Hereditary angioedema. Complex symptoms can make diagnosis difficult.
Although rare, hereditary angioedema is a potentially life-threatening disorder that can be difficult to diagnose. It is characterized by a deficiency in C1 esterase inhibitor (C1 INH). Manifestations include gastrointestinal, subcutaneous, and respiratory edema. Factors that trigger episodes vary. Symptoms typically last 48 to 72 hours, but they can last 4 hours to 1 week. Treatment includes prophylactic therapy with attenuated androgens or antifibrinolytic agents. Acute episodes can be medical emergencies, and airway management is a major concern. The treatment of choice in an acute episode is administration of plasma concentrate of C1 INH.
Borum
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George Washington School of Medicine and Health Sciences, Washington, DC 20047, USA.
Howard
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D015746:Abdominal Pain / Q000209:etiology; D000328:Adult; D000799:Angioedema / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D003613:Danazol / Q000627:therapeutic use; D004965:Estrogen Antagonists / Q000627:therapeutic use; D005260:Female; D006801:Humans
D004965:Estrogen Antagonists; D003613:Danazol