Hereditary angioedema: the clinical syndrome and its management in the United States.


Journal Article (Review)

There have been important breakthroughs in the understanding and treatment of hereditary angioedema (HAE). An associated abnormality of the serum protein C1 inhibitor led to purified protein use to end attacks. Consideration of the endocrine functions led to rediscovery of impeded androgen use in disease prophylaxis. Considerations of pathophysiology led to introduction of epsilon aminocaproic and tranexemic acids in prophylaxis and to a resurgence in trials of new therapeutic agents. We have gone from a situation where it was not uncommon for patients to have a severe attack sometime in their lives that led to airway compromise and possible death to a situation where death from disease is highly unusual. Thus HAE is in many ways a success story of modern medicine.

Full Text

Cited Authors

  • Frank, MM

Published Date

  • November 2006

Published In

Volume / Issue

  • 26 / 4

Start / End Page

  • 653 - 668

PubMed ID

  • 17085283

Pubmed Central ID

  • 17085283

International Standard Serial Number (ISSN)

  • 0889-8561

Digital Object Identifier (DOI)

  • 10.1016/j.iac.2006.09.005


  • eng

Conference Location

  • United States