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Global Systematic Review of Primary Immunodeficiency Registries Publisher Pubmed



Abolhassani H1, 2 ; Azizi G3 ; Sharifi L1, 28 ; Yazdani R1 ; Mohsenzadegan M1 ; Delavari S1, 29 ; Sohani M1 ; Shirmast P1 ; Chavoshzadeh Z4 ; Mahdaviani SA5 ; Kalantari A6 ; Tavakol M3 ; Jabbariazad F7 ; Ahanchian H7 Show All Authors
Authors
  1. Abolhassani H1, 2
  2. Azizi G3
  3. Sharifi L1, 28
  4. Yazdani R1
  5. Mohsenzadegan M1
  6. Delavari S1, 29
  7. Sohani M1
  8. Shirmast P1
  9. Chavoshzadeh Z4
  10. Mahdaviani SA5
  11. Kalantari A6
  12. Tavakol M3
  13. Jabbariazad F7
  14. Ahanchian H7
  15. Momen T8
  16. Sherkat R9
  17. Sadeghishabestari M10
  18. Aleyasin S11
  19. Esmaeilzadeh H11
  20. Alherz W12, 13
  21. Bousfiha AA14, 15, 16
  22. Condinoneto A17, 18
  23. Seppanen M19, 20, 21
  24. Sullivan KE22, 23
  25. Hammarstrom L2
  26. Modell V24
  27. Modell F24
  28. Quinn J24
  29. Orange JS24, 25
  30. Aghamohammadi A1, 26, 27

Source: Expert Review of Clinical Immunology Published:2020


Abstract

Introduction: During the last 4 decades, registration of patients with primary immunodeficiencies (PID) has played an essential role in different aspects of these diseases worldwide including epidemiological indexes, policymaking, quality controls of care/life, facilitation of genetic studies and clinical trials as well as improving our understanding about the natural history of the disease and the immune system function. However, due to the limitation of sustainable resources supporting these registries, inconsistency in diagnostic criteria and lack of molecular diagnosis as well as difficulties in the documentation and designing any universal platform, the global perspective of these diseases remains unclear. Areas covered: Published and unpublished studies from January 1981 to June 2020 were systematically reviewed on PubMed, Web of Science and Scopus. Additionally, the reference list of all studies was hand-searched for additional studies. This effort identified a total of 104614 registered patients and suggests identification of at least 10590 additional PID patients, mainly from countries located in Asia and Africa. Molecular defects in genes known to cause PID were identified and reported in 13852 (13.2% of all registered) patients. Expert opinion: Although these data suggest some progress in the identification and documentation of PID patients worldwide, achieving the basic requirement for the global PID burden estimation and registration of undiagnosed patients will require more reinforcement of the progress, involving both improved diagnostic facilities and neonatal screening. © 2020 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.
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