Pediatric hereditary angioedema
Article first published online: 9 DEC 2013
© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
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Pediatric Allergy and Immunology
Volume 25, Issue 5, pages 420–427, August 2014
How to Cite
Pediatric hereditary angioedema. Pediatr Allergy Immunol 2014: 25: 420–427..
- Issue published online: 29 AUG 2014
- Article first published online: 9 DEC 2013
- Manuscript Accepted: 22 OCT 2013
- ViroPharma Incorporated
- hereditary angioedema
Hereditary angioedema (HAE) is a lifelong illness characterized by recurrent swelling of the skin, intestinal tract, and, ominously, the upper airway. It is caused by inadequate activity of the protein C1-inhibitor, with dysfunction in the kallikrein/bradykinin pathway underlying the clinical symptoms. In addition to the physical symptoms, patients experience significant decrements in vocational and school achievement as well as in overall quality of life. Symptoms often begin in childhood and occur by age 20 in most patients, but life-threatening attacks are uncommon in the pediatric population. The availability of new therapies has transformed the management of HAE.