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Mohammadinejad, Payam, Mirminachi, Babak, Sadeghi, Bamdad, Movahedi, Masoud, Gharagozlou, Mohammad, Mohammadi, Javad, Abolhassani, Hassan, Rezaei, Nima, Aghamohammadi, Asghar. (1393). Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013). سامانه مدیریت نشریات علمی, 11(4), 282-291.
Payam Mohammadinejad; Babak Mirminachi; Bamdad Sadeghi; Masoud Movahedi; Mohammad Gharagozlou; Javad Mohammadi; Hassan Abolhassani; Nima Rezaei; Asghar Aghamohammadi. "Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013)". سامانه مدیریت نشریات علمی, 11, 4, 1393, 282-291.
Mohammadinejad, Payam, Mirminachi, Babak, Sadeghi, Bamdad, Movahedi, Masoud, Gharagozlou, Mohammad, Mohammadi, Javad, Abolhassani, Hassan, Rezaei, Nima, Aghamohammadi, Asghar. (1393). 'Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013)', سامانه مدیریت نشریات علمی, 11(4), pp. 282-291.
Mohammadinejad, Payam, Mirminachi, Babak, Sadeghi, Bamdad, Movahedi, Masoud, Gharagozlou, Mohammad, Mohammadi, Javad, Abolhassani, Hassan, Rezaei, Nima, Aghamohammadi, Asghar. Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013). سامانه مدیریت نشریات علمی, 1393; 11(4): 282-291.

Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013)

Iranian Journal of Immunology
مقاله 7، دوره 11، شماره 4، اسفند 2014، صفحه 282-291    اصل مقاله (222.09 K)
نوع مقاله: Original Article
نویسندگان
Payam Mohammadinejad1؛ Babak Mirminachi1؛ Bamdad Sadeghi1؛ Masoud Movahedi2؛ Mohammad Gharagozlou2؛ Javad Mohammadi3؛ Hassan Abolhassani1؛ Nima Rezaei1، 4؛ Asghar Aghamohammadi* 1
1Research Center for Immunodeficiencies
2Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences
3Department of Life Science, Faculty of New Science and Technology, Tehran University
4Molecular Immunology Research Center, and Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
چکیده
Background: Primary immunodeficiency disorders (PID) are a group of hereditary disorders characterized by an increased susceptibility to severe and recurrent infections, autoimmunity, lymphoproliferative disorders, and malignancy.
Objective: To evaluate the demographic and clinical data of PID patients diagnosed in a referral pediatric hospital.
Method: All PID cases with a confirmed diagnosis, according to the criteria of International Union of Immunological Societies, who were referred to the Children’s Medical Center in Tehran, Iran, between March 2006 and March 2013 were enrolled in this retrospective cohort study.
Results: Three-hundred and seven PID patients were investigated. Predominantly antibody deficiencies were the most common group of PID observed in 118 cases (38.4%), followed by the well-defined syndromes with immunodeficiency in 52 (16.9%), congenital defects of phagocyte in 45 (14.7%), combined immunodeficiencies in 36 (11.7%), autoinflammatory disorders in 34 (11.4%), immune dysregulation in 11 (3.6%), complement deficiencies in 7 (2.3%), and defects in innate immunity in 3 (1%). Selective IgA deficiency was the most prevalent disorder which affected 46 individuals (14.9%). The median diagnostic delay was 15 months.
Conclusion: Increased awareness and availability of diagnostic tests could result in the better recognition of more undiagnosed PID cases and a decrease in diagnostic delay.
کلیدواژه‌ها
diagnosis؛ prevalence؛ Primary Immunodeficiency Disorders
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