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Primary immunodeficiency disorders in Iran: Update and new insights from the third report of the national registry

Aghamohammadi, A. and Mohammadinejad, P. and Abolhassani, H. and Mirminachi, B. and Movahedi, M. and Gharagozlou, M. and Parvaneh, N. and Zeiaee, V. and Mirsaeed-Ghazi, B. and Chavoushzadeh, Z. and Mahdaviani, A. and Mansouri, M. and Yousefzadegan, S. and Sharifi, B. and Zandieh, F. and Hedayat, E. and Nadjafi, A. and Sherkat, R. and Shakerian, B. and Sadeghi-Shabestari, M. and Farid Hosseini, R. and Jabbari-Azad, F. and Ahanchian, H. and Behmanesh, F. and Zandkarimi, M. and Shirkani, A. and Cheraghi, T. and Fayezi, A. and Mohammadzadeh, I. and Amin, R. and Aleyasin, S. and Moghtaderi, M. and Ghaffari, J. and Arshi, S. and Javahertrash, N. and Nabavi, M. and Bemanian, M.H. and Shafiei, A. and Kalantari, N. and Ahmadiafshar, A. and Khazaei, H.A. and Atarod, L. and Rezaei, N. (2014) Primary immunodeficiency disorders in Iran: Update and new insights from the third report of the national registry. Journal of Clinical Immunology, 34 (4). pp. 478-490. ISSN 02719142 (ISSN)

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Abstract

Background: Primary immunodeficiency disorders (PID) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections and increased susceptibility to malignancies, lymphoproliferative and autoimmune conditions. National registries of PID disorders provide epidemiological data and increase the awareness of medical personnel as well as health care providers. Methods: This study presents the demographic data and clinical manifestations of Iranian PID patients who were diagnosed from March 2006 till the March of 2013 and were registered in Iranian PID Registry (IPIDR) after its second report of 2006. Results: A total number of 731 new PID patients (455 male and 276 female) from 14 medical centers were enrolled in the current study. Predominantly antibody deficiencies were the most common subcategory of PID (32.3 %) and were followed by combined immunodeficiencies (22.3 %), congenital defects of phagocyte number, function, or both (17.4 %), well-defined syndromes with immunodeficiency (17.2 %), autoinflammatory disorders (5.2 %), diseases of immune dysregulation (2.6 %), defects in innate immunity (1.6 %), and complement deficiencies (1.4 %). Severe combined immunodeficiency was the most common disorder (21.1 %). Other prevalent disorders were common variable immunodeficiency (14.9 %), hyper IgE syndrome (7.7 %), and selective IgA deficiency (7.5 %). Conclusions: Registration of Iranian PID patients increased the awareness of medical community of Iran and developed diagnostic and therapeutic techniques across more parts of the country. Further efforts must be taken by increasing the coverage of IPIDR via electronically registration and gradual referral system in order to provide better estimation of PID in Iran and reduce the number of undiagnosed cases. © 2014 Springer Science+Business Media.

Item Type: Article
Additional Information: Unmapped bibliographic data: LA - English [Field not mapped to EPrints] J2 - J. Clin. Immunol. [Field not mapped to EPrints] C2 - 24659230 [Field not mapped to EPrints] AD - Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, 62 Qarib St., Keshavarz Blvd., Tehran 14194, Iran [Field not mapped to EPrints] AD - Department of Allergy and Clinical Immunology, Pediatrics Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran [Field not mapped to EPrints] AD - Department of Rheumatology, Pediatrics Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran [Field not mapped to EPrints] AD - Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Al-Zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Mashhad University of Medical Sciences, Mashhad, Iran [Field not mapped to EPrints] AD - Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Ahvaz University of Medical Sciences, Ahvaz, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Mazandaran University of Medical Sciences, Sari, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Yazd University of Medical Sciences, Yazd, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Golestan University of Medical Sciences, Gorgan, Iran [Field not mapped to EPrints] AD - Department of Immunology and Allergy, Ghazvin University of Medical Sciences, Ghazvin, Iran [Field not mapped to EPrints] AD - Department of Immunology and Hematology, Zahedan Medical Sciences University, Zahedan, Iran [Field not mapped to EPrints] AD - Department of Pediatrics, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran [Field not mapped to EPrints] AD - Molecular Immunology Research Center, Department of Immunology, Tehran University of Medical Sciences, Tehran, Iran [Field not mapped to EPrints] DB - Scopus [Field not mapped to EPrints]
Uncontrolled Keywords: Epidemiology, infection, Iran, primary immunodeficiency, adolescent, adult, age distribution, article, autoinflammatory disease, child, consanguinity, family history, female, geographic distribution, health care personnel, human, hyper IgE syndrome, immune deficiency, immunoglobulin A deficiency, infant, innate immunity, Iran, major clinical study, male, newborn, phagocyte, primary immunodeficiency disorder, priority journal, register, classification, clinical trial, Immunologic Deficiency Syndromes, Iran, middle aged, multicenter study, pathology, preschool child, prevalence, register, Adolescent, Adult, Child, Child, Preschool, Consanguinity, Female, Humans, Immunologic Deficiency Syndromes, Infant, Infant, Newborn, Iran, Male, Middle Aged, Prevalence, Registries
Subjects: مقالات نمایه شده محققین دانشگاه در سایت ,Web of Science ,Scopus
موارد کلی
Divisions: معاونت تحقیقات و فناوری
Depositing User: GOUMS
Date Deposited: 18 Apr 2015 08:51
Last Modified: 04 May 2015 06:50
URI: http://eprints.goums.ac.ir/id/eprint/1748

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