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Recent developments in the treatment of acute abdominal and facial attacks of hereditary angioedema: focus on human C1 esterase inhibitor

Authors Pasto-Cardona L, Lleonart-Bonfill, Marcoval-Caus J

Published 3 December 2010 Volume 2010:3 Pages 133—146


Review by Single-blind

Peer reviewer comments 3

Lourdes Pastó Cardona1 Ramon Lleonart Bellfill2 Joaquim Marcoval Caus3
1Pharmacy Service, 2Allergy Unit, Internal Medicine Service, 3Dermatology Service, Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain

Abstract: Hereditary angioedema (HAE) is a potentially fatal genetic disorder typified by a deficiency (type I) or dysfunction (type II) of the C1-inhibitor (C1-INH) and characterized by swelling of the extremities, face, trunk, abdominal viscera, and upper airway. Type III is normal estrogen-sensitive C1-INH HAE. Bradykinin, the main mediator of HAE, binds to endothelial B2 receptors, increasing vascular permeability and resulting in edema. HAE management includes short- and long-term prophylaxis. For treating acute episodes, C1-INH concentrate is recommended with regression of symptoms achieved in 30–90 min. Infusions of 500–1000 U have been used in Europe for years. Two plasma-derived C1-INH concentrates have been licensed recently in the United States: Berinert® for treating acute attacks and Cinryze® for prophylaxis in adolescent/adult patients. A recombinant C1-INH that is being considered for approval (conestat alfa) exhibited significant superiority versus placebo. Ecallantide (Kalbitor®) is a selective kallikrein inhibitor recently licensed in the United States for treating acute attacks in patients aged >16 years. It is administered in three 10-mg subcutaneous injections with the risk of anaphylactic reactions. Icatibant (Firazyr®) is a bradykinin B2 receptor competitor. It is administered subcutaneously as a 30-mg injection and approved in Europe but not in the United States.

Keywords: hereditary angioedema, C1 esterase inhibitor, acute attacks

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