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Primary Immunodeficiency Disorders in Iran: Update and New Insights From the Third Report of the National Registry Publisher Pubmed



Aghamohammadi A1 ; Mohammadinejad P1 ; Abolhassani H1 ; Mirminachi B1 ; Movahedi M2 ; Gharagozlou M2 ; Parvaneh N1 ; Zeiaee V3 ; Mirsaeedghazi B4 ; Chavoushzadeh Z5 ; Mahdaviani A5 ; Mansouri M5 ; Yousefzadegan S1 ; Sharifi B1 Show All Authors
Authors
  1. Aghamohammadi A1
  2. Mohammadinejad P1
  3. Abolhassani H1
  4. Mirminachi B1
  5. Movahedi M2
  6. Gharagozlou M2
  7. Parvaneh N1
  8. Zeiaee V3
  9. Mirsaeedghazi B4
  10. Chavoushzadeh Z5
  11. Mahdaviani A5
  12. Mansouri M5
  13. Yousefzadegan S1
  14. Sharifi B1
  15. Zandieh F4
  16. Hedayat E1
  17. Nadjafi A1
  18. Sherkat R6
  19. Shakerian B6
  20. Sadeghishabestari M7
  21. Farid Hosseini R8
  22. Jabbariazad F8
  23. Ahanchian H8
  24. Behmanesh F8
  25. Zandkarimi M8
  26. Shirkani A8
  27. Cheraghi T9
  28. Fayezi A10
  29. Mohammadzadeh I11
  30. Amin R12
  31. Aleyasin S12
  32. Moghtaderi M12
  33. Ghaffari J13
  34. Arshi S14
  35. Javahertrash N14
  36. Nabavi M14
  37. Bemanian MH14
  38. Shafiei A14
  39. Kalantari N15
  40. Ahmadiafshar A16
  41. Khazaei HA17
  42. Atarod L18
  43. Rezaei N1, 19

Source: Journal of Clinical Immunology Published:2014


Abstract

Background: Primary immunodeficiency disorders (PID) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections and increased susceptibility to malignancies, lymphoproliferative and autoimmune conditions. National registries of PID disorders provide epidemiological data and increase the awareness of medical personnel as well as health care providers. Methods: This study presents the demographic data and clinical manifestations of Iranian PID patients who were diagnosed from March 2006 till the March of 2013 and were registered in Iranian PID Registry (IPIDR) after its second report of 2006. Results: A total number of 731 new PID patients (455 male and 276 female) from 14 medical centers were enrolled in the current study. Predominantly antibody deficiencies were the most common subcategory of PID (32.3 %) and were followed by combined immunodeficiencies (22.3 %), congenital defects of phagocyte number, function, or both (17.4 %), well-defined syndromes with immunodeficiency (17.2 %), autoinflammatory disorders (5.2 %), diseases of immune dysregulation (2.6 %), defects in innate immunity (1.6 %), and complement deficiencies (1.4 %). Severe combined immunodeficiency was the most common disorder (21.1 %). Other prevalent disorders were common variable immunodeficiency (14.9 %), hyper IgE syndrome (7.7 %), and selective IgA deficiency (7.5 %). Conclusions: Registration of Iranian PID patients increased the awareness of medical community of Iran and developed diagnostic and therapeutic techniques across more parts of the country. Further efforts must be taken by increasing the coverage of IPIDR via electronically registration and gradual referral system in order to provide better estimation of PID in Iran and reduce the number of undiagnosed cases. © 2014 Springer Science+Business Media.
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