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Review
. 2017 Jul 24:6:F1000 Faculty Rev-1205.
doi: 10.12688/f1000research.11320.1. eCollection 2017.

Pediatric hereditary angioedema: an update

Affiliations
Review

Pediatric hereditary angioedema: an update

Geetika Sabharwal et al. F1000Res. .

Abstract

Hereditary angioedema (HAE) with C1-inhibitor (C1-Inh) deficiency (C1-Inh-HAE) is a rare, life-threatening, and disabling genetic disorder characterized by self-limited tissue swelling caused by deficiency or dysfunction of C1-Inh. Our aim in this update is to discuss new advances in HAE therapy, focusing mainly on the various treatment options that have become available recently and also drugs that are under trial for prophylaxis to prevent attacks. There is a paradigm shift to where the treatment of HAE is headed, focusing now on prophylactic treatment rather than abortive management.

Keywords: C1-INH gene; Genetic disorder; HAE; Hereditary angioedema.

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Conflict of interest statement

Competing interests: GS declares that they have no competing interests. TC is a speaker at CSL Behring, Shire and Grifols, a researcher at CSL Behring, Shire, Grifols and BioCryst, and a consultant at CSL Behring and BioCryst.No competing interests were disclosed.No competing interests were disclosed.

References

    1. Bhardwaj N, Craig TJ: Treatment of hereditary angioedema: a review (CME). Transfusion. 2014;54(11):2989–96; quiz 2988. 10.1111/trf.12674 - DOI - PubMed
    1. Ghazi A, Grant JA: Hereditary angioedema: epidemiology, management, and role of icatibant. Biologics. 2013;7:103–13. 10.2147/BTT.S27566 - DOI - PMC - PubMed
    1. Talavera A, Larraona JL, Ramos JL, et al. : Hereditary angioedema: an infrequent cause of abdominal pain with ascites. Am J Gastroenterol. 1995;90(3):471–4. - PubMed
    1. Gompels MM, Lock RJ, Abinun M, et al. : C1 inhibitor deficiency: consensus document. Clin Exp Immunol. 2005;139(3):379–94. 10.1111/j.1365-2249.2005.02726.x - DOI - PMC - PubMed
    1. Nzeako UC, Frigas E, Tremaine WJ: Hereditary angioedema: a broad review for clinicians. Arch Intern Med. 2001;161(20):2417–29. 10.1001/archinte.161.20.2417 - DOI - PubMed

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