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Primary Immunodeficiency in Iran: First Report of the National Registry of Pid in Children and Adults Publisher Pubmed



Aghamohammadi A1 ; Moein M1 ; Farhoudi A1 ; Pourpak Z1 ; Rezaei N1 ; Abolmaali K1 ; Movahedi M1 ; Gharagozlou M1 ; Ghazi BMS1 ; Mahmoudi M1 ; Mansouri D2 ; Arshi S3 ; Trash NJ3 ; Akbari H4 Show All Authors
Authors
  1. Aghamohammadi A1
  2. Moein M1
  3. Farhoudi A1
  4. Pourpak Z1
  5. Rezaei N1
  6. Abolmaali K1
  7. Movahedi M1
  8. Gharagozlou M1
  9. Ghazi BMS1
  10. Mahmoudi M1
  11. Mansouri D2
  12. Arshi S3
  13. Trash NJ3
  14. Akbari H4
  15. Sherkat R4
  16. Hosayni RF5
  17. Hashemzadeh A5
  18. Mohammadzadeh I6
  19. Amin R7
  20. Kashef S7
  21. Alborzi A8
  22. Karimi A8
  23. Khazaei H9
Show Affiliations
Authors Affiliations
  1. 1. Department of Clinical Pediatric Immunology, Children's Medical Center Tehran, University of Medical Sciences, Tehran, Iran
  2. 2. Department of Infectious Disease, Masih Daneshvari Hospital Beheshti, University of Medical Sciences, Tehran, Iran
  3. 3. Department of Immunology and Allergy, Al-rasoul Hospital, University of Medical Sciences, Tehran, Iran
  4. 4. Department of Clinical Pediatric Immunology, Al-Zahara Hospital. Isfahan, University of Medical Sciences, Isfahan, Iran
  5. 5. Department of Immunology and Allergy, University of Medical Sciences, Mashhad, Mashhad, Iran
  6. 6. Department of Clinical Pediatric Immunology, University of Medical Sciences, Babol, Babol, Iran
  7. 7. Department of Immunology and Allergy, Nemazi Hospital, University of Medical Sciences, Shiraz, Iran
  8. 8. Department of Pediatric Infectious Diseases, Nemazi Hospital, University of Medical Sciences, Shiraz, Shiraz, Iran
  9. 9. Department of Immunology, University of Medical Sciences, Zahedan, Zahedan, Iran

Source: Journal of Clinical Immunology Published:2002


Abstract

Epidemiological studies have shown wide geographical and racial variation in the prevalence and patterns of immunodeficiency disorders. To determine the frequency of primary immunodeficiencies (PID) in Iran, the Iranian Primary Immunodeficiency Registry (IPIDR) was organized in 1999. We extracted the patient's data, by using a uniform questionnaire from their hospital records. The diagnosis of patients was based on WHO criteria. By now, 440 patients with PID, who were observed during a period of 20 years, have been registered in our registry. Among these patients, the following frequencies were found: predominantly antibody deficiency in 45.9% of patients (n = 202), phagocytic disorders in 29.09% (n = 128), T-cell disorders in 24.31% (n = 107), and complement deficiencies in 0.68% (n = 3). Common variable immunodeficiency was the most frequent disorder (n = 98), followed by chronic granulomatous disease (n = 86), ataxia telangiectasia (n = 48), x-linked agammaglobulinemia (n = 45), selective IgA deficiency (n = 42), combined immunodeficiency (n = 15), and severe combined immunodeficiency (n = 14). This study revealed that antibody deficiencies is the most frequently diagnosed primary immunodeficiency disorder in our patients, which is similar to that observed in other registries. A comparative study shows some differences between our results and other registries.
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