title: Pasteurized and nanofiltered, plasma-derived C1 esterase inhibitor concentrate for the treatment of hereditary angioedema.
abstract: Hereditary angioedema (HAE) is a relatively rare autosomal dominant disorder that is typically characterized by recurrent episodes of edema in various body locations. It is most commonly caused by an inherited deficiency of functionally active C1 esterase inhibitor (C1-INH). Replacement therapy with a human plasma-derived C1-INH concentrate is recommended for the treatment and prophylaxis of acute attacks of HAE due to C1-INH deficiency (HAE-C1-INH). This article will discuss the current therapies available for the treatment of HAE-C1-INH, latest treatment guidelines, results of several studies demonstrating the efficacy and safety of the plasma-derived, pasteurized and nanofiltered C1-INH concentrate Berinert(®) (CSL Behring GmBH, Marburg, Germany), and future perspectives for the treatment and management of HAE-C1-INH.
authors.affiliation: Department of Dermatology, University Medical Center, Langenbeckstr 1, 55131 Mainz, Germany. konrad.bork@unimedizin-mainz.de.
authors.forename: Konrad
authors.initials: K
authors.lastname: Bork
mesh_terms: D054179:Angioedemas, Hereditary / Q000628:therapy*; D050718:Complement C1 Inhibitor Protein / Q000737:chemistry / Q000627:therapeutic use*; D006801:Humans; D061352:Pasteurization / Q000379:methods; D010949:Plasma / Q000737:chemistry*; D017410:Practice Guidelines as Topic
chemical_list: D050718:Complement C1 Inhibitor Protein
keywords: Berinert®; C1 esterase inhibitor; C1-INH; complement system; contact system; hereditary angioedema; pdC1-INH concentrate; pnfC1-INH; prophylaxis