifications":[156,195]}],"children":[{"orpha":290839,"name":"Autoinflammatory syndrome with immune deficiency","type":"Category"},{"orpha":183710,"name":"Genetic susceptibility to infections due to particular pathogens","type":"Category"},{"orpha":101992,"name":"Immunodeficiency due to a complement cascade protein anomaly","type":"Category"},{"orpha":331193,"name":"Other immunodeficiency syndromes due to defects in innate immunity","type":"Category"},{"orpha":431156,"name":"Primary immunodeficiency with predisposition to severe viral infection","type":"Category"},{"orpha":101985,"name":"Quantitative and/or qualitative congenital phagocyte defect","type":"Category"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}