title: Diagnosis and management of hereditary angioedema: an emergency medicine perspective.
abstract: BACKGROUND
  Hereditary angioedema (HAE) is a rare and often debilitating condition associated with substantial morbidity and mortality in the absence of appropriate intervention. An underlying deficiency in functional C1-inhibitor (C1-INH) protein induces a vulnerability to unchecked activation of the complement, contact, and coagulation/fibrinolytic systems. The clinical consequence is a pattern of recurring attacks of non-pitting, non-pruritic edema, the urgency of which varies by the affected site. Laryngeal edema can escalate rapidly to asphyxiation, and severe cases of abdominal swelling can lead to hypovolemic shock.
  
  
  OBJECTIVE
  This report reviews the emergency diagnosis and treatment of hereditary angioedema and the impact of recently introduced treatments on treatment in the United States.
  
  
  CONCLUSIONS
  Until recently, emergency physicians in the United States were hindered by the lack of rapidly effective treatment options for HAE attacks. In this article, general clinical and laboratory diagnostic procedures are reviewed against the backdrop of two case studies: one patient presenting with a known history of HAE and one with previously undiagnosed HAE. In many countries outside the United States, plasma-derived C1-INH concentrate has for decades been the first-line treatment for acute attacks. The end of 2009 ushered in a new era in the pharmacologic management of HAE attacks in the United States with the approval of two new treatment options for acute treatment: a plasma-derived C1-INH concentrate and a kallikrein inhibitor.
  
  
  CONCLUSIONS
  With access to targeted and effective treatments, emergency physicians are now better equipped for successful and rapid intervention in urgent HAE cases.
authors.affiliation: Department of Emergency Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio 45267-0563, USA.
authors.forename: Joseph J
authors.initials: JJ
authors.lastname: Moellman
authors.forename: Jonathan A
authors.initials: JA
authors.lastname: Bernstein
mesh_terms: D000328:Adult; D058109:Airway Management; D000728:Androgens / Q000627:therapeutic use; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use; D003181:Complement C4 / Q000378:metabolism; D004636:Emergency Service, Hospital; D005260:Female; D056829:Hereditary Angioedema Types I and II / Q000097:blood / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D007610:Kallikreins / Q000037:antagonists & inhibitors; D008297:Male; D010808:Physical Examination; D014481:United States; D055815:Young Adult
chemical_list: D000728:Androgens; D000933:Antifibrinolytic Agents; D050718:Complement C1 Inhibitor Protein; D003181:Complement C4; D007610:Kallikreins