Management of Children With Hereditary Angioedema Due to C1 Inhibitor Deficiency.


Journal Article

Hereditary angioedema (HAE) is a potentially life-threatening inherited disease characterized by attacks of skin swelling, severe abdominal pain, and upper airway swelling. Attacks typically begin in childhood, but the appropriate diagnosis is often missed. Attacks do not respond to epinephrine, antihistamines, or glucocorticoids. Recently, many effective drugs have been approved for treatment of adults with HAE, and the Medical Advisory Board of the HAE Patient's Association has developed and reported treatment recommendations for adults. Only 1 medication is approved for treatment of children <12 years of age, and there are no reported consensus recommendations for treatment of young children in the United States. The 11-member Medical Advisory Board, with extensive experience in the treatment of children, in concert with the leaders of the HAE Patient's Association, has developed these consensus recommendations to help in recognition, diagnosis, treatment of attacks, and prophylaxis of children with HAE.

Full Text

Cited Authors

  • Frank, MM; Zuraw, B; Banerji, A; Bernstein, JA; Craig, T; Busse, P; Christiansen, S; Davis-Lorton, M; Li, HH; Lumry, WR; Riedl, M; US Hereditary Angioedema Association Medical Advisory Board,

Published Date

  • November 2016

Published In

Volume / Issue

  • 138 / 5

PubMed ID

  • 27940765

Pubmed Central ID

  • 27940765

Electronic International Standard Serial Number (EISSN)

  • 1098-4275

Digital Object Identifier (DOI)

  • 10.1542/peds.2016-0575


  • eng

Conference Location

  • United States