title: Results of an on-line survey of patients with hereditary angioedema.
abstract: Hereditary angioedema (HAE) is a rare but potentially fatal disease. An on-line survey was conducted of 63 patients in order to analyze the current clinical status of these patients. Sixty percent (n = 38) had type 1 HAE, 16% (n = 10) had type 2 HAE, and 24% (n = 15) did not know their type. The mean and median ages of onset of symptoms were predominantly in the teenage years. The diagnosis was typically made from 3.8 to 7.8 years after the onset of symptoms. The most common symptoms were abdominal pain and swelling (70%), swelling of extremities (45%), and throat closure (25%). Allergy-like symptoms were present in only 11.7% (n = 7) of patients; hypotension occurred in only one patient (1.6%). The patients averaged 4.7 emergency room visits per year. Nearly one-quarter of the patients were treated for anaphylaxis in the emergency room. There was a high level of dissatisfaction among the patients with the current management of the disease.
authors.affiliation: Division of Immunology and Infectious Diseases, Department of Pediatrics, Box 100296, University of Florida College of Medicine, Gainesville, FL 32610, USA.
authors.forename: Shih-Wen
authors.initials: SW
authors.lastname: Huang
mesh_terms: D000328:Adult; D000799:Angioedema / Q000150:complications / Q000235:genetics* / Q000523:psychology / Q000628:therapy; D002983:Clinical Competence; D004636:Emergency Service, Hospital; D005239:Fear; D005260:Female; D006304:Health Status; D006306:Health Surveys; D006801:Humans; D020407:Internet; D008297:Male; D017060:Patient Satisfaction; D014481:United States