Hereditary angioedema: the clinical syndrome and its management in the United States.
There have been important breakthroughs in the understanding and treatment of hereditary angioedema (HAE). An associated abnormality of the serum protein C1 inhibitor led to purified protein use to end attacks. Consideration of the endocrine functions led to rediscovery of impeded androgen use in disease prophylaxis. Considerations of pathophysiology led to introduction of epsilon aminocaproic and tranexemic acids in prophylaxis and to a resurgence in trials of new therapeutic agents. We have gone from a situation where it was not uncommon for patients to have a severe attack sometime in their lives that led to airway compromise and possible death to a situation where death from disease is highly unusual. Thus HAE is in many ways a success story of modern medicine.
Frank
Michael M
MM
Duke University Medical Center, Durham, NC 27710, USA. frank007@mc.duke.edu
D015119:Aminocaproic Acid / Q000627:therapeutic use; D000728:Androgens / Q000627:therapeutic use; D000799:Angioedema / Q000188:drug therapy* / Q000503:physiopathology*; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D050718:Complement C1 Inhibitor Protein / Q000235:genetics / Q000378:metabolism; D005341:Fibrinolysin / Q000037:antagonists & inhibitors; D006801:Humans; D009154:Mutation; D014481:United States
D000728:Androgens; D000933:Antifibrinolytic Agents; D050718:Complement C1 Inhibitor Protein; D005341:Fibrinolysin; D015119:Aminocaproic Acid