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Frequency and Clinical Manifestations of Patients With Primary Immunodeficiency Disorders in Iran: Update From the Iranian Primary Immunodeficiency Registry Publisher Pubmed



Rezaei N1, 10 ; Aghamohammadi A1 ; Moin M1 ; Pourpak Z1 ; Movahedi M1 ; Gharagozlou M1 ; Atarod L1 ; Ghazi BM1 ; Isaeian A1 ; Mahmoudi M1 ; Abolmaali K1 ; Mansouri D2 ; Arshi S3 ; Tarash NJ3 Show All Authors
Authors
  1. Rezaei N1, 10
  2. Aghamohammadi A1
  3. Moin M1
  4. Pourpak Z1
  5. Movahedi M1
  6. Gharagozlou M1
  7. Atarod L1
  8. Ghazi BM1
  9. Isaeian A1
  10. Mahmoudi M1
  11. Abolmaali K1
  12. Mansouri D2
  13. Arshi S3
  14. Tarash NJ3
  15. Sherkat R4
  16. Akbari H4
  17. Amin R5
  18. Alborzi A5
  19. Kashef S5
  20. Farid R6
  21. Mohammadzadeh I7
  22. Shabestari MS8
  23. Nabavi M9
  24. Farhoudi A1
Show Affiliations
Authors Affiliations
  1. 1. Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Department of Infectious Diseases, Masih Daneshvari Hospital, Beheshti University of Medical Sciences, Tehran, Iran
  3. 3. Department of Immunology and Allergy, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
  4. 4. Al-Zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran
  5. 5. Department of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
  6. 6. Department of Immunology and Allergy, Mashhad University of Medical Sciences, Mashhad, Iran
  7. 7. Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran
  8. 8. Tabriz University of Medical Sciences, Tabriz, Iran
  9. 9. Semnan University of Medical Sciences, Semnan, Iran
  10. 10. Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran 14194, No. 62, Dr. G. St., Keshavarz Blvd., Iran

Source: Journal of Clinical Immunology Published:2006


Abstract

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. © 2006 Springer Science+Business Media, LLC.
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