Isfahan University of Medical Sciences

Science Communicator Platform

Stay connected! Follow us on X network (Twitter):
Share this content! On (X network) By
Long-Term Evaluation of a Historical Cohort of Iranian Common Variable Immunodeficiency Patients Publisher Pubmed



Aghamohammadi A1 ; Abolhassani H1, 2 ; Latif A1 ; Tabassomi F1 ; Shokuhfar T1 ; Torabi Sagvand B1 ; Shahinpour S1 ; Mirminachi B1 ; Parvaneh N1 ; Movahedi M3 ; Gharagozlou M3 ; Sherkat R4 ; Amin R5 ; Aleyasin S5 Show All Authors
Authors
  1. Aghamohammadi A1
  2. Abolhassani H1, 2
  3. Latif A1
  4. Tabassomi F1
  5. Shokuhfar T1
  6. Torabi Sagvand B1
  7. Shahinpour S1
  8. Mirminachi B1
  9. Parvaneh N1
  10. Movahedi M3
  11. Gharagozlou M3
  12. Sherkat R4
  13. Amin R5
  14. Aleyasin S5
  15. Faridhosseini R6
  16. Jabbariazad F7
  17. Cheraghi T8
  18. Eslamian MH1, 3
  19. Khalili A1, 3
  20. Kalantari N9
  21. Shafiei A10
  22. Dabbaghzade A1, 3
  23. Khayatzadeh A1, 3
  24. Ebrahimi M1, 3
  25. Razavinejad D1
  26. Bazregari S1, 3
  27. Ebrahimi M1, 3
  28. Ghaffari J11
  29. Bemanian MH12
  30. Behniafard N1, 10
  31. Kashef S13
  32. Mohammadzadeh I14
  33. Hammarstrom L2
  34. Rezaei N1
Show Affiliations
Authors Affiliations
  1. 1. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, 62 Qarib St., Keshavarz Blvd., Tehran, 14194, Iran
  2. 2. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at Karolinska University Hospital Huddinge, Stockholm, Sweden
  3. 3. Department of Allergy and Clinical Immunology, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  4. 4. Acquired Immuno deficiency Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
  5. 5. Allergy Research Center, Allergy and Clinical Immunology Department, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
  6. 6. Immunology Research Center, BuAli Research Institute, Mashhad University of Medical Sciences, Mashhad, Iran
  7. 7. Allergy Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
  8. 8. Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran
  9. 9. Department of Immunology and Allergy, Golestan University of Medical Sciences, Gorgan, Iran
  10. 10. Department of Pediatrics, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
  11. 11. Department of Immunology and Allergy, Mazandaran University of Medical Sciences, Sari, Iran
  12. 12. Department of Immunology and Allergy, Yazd University of Medical Sciences, Yazd, Iran
  13. 13. Department of Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
  14. 14. Non-Communicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, Iran

Source: Expert Review of Clinical Immunology Published:2014


Abstract

Objectives: Common variable immune deficiency (CVID) is the most frequent form of symptomatic primary immunodeficiency disease, characterized by hypogammaglobulinemia, recurrent infections and increased predisposition to autoimmunity and malignancies. The aim of this study was to reconsider important points of previously performed studies on Iranian CVID patients diagnosed and followed from 1984 to 2013. Methods: Diagnosis was made using approved criteria including reductions of serum levels of immunoglobulins and exclusion of well-known single gene defects in individuals with an age >4 years and evidence of specific antibody deficiency. Results: Detailed information on demographic data, survival rates, clinical phenotypes, immunologic and genetic data and treatment of 173 patients are provided. The early onset presentation (74.5%) and rate of consanguineous marriage (61.2%) were considerably higher in our cohort. Our study revealed clinically related correlations regarding consanguinity, the population of naive CD4+T cells and switched-memory B cells, cytokine levels and special genetic factors (including HLA and AID genes). Conclusion: Despite current efforts, more comprehensive studies are needed, especially for classification and investigation of the genetic background and prognostic factors for patients with CVID in order to better managment and followup of patinets. © 2014 Informa UK, Ltd.
Other Related Docs
10. Primary Antibody Deficiency in a Tertiary Referral Hospital: A 30-Year Experiment, Journal of Investigational Allergology and Clinical Immunology (2015)
11. Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency, Journal of Investigational Allergology and Clinical Immunology (2024)
13. Autoimmunity in Primary Antibody Deficiencies, International Archives of Allergy and Immunology (2017)
14. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders, Journal of Investigational Allergology and Clinical Immunology (2017)
15. Role of Rare Immune Cells in Common Variable Immunodeficiency, Pediatric Allergy and Immunology (2022)
16. Role of Apoptosis in the Pathogenesis of Common Variable Immunodeficiency (Cvid), Endocrine, Metabolic and Immune Disorders - Drug Targets (2017)
17. The Use of Immunoglobulin Therapy in Primary Immunodeficiency Diseases, Endocrine, Metabolic and Immune Disorders - Drug Targets (2016)
23. A Review on Defects of Dendritic Cells in Common Variable Immunodeficiency, Endocrine, Metabolic and Immune Disorders - Drug Targets (2017)
25. Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort, Journal of Allergy and Clinical Immunology: In Practice (2019)
29. Approach to the Management of Autoimmunity in Primary Immunodeficiency, Scandinavian Journal of Immunology (2017)
30. Global Systematic Review of Primary Immunodeficiency Registries, Expert Review of Clinical Immunology (2020)
46. Immunodeficiencies Affecting Cellular and Humoral Immunity, Inborn Errors of Immunity: A Practical Guide (2021)