title: Report on the First Survey of Iranian Patients with Hereditary Angioedema.
abstract: BACKGROUND
  Hereditary angioedema (HAE) is a rare autosomal dominant primary immunodeficiency with complement system defect characterized by recurrent episodes of angioedema involving the skin or mucosa of the upper respiratory and gastrointestinal tracts.
  
  
  OBJECTIVE
  To characterize the clinical and laboratory data of hereditary angioedema in Iran.
  
  
  METHODS
  Patients with probable diagnosis of angioedema were enrolled in this study. Demographic and clinical data were documented in the designed questionnaire including history of attacks, triggering factors and laboratory data such as C4, C1 esterase inhibitor level and function.
  
  
  RESULTS
  Among 63 patients who were clinically suspicious for angioedema (23 males and 40 females), 8 cases (12.7%) were diagnosed with HAE. Among these 8 HAE patients, 3 were diagnosed with HAE type 1 and five patients were diagnosed with HAE type 2. The mean ages of HAE type 1 and type 2 patients were 25.6 ± 13.5 and 22.4 ± 12.32 years. The mean age of onset in HAE type 1 group was 8 ± 5 years and in HAE type 2 group was 18.8 ± 11.84 years. The mean diagnosis delay was 17.6 years in HAE type 1 patients and 2.6 years in HAE type 2. The most common clinical manifestation was facial swelling presented in all HAE patients followed by swelling of extremities which was present in 7 patients with HAE.
  
  
  CONCLUSIONS
  The clinical criteria of the Iranian patients with HAE were consistent with the known clinical patterns of the disease.
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, e-mail: and1_sh@yahoo.com.
authors.forename: Shervin
authors.initials: S
authors.lastname: Shahinpour
authors.forename: Marzieh
authors.initials: M
authors.lastname: Tavakol
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.forename: Payam
authors.initials: P
authors.lastname: Mohammadinejad
authors.forename: Masoud
authors.initials: M
authors.lastname: Movahedi
authors.forename: Saba
authors.initials: S
authors.lastname: Arshi
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
mesh_terms: D000293:Adolescent; D000328:Adult; D054179:Angioedemas, Hereditary / Q000453:epidemiology* / Q000503:physiopathology; D002648:Child; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism*; D003181:Complement C4 / Q000378:metabolism*; D005260:Female; D006801:Humans; D007492:Iran; D008297:Male; D058028:Research Report; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D050718:Complement C1 Inhibitor Protein; D003181:Complement C4