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

Authors

    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • ASGHAR AGHAMOHAMMADI
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • MOSTAFA MOIN
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • ZAHRA POURPAK
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • MASOUD MOVAHEDI
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • MOHAMMAD GHARAGOZLOU
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • LIDA ATAROD
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • BAHRAM MIRSAEID GHAZI
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • ANNA ISAEIAN
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • MARYAM MAHMOUDI
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • KAMRAN ABOLMAALI
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
  • DAVOUD MANSOURI
    • Department of Infectious Diseases, Masih Daneshvari HospitalBeheshti University of Medical Sciences
  • SABA ARSHI
    • Department of Immunology and AllergyRasoul Akram Hospital, Iran University of Medical Sciences
  • NASER JAVAHER TARASH
    • Department of Immunology and AllergyRasoul Akram Hospital, Iran University of Medical Sciences
  • ROYA SHERKAT
    • Al-Zahra HospitalIsfahan University of Medical Sciences
  • HEDAYAT AKBARI
    • Al-Zahra HospitalIsfahan University of Medical Sciences
  • REZA AMIN
    • Department of Immunology and AllergyNamazi Hospital, Shiraz University of Medical Sciences
  • ABDOLVAHAB ALBORZI
    • Department of Immunology and AllergyNamazi Hospital, Shiraz University of Medical Sciences
  • SARA KASHEF
    • Department of Immunology and AllergyNamazi Hospital, Shiraz University of Medical Sciences
  • REZA FARID
    • Department of Immunology and AllergyMashhad University of Medical Sciences
  • IRAJ MOHAMMADZADEH
    • Amirkola HospitalBabol University of Medical Sciences
  • MEHRNAZ SADEGHI SHABESTARI
    • Tabriz University of Medical Sciences
  • MOHAMMAD NABAVI
    • Semnan University of Medical Sciences
  • ABOLHASSAN FARHOUDI
    • Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical Sciences
Article

DOI: 10.1007/s10875-006-9047-x

Cite this article as:
REZAEI, N., AGHAMOHAMMADI, A., MOIN, M. et al. J Clin Immunol (2006) 26: 519. doi:10.1007/s10875-006-9047-x

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

Copyright information

© Springer Science+Business Media, LLC 2006