Article

Journal of Clinical Immunology

, Volume 26, Issue 6, pp 519-532

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

  • NIMA REZAEIAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical SciencesDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences Email author 
  • , ASGHAR AGHAMOHAMMADIAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
  • , MOSTAFA MOINAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
  • , ZAHRA POURPAKAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
  • , MASOUD MOVAHEDIAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
  • , MOHAMMAD GHARAGOZLOUAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
  • , LIDA ATARODAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
  • , BAHRAM MIRSAEID GHAZIAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
  • , ANNA ISAEIANAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
    • , MARYAM MAHMOUDIAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
    • , KAMRAN ABOLMAALIAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences
    • , DAVOUD MANSOURIAffiliated withDepartment of Infectious Diseases, Masih Daneshvari Hospital, Beheshti University of Medical Sciences
    • , SABA ARSHIAffiliated withDepartment of Immunology and Allergy, Rasoul Akram Hospital, Iran University of Medical Sciences
    • , NASER JAVAHER TARASHAffiliated withDepartment of Immunology and Allergy, Rasoul Akram Hospital, Iran University of Medical Sciences
    • , ROYA SHERKATAffiliated withAl-Zahra Hospital, Isfahan University of Medical Sciences
    • , HEDAYAT AKBARIAffiliated withAl-Zahra Hospital, Isfahan University of Medical Sciences
    • , REZA AMINAffiliated withDepartment of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences
    • , ABDOLVAHAB ALBORZIAffiliated withDepartment of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences
    • , SARA KASHEFAffiliated withDepartment of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences
    • , REZA FARIDAffiliated withDepartment of Immunology and Allergy, Mashhad University of Medical Sciences
    • , IRAJ MOHAMMADZADEHAffiliated withAmirkola Hospital, Babol University of Medical Sciences
    • , MEHRNAZ SADEGHI SHABESTARIAffiliated withTabriz University of Medical Sciences
    • , MOHAMMAD NABAVIAffiliated withSemnan University of Medical Sciences
    • , ABOLHASSAN FARHOUDIAffiliated withDepartment of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences

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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.

KEYWORDS

Epidemiology immunological deficiency syndromes infection Iran