title: An update on hereditary angioedema.
abstract: OBJECTIVE
  To review and update the management and understanding of hereditary angioedema (HAE), while integrating insights into pediatric subtleties that exist in practice.
  
  
  RESULTS
  Major advances have recently been made in HAE treatment. Ecallantide (a kallikrein inhibitor approved for use in the United States in December 2009) and icatibant (a selective bradykinin B2 receptor antagonist approved for use in the United States in August 2011) represent novel subcutaneous therapies for acute HAE exacerbations. Recombinant human C1 esterase inhibitor (C1INH) serves as a promising future alternative to current mainstay acute and prophylactic treatment with plasma-derived C1INH. Recent guidelines have outlined new algorithms for short-term and long-term prophylaxis against HAE exacerbations.
  
  
  CONCLUSIONS
  The evolving standard of care for HAE management involves not only treatment of acute exacerbations but also individualized patient preference-sensitive short-term and long-term prophylaxis. Updated international consensus guidelines provide useful protocols, whereas recent clinical reviews have raised awareness of HAE. Further advances will likely focus on improving patient access to convenient acute and prophylactic treatment with C1INH.
authors.affiliation: Geisel School of Medicine, Hanover, New Hampshire, USA.
authors.forename: Derek
authors.initials: D
authors.lastname: Hsu
authors.forename: Marcus
authors.initials: M
authors.lastname: Shaker
mesh_terms: D000293:Adolescent; D000465:Algorithms; D054179:Angioedemas, Hereditary / Q000188:drug therapy* / Q000453:epidemiology; D001920:Bradykinin / Q000031:analogs & derivatives* / Q000627:therapeutic use; D065168:Bradykinin Receptor Antagonists; D002648:Child; D002675:Child, Preschool; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use*; D019317:Evidence-Based Medicine; D005260:Female; D006801:Humans; D007223:Infant; D007610:Kallikreins / Q000037:antagonists & inhibitors; D008297:Male; D010455:Peptides / Q000627:therapeutic use*; D017410:Practice Guidelines as Topic; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology
chemical_list: D065168:Bradykinin Receptor Antagonists; D050718:Complement C1 Inhibitor Protein; D010455:Peptides; C511194:ecallantide; C065679:icatibant; D007610:Kallikreins; D001920:Bradykinin