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Primary Immunodeficiency Disorders in Iran: Update and New Insights from the Third Report of the National Registry

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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.

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Authors and Affiliations

  1. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, 62 Qarib St., Keshavarz Blvd., Tehran, 14194, Iran

    Asghar Aghamohammadi, Payam Mohammadinejad, Hassan Abolhassani, Babak Mirminachi, Nima Parvaneh, Sedigheh Yousefzadegan, Bahareh Sharifi, Ehsan Hedayat, Ali Nadjafi & Nima Rezaei

  2. Department of Allergy and Clinical Immunology, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran

    Masoud Movahedi & Mohammad Gharagozlou

  3. Department of Rheumatology, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran

    Vaheid Zeiaee

  4. Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran

    Bahram Mirsaeed-Ghazi & Fariborz Zandieh

  5. Department of Immunology and Allergy, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

    Zahra Chavoushzadeh, Alireza Mahdaviani & Mahboubeh Mansouri

  6. Department of Immunology and Allergy, Al-Zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran

    Roya Sherkat & Behzad Shakerian

  7. Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran

    Mahnaz Sadeghi-Shabestari

  8. Department of Immunology and Allergy, Mashhad University of Medical Sciences, Mashhad, Iran

    Reza Farid Hosseini, Farahzad Jabbari-Azad, Hamid Ahanchian, Fatemeh Behmanesh, Mohammadreza Zandkarimi & Afshin Shirkani

  9. Department of Pediatrics, 17th Shahrivar Children’s Hospital, Guilan University of Medical Sciences, Rasht, Iran

    Taher Cheraghi

  10. Department of Immunology and Allergy, Ahvaz University of Medical Sciences, Ahvaz, Iran

    Abbas Fayezi

  11. Department of Immunology and Allergy, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran

    Iraj Mohammadzadeh

  12. Department of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran

    Reza Amin, Soheila Aleyasin & Mojgan Moghtaderi

  13. Department of Immunology and Allergy, Mazandaran University of Medical Sciences, Sari, Iran

    Javad Ghaffari

  14. Department of Immunology and Allergy, Yazd University of Medical Sciences, Yazd, Iran

    Saba Arshi, Naser Javahertrash, Mohammad Nabavi, Mohammad Hassan Bemanian & Alireza Shafiei

  15. Department of Immunology and Allergy, Golestan University of Medical Sciences, Gorgan, Iran

    Najmedin Kalantari

  16. Department of Immunology and Allergy, Ghazvin University of Medical Sciences, Ghazvin, Iran

    Akefeh Ahmadiafshar

  17. Department of Immunology and Hematology, Zahedan Medical Sciences University, Zahedan, Iran

    Hossein Ali Khazaei

  18. Department of Pediatrics, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran

    Lida Atarod

  19. Molecular Immunology Research Center; and Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

    Nima Rezaei

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  1. Asghar Aghamohammadi
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  2. Payam Mohammadinejad
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  3. Hassan Abolhassani
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  9. Bahram Mirsaeed-Ghazi
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  40. Akefeh Ahmadiafshar
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  41. Hossein Ali Khazaei
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  42. Lida Atarod
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  43. Nima Rezaei
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Corresponding author

Correspondence to Asghar Aghamohammadi.

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Aghamohammadi, A., Mohammadinejad, P., Abolhassani, H. et al. Primary Immunodeficiency Disorders in Iran: Update and New Insights from the Third Report of the National Registry. J Clin Immunol 34, 478–490 (2014). https://doi.org/10.1007/s10875-014-0001-z

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  • DOI: https://doi.org/10.1007/s10875-014-0001-z

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