The therapeutic potential of a kallikrein inhibitor for treating hereditary angioedema.

Other Article (Journal Article;Review)

Hereditary angioedema (HAE) manifests as intermittent, painful attacks of submucosal oedema affecting the larynx, gastrointestinal tract or limbs. Currently, acute treatment is available in Europe but not USA, and requires intravenous administration of a pooled blood product. HAE is most likely caused by dysinhibition of the contact cascade, resulting in overproduction of bradykinin. DX-88 (ecallantide, Dyax Corp.) is a highly specific recombinant plasma kallikrein inhibitor that halts the production of bradykinin and can be dosed subcutaneously. In a placebo-controlled Phase II trial in patients with HAE, DX-88 resulted in significant improvement in symptoms compared with placebo. A Phase III trial is ongoing. This review explains the pathophysiology of HAE and the mechanism by which DX-88, a non-intravenous, nonplasma-derived therapy, might improve the disease, and discusses the clinical course of HAE and available treatments. Finally, it explores the potential value and efficacy of DX-88 in treating HAE.

Full Text

Duke Authors

Cited Authors

  • Levy, JH; O'Donnell, PS

Published Date

  • September 2006

Published In

Volume / Issue

  • 15 / 9

Start / End Page

  • 1077 - 1090

PubMed ID

  • 16916274

Electronic International Standard Serial Number (EISSN)

  • 1744-7658

Digital Object Identifier (DOI)

  • 10.1517/13543784.15.9.1077


  • eng

Conference Location

  • England