title: Type I and Type II Hereditary Angioedema: Clinical and Laboratory Findings in Iranian Patients.
abstract: BACKGROUND
  Hereditary angioedema (HAE) is a rare autosomal dominant disorder characterized by C1-INH (C1 esterase inhibitor), low serum levels (type I), dysfunction (type II) or normal serum levels and function (type III), which lead to subcutaneous and submucosal edema attacks. The aim of this study was to investigate the demographic, clinical and laboratory findings of Iranian patients with HAE.
  
  
  METHODS
  The patients with a history or symptoms of angioedema who were referred to Immunology, Asthma and Allergy Research Institute (IAARI) between Jan 2006 and Jan 2014, were assessed based on a specific questionnaire and laboratory evaluation. The patients with a definite diagnosis of HAE type I and type II were entered into this study.
  
  
  RESULTS
  Among 51 patients, 63.3% were diagnosed with HAE type I and 36.7% with HAE type II. Fifteen patients were under 18 years and 36 were adults. The mean age of symptoms onset and diagnosis were 12.33 ± 10.20 years and 24.48 ± 14.64 years, respectively. The mean delay of diagnosis was 11.02 ± 11.60 years. The most commonly involved locations of edema were hands, face and genitalia. Moreover, laryngeal edema was observed in 61.2% of patients, which led to death in two patients during this study.
  
  
  CONCLUSIONS
  Hereditary angioedema is a life threatening disease with considerable morbidity and mortality. The outcomes of this study can be used to inform clinicians and health care providers about HAE, which can help earlier diagnosis and better management of the patients, specifically in life threatening attacks.
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Kargarsharif
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Narges
authors.initials: N
authors.lastname: Mehranmehr
authors.affiliation: Department of Pathology, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Sara
authors.initials: S
authors.lastname: Zahedi Fard
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Mohammad Reza
authors.initials: MR
authors.lastname: Fazlollahi
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Maryam
authors.initials: M
authors.lastname: Ayazi
authors.affiliation: Non-Communicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, Iran.
authors.forename: Iraj
authors.initials: I
authors.lastname: Mohammadzadeh
authors.affiliation: Department of Immunology and Allergy, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran.
authors.forename: Mohammad
authors.initials: M
authors.lastname: Nabavi
authors.affiliation: Department of Immunology and Allergy, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran.
authors.forename: Mohammad Hasan
authors.initials: MH
authors.lastname: Bemanian
authors.affiliation: Division of Allergy and Immunology, School of Medicine, Ahvaz University of Medical Sciences, Ahvaz, Iran.
authors.forename: Abbas
authors.initials: A
authors.lastname: Fayezi
authors.affiliation: Department of Immunology and Allergy, Children Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Masoud
authors.initials: M
authors.lastname: Movahedi
authors.affiliation: Department of Pediatrics, Kashan University of Medical Sciences, Kashan, Iran.
authors.forename: Marzieh
authors.initials: M
authors.lastname: Heidarzadeh
authors.affiliation: Department of Pediatrics, Kashan University of Medical Sciences, Kashan, Iran.
authors.forename: Najmodin
authors.initials: N
authors.lastname: Kalantari
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Somaieh
authors.initials: S
authors.lastname: Arefimehr
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Shiva
authors.initials: S
authors.lastname: Saghafi
authors.affiliation: Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran. pourpakz@tums.ac.ir, pourpakz@sina.tums.ac.ir, zpourpak@yahoo.com.
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pourpak
mesh_terms: D000293:Adolescent; D000328:Adult; D054179:Angioedemas, Hereditary / Q000145:classification* / Q000453:epidemiology* / Q000235:genetics; D002648:Child; D050718:Complement C1 Inhibitor Protein / Q000032:analysis*; D005260:Female; D006801:Humans; D007492:Iran; D008297:Male; D055815:Young Adult
chemical_list: D050718:Complement C1 Inhibitor Protein