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Iranian Journal of Immunology، جلد ۱۱، شماره ۴، صفحات ۲۸۲-۲۹۱
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عنوان انگلیسی Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013)
چکیده انگلیسی مقاله 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.
کلیدواژه‌های انگلیسی مقاله diagnosis,prevalence,Primary Immunodeficiency Disorders
نویسندگان مقاله پیام محمدی نژاد |
research center for immunodeficiencies

بابک mirminachi |
research center for immunodeficiencies

بامداد صادقی |
research center for immunodeficiencies

مسعود موحدی |
pediatrics center of excellence, children amp;amp; 039;s medical center, tehran university of medical sciences
سازمان اصلی تایید شده: دانشگاه علوم پزشکی تهران (Tehran university of medical sciences)

محمد قره گوزلو |
pediatrics center of excellence, children amp;amp; 039;s medical center, tehran university of medical sciences
سازمان اصلی تایید شده: دانشگاه علوم پزشکی تهران (Tehran university of medical sciences)

جواد محمدی |
department of life science, faculty of new science and technology, tehran university
سازمان اصلی تایید شده: دانشگاه تهران (Tehran university)

حسن ابوالحسنی |
research center for immunodeficiencies

نیما رضایی |
research center for immunodeficiencies

اصغر آقامحمدی |
research center for immunodeficiencies

نشانی اینترنتی http://iji.sums.ac.ir/article_16789_baa2fb65721761c0be3d6ae842d7bc54.pdf
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