title: [Progress with management of hereditary angioedema].
abstract: Hereditary angioedema (HAE) is a rare type of angioedema caused by a quantitative or functional deficit of C1 inhibitor (C1 INH) that leads to excess production of bradykinin, which can result in acute localized swelling attacks in the skin or mucous membranes of the mouth, head and neck, extremities, gastrointestinal (GI) tract, genitals, trunk, and larynx. Angioedema in the respiratorytract maycause airway obstruction; severe abdominal pain, vomiting, or diarrhea may occur in the GI tract. Patients with HAE may be diagnosed and managed by HAE specialists or by primary care physicians depending on individual circumstances. Proper treatment requires differentiation from other forms of angioedema. Patients with HAE who are managed appropriately with medications that treat and prevent atttacks may have a lower risk of death from laryngeal edema and a better quality of life. Less frequent attacks may allow them to attend work, school, and leisure activities more regularlyand be free of the pain and disfigurement of HAE attacks moreoften.
authors.affiliation: Edward Via College of Osteopathic Medicine, Carolina Campus, Charlotte, North Carolina, USA. djohnston@asthmanc.com
authors.forename: D T
authors.initials: DT
authors.lastname: Johnston
authors.forename: H
authors.initials: H
authors.lastname: Lode
mesh_terms: D000465:Algorithms; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001920:Bradykinin / Q000031:analogs & derivatives / Q000627:therapeutic use; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D050718:Complement C1 Inhibitor Protein / Q000235:genetics / Q000627:therapeutic use; D004252:DNA Mutational Analysis; D056829:Hereditary Angioedema Types I and II / Q000175:diagnosis* / Q000235:genetics / Q000517:prevention & control / Q000628:therapy*; D006801:Humans; D010455:Peptides / Q000627:therapeutic use; D010949:Plasma
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D050718:Complement C1 Inhibitor Protein; D010455:Peptides; C511194:ecallantide; C065679:icatibant; D001920:Bradykinin