[Hereditary angioedema]

Emel Aygören-Pürsün1, Konrad Bork2

  • 1Angioödem-Ambulanz und Comprehensive Care Center für hereditäres Angioödem, Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Frankfurt, Goethe-Universität, Theodor-Stern-Kai 7, 60590, Frankfurt am Main, Deutschland. aygoeren@em.uni-frankfurt.de.

Der Internist
|August 1, 2019
PubMed

Hereditary angioedema (HAE) encompasses a heterogeneous group of diseases with similar phenotypes but different underlying genotypes. Specific clinical signs may point to HAE as opposed to histaminergic angioedema: the typical prolonged development of angioedema over time, positive family history, a lack of response to antihistamines and steroids and response to bradykinin antagonists are typical signs of HAE. The different types of HAE may be associated with a severe clinical course. They are life-long conditions and are still potentially life-threatening. The quality of life of patients with HAE may be considerably impaired. Management plans should be individualized, which is facilitated by the variety of specific medicastions available.

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