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Frequency and Clinical Manifestations of Patients with Primary Immunodeficiency Disorders in Iran: Update from the Iranian Primary Immunodeficiency Registry

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Primary immunodeficiency disorders (PID) are a heterogeneous group of diseases, characterized by an increased susceptibility to infections. A total of 930 patients (573 males and 357 females) are registered in Iranian PID Registry (IPIDR) during three decades. Predominantly antibody deficiencies were the most common (38.4%), followed by congenital defects of phagocyte number and/or function (28.3%), other well-defined immunodeficiency syndromes (17.7%), combined T- and B-cell immunodeficiencies (11.0%), complement deficiencies (2.4%), and diseases of immune dysregulation (2.3%). Common variable immunodeficiency was the most frequent disorder (20.8%), followed by chronic granulomatous disease, ataxia-telangiectasia, btk deficiency, selective IgA deficiency, and T-B-severe combined immunodeficiency. The frequency of other PID disorders was less than 50 in number (<5%). There is an increasing trend in recognition of more PID in the recent years. Construction of such registry is not only important for its epidemiological aspect but also for its role in increasing the physician's knowledge about such disorders.

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ACKNOWLEDGMENTS

This registry has been accomplished by the grant of Tehran University of Medical Sciences. We thank all contributors to this registry, who provided information on PID patients; we gratefully acknowledge the efforts of Dr. Akefeh Ahmadi Afshar, Dr. Hengameh Abdollahpour, Dr. Laleh Amiri Kordestani, Dr. Ali Babaei Jandaghi, Dr. Jafar Bakhshaei, Dr. Nasrin Bazargan, Dr. Mohammad Hassan Bemanian, Dr. Leila Emami, Dr. Mohammad Reza Fazlollahi, Dr. Zohreh Habibi, Dr. Taha Hojjati Ashrafi, Dr. Ali Kouhi, Dr. Mahboubeh Mansouri, Dr. Fereshteh Rafiei Samani, Dr. Afsaneh Shirani, Dr. Mojdeh Vaziri, Dr. Mehdi Yeganeh, and Dr. Fariborz Zandieh for their role in collecting the data. We are also thankful to laboratory personnel, especially Mrs. Anahita Azimdoust, and to secretarial personnel Miss Tahereh Aghabagheri Kashi, Miss Zahra Arij, Miss Zahra Shobayri, and Mrs Maryam Anvari for their arrangements and administrative efforts.

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Authors and Affiliations

  1. Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran

    NIMA REZAEI, ASGHAR AGHAMOHAMMADI, MOSTAFA MOIN, ZAHRA POURPAK, MASOUD MOVAHEDI, MOHAMMAD GHARAGOZLOU, LIDA ATAROD, BAHRAM MIRSAEID GHAZI, ANNA ISAEIAN, MARYAM MAHMOUDI, KAMRAN ABOLMAALI & ABOLHASSAN FARHOUDI

  2. Department of Infectious Diseases, Masih Daneshvari Hospital, Beheshti University of Medical Sciences, Tehran, Iran

    DAVOUD MANSOURI

  3. Department of Immunology and Allergy, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran

    SABA ARSHI & NASER JAVAHER TARASH

  4. Al-Zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran

    ROYA SHERKAT & HEDAYAT AKBARI

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

    REZA AMIN, ABDOLVAHAB ALBORZI & SARA KASHEF

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

    REZA FARID

  7. Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran

    IRAJ MOHAMMADZADEH

  8. Tabriz University of Medical Sciences, Tabriz, Iran

    MEHRNAZ SADEGHI SHABESTARI

  9. Semnan University of Medical Sciences, Semnan, Iran

    MOHAMMAD NABAVI

  10. Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, No. 62, Dr. Gharib Street, Keshavarz Boulevard, P.O. Box 14185-863, 14194, Tehran, Iran

    NIMA REZAEI

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  1. NIMA REZAEI
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REZAEI, N., AGHAMOHAMMADI, A., MOIN, M. et al. Frequency and Clinical Manifestations of Patients with Primary Immunodeficiency Disorders in Iran: Update from the Iranian Primary Immunodeficiency Registry. J Clin Immunol 26, 519–532 (2006). https://doi.org/10.1007/s10875-006-9047-x

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