L’angio-oedème héréditaire Mécanismes, diagnostic et prise en charge

(2015) Louvain médical — Vol. 134, n° 4, p. 144-152 (2015)

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Abstract
(en) [Hereditary angioedema Mechanisms, diagnosis, and management] Hereditary angioedema (HAE) is caused by a heterozygous deficiency of C1-inhibitor. HAE patients have intermittent cutaneous or mucosal swellings because of a failure to control local production of bradykinin. Swellings typically evolve in several hours and persist for a few days. In addition to orofacial angioedema, painless swellings affect the extremities, leading to disfigurement or interference with work and other daily activities. Angioedema affecting the gastrointestinal tract or abdominal viscera is responsible for severe pain, often associated with vomiting due to edematous bowel obstruction. Swellings may involve the larynx and be fatal, if untreated. In this paper, we review the clinical features, diagnosis, and management of HAE, with specific emphasis on the new therapeutic agents and treatment modalities such as self-treatment at home and prolonged prophylaxis
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Hermans, C., & Lambert, C. (2015). L’angio-oedème héréditaire Mécanismes, diagnostic et prise en charge. Louvain médical, 134(4), 144-152. https://hdl.handle.net/2078.5/184329 (Original work published 2015)