title: Emergency treatment of acute attacks in hereditary angioedema due to C1 inhibitor deficiency: what is the evidence?
abstract: Hereditary angioedema (HAE) is caused by a deficiency in C1 esterase-inhibitor (C1-INH) and is characterised by skin swelling, abdominal pain and episodes of upper respiratory tract obstruction. Oedema of the larynx can result in rapid asphyxiation and requires emergency treatment. Three treatment options for emergency treatment of HAE are reviewed: fresh frozen plasma, solvent/detergent-treated plasma and C1-INH concentrate. It is concluded that while all three treatment options are theoretically effective in the emergency treatment of HAE, C1-INH is the treatment of choice.
authors.affiliation: Department of Immunology, Barts and The London NHS Trust, London, UK. hilary.longhurst@bartsandthelondon.nhs.uk
authors.forename: H J
authors.initials: HJ
authors.lastname: Longhurst
mesh_terms: D000208:Acute Disease; D000799:Angioedema / Q000188:drug therapy*; D003174:Complement C1 Inactivator Proteins / Q000191:economics / Q000627:therapeutic use*; D016527:Drug Costs; D004638:Emergency Treatment; D006801:Humans; D010949:Plasma
chemical_list: D003174:Complement C1 Inactivator Proteins