Hereditary angioedema (Q39703): Difference between revisions
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Angioedema hereditário é causado na maioria dos casos por baixos níveis absolutos (tipo I) ou funcionais (tipo II) geneticamente determinados do inibidor de C1, um inibidor da proteinase plasmática envolvido na regulação da ativação do complemento. É caracterizado clinicamente por edema subcutâneo e/ou submucoso recorrente e pode resultar em obstrução laríngea com risco de vida. O envolvimento do trato digestivo geralmente causa dor abdominal. Isso e a ausência de pápulas urticariformes ou coceira a distinguem da forma comum de angioedema, que faz parte do espectro da urticária. | |||
| description / en | description / en | ||
Hereditary angioedema is caused in the majority of cases by genetically determined low absolute (type I) or functional (type II) levels of C1 inhibitor, a plasma proteinase inhibitor involved in regulation of complement activation. It is characterised clinically by recurrent subcutaneous and/or submucosal oedema and can result in life-threatening laryngeal obstruction. Involvement of the digestive tract commonly causes abdominal pain. This and the absence of accompanying urticarial weals or itch distinguish it from the common form of angioedema, which is part of the spectrum of urticaria. | |||
Revision as of 05:13, 13 August 2026
Hereditary angioedema is caused in the majority of cases by genetically determined low absolute (type I) or functional (type II) levels of C1 inhibitor, a plasma proteinase inhibitor involved in regulation of complement activation. It is characterised clinically by recurrent subcutaneous and/or submucosal oedema and can result in life-threatening laryngeal obstruction. Involvement of the digestive tract commonly causes abdominal pain. This and the absence of accompanying urticarial weals or itch distinguish it from the common form of angioedema, which is part of the spectrum of urticaria.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 4A00.14 |
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| English | Hereditary angioedema |
Hereditary angioedema is caused in the majority of cases by genetically determined low absolute (type I) or functional (type II) levels of C1 inhibitor, a plasma proteinase inhibitor involved in regulation of complement activation. It is characterised clinically by recurrent subcutaneous and/or submucosal oedema and can result in life-threatening laryngeal obstruction. Involvement of the digestive tract commonly causes abdominal pain. This and the absence of accompanying urticarial weals or itch distinguish it from the common form of angioedema, which is part of the spectrum of urticaria. |
