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.
Management of Children With Hereditary Angioedema Due to C1 Inhibitor Deficiency
POTENTIAL CONFLICT OF INTEREST: The members of the MAB are leading practitioners in the United States with interest in treatment of hereditary angioedema, a rare disease. As such, many have participated in all trials that have been performed. The following is a specific list of potential conflicts associated with each author: Dr Frank: consultant, BioCryst, Shire; Dr Zuraw: consultant, Alnylan, Arrowhead, BioCryst, CSL Behring, Ionis, Nektar, Shire, Salix; Dr Banerji: consultant, CSL Behring, BioCryst, Shire; grants to institution, BioCryst, Shire; Dr Bernstein: speakers’ bureau, Shire, CSL Behring, BI, Baxalta, Greer, Novartis/Genentech; consultant, CSL Behring, Shire, BioCryst; grants, CSL Behring, Shire, BioCryst; Dr Craig: speakers’ bureau, Shire, Grifols, CSL Behring; grants >$50,000, Shire, Merck, Novartis, Sanofi-Aventis, Boehringer Ingelheim, Genentech, GSK AstraZeneca, Teva; grants >$10,000, Grifols, BioCryst, Pharming, Genentech, GSK, BioCryst; Dr Busse: speaker, Shire, CSL Behring; Dr Christiansen: none; Dr Davis-Lorton: consultant, Shire, Dyax; grant, Dyax; Dr Li: speakers’ bureau, CSL Behring, Shire; consultant, CSL Behring, Shire, BioCryst, Dyax; grants, CSL Behring, Shire; Dr Lumry: speakers’ bureau, CSL Behring, Dyax, Shire/Viropharma, Salix; consultant, BioCryst, CSL Behring, Shire/Viropharma; research grants, BioCryst, CSL Behring, Salix Shire/Viropharma; and Dr Riedl: consultant, BioCryst, CSL Behring, Shire, Baxalta, Salix, Arrowhead; grants, CSL Behring, Shire, Dyax, Pharming, BioCryst, Amgen; speaker, CSL Behring, Dyax, Shire, Salix, Baxalta.
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
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Michael M. Frank, Bruce Zuraw, Aleena Banerji, Jonathan A. Bernstein, Timothy Craig, Paula Busse, Sandra Christiansen, Marc Davis-Lorton, H. Henry Li, William R. Lumry, Marc Riedl, US Hereditary Angioedema Association Medical Advisory Board; Management of Children With Hereditary Angioedema Due to C1 Inhibitor Deficiency. Pediatrics November 2016; 138 (5): e20160575. 10.1542/peds.2016-0575
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