Author/Authors :
Shahinpour, Shervin Research Center for Immunodeficiencies, Pediatrics Center of Excellence - Children's Medical Center - Tehran University of Medical Sciences - Tehran , Abolhassani, Hassan Research Center for Immunodeficiencies, Pediatrics Center of Excellence - Children's Medical Center - Tehran University of Medical Sciences - Tehran , Movahedi, Masoud Research Center for Immunodeficiencies, Pediatrics Center of Excellence - Children's Medical Center - Tehran University of Medical Sciences - Tehran , Mohammadinejad, Payam Research Center for Immunodeficiencies, Pediatrics Center of Excellence - Children's Medical Center - Tehran University of Medical Sciences - Tehran , Aghamohammadi, Asghar Research Center for Immunodeficiencies, Pediatrics Center of Excellence - Children's Medical Center - Tehran University of Medical Sciences - Tehran , Tavakol, Marzieh Department of Allergy and Clinical Immunology - Shahid Bahonar Hospital - Alborz University of Medical Sciences - Karaj , Arshi, Saba Allergy and Clinical Immunology Department of Rasol- E-Akram Hospital - Iran University of Medical Sciences - Tehran
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. Conclusion: The clinical criteria of the Irani
