• Lancet · Feb 2012

    Review

    Hereditary angio-oedema.

    • Hilary Longhurst and Marco Cicardi.
    • Department of Immunology, Barts and The London National Health Service Trust, Whitechapel, London, UK. hilary.longhurst@bartsandthelondon.nhs.uk
    • Lancet. 2012 Feb 4; 379 (9814): 474-81.

    AbstractHereditary angio-oedema is caused by a heterozygous deficiency of C1 inhibitor. This inhibitor regulates several inflammatory pathways, and patients with hereditary angio-oedema have intermittent cutaneous or mucosal swellings because of a failure to control local production of bradykinin. Swellings typically evolve in several hours and persist for a few days. In addition to orofacial angio-oedema, painless swellings affect peripheries, which causes disfigurement or interference with work and other activities of daily living. Angio-oedema affecting the gastrointestinal tract or abdominal viscera causes severe pain often with vomiting due to oedematous bowel obstruction. About 2% of swellings involve the larynx and can be fatal if untreated. About 50% of patients have laryngeal swellings that are potentially fatal despite prophylaxis. In this Seminar we review the clinical features, diagnosis, and management of hereditary angio-oedema, with specific emphasis on the new treatments available for acute swellings.Copyright © 2012 Elsevier Ltd. All rights reserved.

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