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 REZAEI
  • ASGHAR AGHAMOHAMMADI
  • MOSTAFA MOIN
  • ZAHRA POURPAK
  • MASOUD MOVAHEDI
  • MOHAMMAD GHARAGOZLOU
  • LIDA ATAROD
  • BAHRAM MIRSAEID GHAZI
  • ANNA ISAEIAN
  • MARYAM MAHMOUDI
  • KAMRAN ABOLMAALI
  • DAVOUD MANSOURI
  • SABA ARSHI
  • NASER JAVAHER TARASH
  • ROYA SHERKAT
  • HEDAYAT AKBARI
  • REZA AMIN
  • ABDOLVAHAB ALBORZI
  • SARA KASHEF
  • REZA FARID
  • IRAJ MOHAMMADZADEH
  • MEHRNAZ SADEGHI SHABESTARI
  • MOHAMMAD NABAVI
  • ABOLHASSAN FARHOUDI
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

Epidemiologyimmunological deficiency syndromesinfectionIran

Copyright information

© Springer Science+Business Media, LLC 2006

Authors and Affiliations

  • NIMA REZAEI
    • 1
    • 10
  • ASGHAR AGHAMOHAMMADI
    • 1
  • MOSTAFA MOIN
    • 1
  • ZAHRA POURPAK
    • 1
  • MASOUD MOVAHEDI
    • 1
  • MOHAMMAD GHARAGOZLOU
    • 1
  • LIDA ATAROD
    • 1
  • BAHRAM MIRSAEID GHAZI
    • 1
  • ANNA ISAEIAN
    • 1
  • MARYAM MAHMOUDI
    • 1
  • KAMRAN ABOLMAALI
    • 1
  • DAVOUD MANSOURI
    • 2
  • SABA ARSHI
    • 3
  • NASER JAVAHER TARASH
    • 3
  • ROYA SHERKAT
    • 4
  • HEDAYAT AKBARI
    • 4
  • REZA AMIN
    • 5
  • ABDOLVAHAB ALBORZI
    • 5
  • SARA KASHEF
    • 5
  • REZA FARID
    • 6
  • IRAJ MOHAMMADZADEH
    • 7
  • MEHRNAZ SADEGHI SHABESTARI
    • 8
  • MOHAMMAD NABAVI
    • 9
  • ABOLHASSAN FARHOUDI
    • 1
  1. 1.Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical SciencesTehranIran
  2. 2.Department of Infectious Diseases, Masih Daneshvari HospitalBeheshti University of Medical SciencesTehranIran
  3. 3.Department of Immunology and AllergyRasoul Akram Hospital, Iran University of Medical SciencesTehranIran
  4. 4.Al-Zahra HospitalIsfahan University of Medical SciencesIsfahanIran
  5. 5.Department of Immunology and AllergyNamazi Hospital, Shiraz University of Medical SciencesShirazIran
  6. 6.Department of Immunology and AllergyMashhad University of Medical SciencesMashhadIran
  7. 7.Amirkola HospitalBabol University of Medical SciencesBabolIran
  8. 8.Tabriz University of Medical SciencesTabrizIran
  9. 9.Semnan University of Medical SciencesSemnanIran
  10. 10.Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research InstituteTehran University of Medical SciencesTehranIran