Hereditary Angioedema due to C1 Inhibitor Deficiency: C1-INH Replacement Therapy

Reviews in Health Care

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Title Hereditary Angioedema due to C1 Inhibitor Deficiency: C1-INH Replacement Therapy
Creator Cancian, Mauro
Subject Hereditary angioedema;
Hereditary angioedema; C1 inhibitor deficiency; Replacement therapy
Description Hereditary angioedema (HAE) is a rare condition affecting about 1 in 50.000 individuals and caused by a mutation in the gene encoding the C1-esterase inhibitor (C1-INH), which is involved in the control of complement, clotting, fibrinolytic and kinin pathways. HAE is characterized by plasma outflow from blood vessels, leading to fluid collecting (edema) in the deep tissue layers of the face, larynx, abdomen, and extremities. Three different types of HAE have been identified: in type I the mutation leads to the lack of production of C1-INH, in type II the mutation leads to the production of dysfunctional C1-INH, while type III is extremely rare and still not fully understood. Therapeutic approaches for HAE include on-demand treatments to stop angioedema attacks and prophylactic treatment to prevent attacks both by pre-procedural (short-term) and routine (long-term) prophylaxis. Aim of the present review is to present an overview of C1-INH replacement therapy with the plasma-derived concentrate of C1-INH Berinert® (CSL Behring GmbH) in the treatment of type I and II HAE.
Publisher SEEd
Contributor CSL Behring
Date 2014-04-29
Type info:eu-repo/semantics/article

Format text/html
Source Reviews in Health Care; Vol 5, No 2 (2014); 55-66
Language eng

Rights Copyright (c) 2014 Seed

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