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Current Allergy and Asthma Reports

, Volume 9, Issue 4, pp 280–285 | Cite as

Hereditary angioedema with normal C1 inhibition

  • Konrad BorkEmail author
Article

Abstract

Until recently, it was assumed that hereditary angioedema was a disease resulting exclusively from a genetic deficiency of the C1 inhibitor. In 2000, families with hereditary angioedema, normal C1 inhibitor activity, and protein in plasma were described. Since then, numerous patients and families with this condition have been reported. Most of the patients were women. In many of the affected women, oral contraceptives, hormone replacement therapy containing estrogens, and pregnancies triggered the clinical symptoms. In some families, mutations in the coagulation factor XII (Hageman factor) gene were detected in the affected persons.

Keywords

Angioedema Allergy Clin Immunol Danazol Hereditary Angioedema Healthy Proband 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Current Medicine Group, LLC 2009

Authors and Affiliations

  1. 1.Department of DermatologyJohannes Gutenberg UniversityMainzGermany

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