| angio-oedeem door verworven deficiëntie van C1-esteraseremmer (aandoening) | | angio-oedeem door verworven deficiëntie van C1-esteraseremmer | | verworven deficiëntie van C1-esteraseremmer
verworven angio-oedeem door deficiëntie van C1-esteraseremmer
|  | Dit betekent dat je huid of slijmvliezen opzwellen. Dat komt omdat je lichaam te weinig heeft van een stof die je lichaam helpt om vocht af te voeren. Dit probleem had je nog niet bij je geboorte, maar is later ontstaan. | | Acquired C1 esterase inhibitor deficiency | | Acquired angioneurotic edema with C1 inhibitor deficiency
Acquired angioneurotic edema with C1Inh (C1 esterase inhibitor) deficiency
Acquired angioedema with C1Inh (C1 esterase inhibitor) deficiency
Acquired angioedema
| | A rare non-histaminic angioedema characterized by potentially life-threatening episodes of edema of subcutaneous and/or mucosal tissues without urticaria, caused by excessive consumption of C1 esterase inhibitor (C1-INH) in the context of lymphoproliferative or autoimmune diseases. Patients typically present in the fourth decade of life or later and without a family history of angioedema. Clinical manifestation includes nonpitting edema of the skin predominantly involving the face, but also the limbs or genitals, as well as abdominal pain due to involvement of the gastrointestinal mucosa and severe edema of the upper airway and oral mucosa. Laboratory examination shows low C1-INH activity and low C3, C4, and C1q levels. Autoantibodies to C1-INH are frequently detectable. |
| | Id | 241955009 | | Status | Primitive |
| DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | D84.1 | | Term | Stoornissen in complementsysteem |
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| SNOMED CT to Orphanet simple map | 528663 |
| SNOMED CT to ICD-10 extended map | | Target | D84.1 | | Rule | TRUE | | Advice | ALWAYS D84.1 | | Correlation | SNOMED CT source code to target map code correlation not specified |
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