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Primary Antibody Deficiency in a Tertiary Referral Hospital: A 30-Year Experiment Pubmed



Mohammadinejad P1 ; Pourhamdi S1 ; Abolhassani H1, 2 ; Mirminachi B1 ; Havaei A1 ; Masoom SN1 ; Sadeghi B1 ; Ghajar A1 ; Afarideh M1 ; Parvaneh N1 ; Mirsaeedghazi B3 ; Movahedi M4 ; Gharagozlou M4 ; Chavoushzadeh Z5 Show All Authors
Authors
  1. Mohammadinejad P1
  2. Pourhamdi S1
  3. Abolhassani H1, 2
  4. Mirminachi B1
  5. Havaei A1
  6. Masoom SN1
  7. Sadeghi B1
  8. Ghajar A1
  9. Afarideh M1
  10. Parvaneh N1
  11. Mirsaeedghazi B3
  12. Movahedi M4
  13. Gharagozlou M4
  14. Chavoushzadeh Z5
  15. Mahdaviani A6
  16. Zandieh F3
  17. Sherkat R7
  18. Sadeghishabestari M8
  19. Faridhosseini R9
  20. Jabbariazad F9
  21. Ahanchian H9
  22. Zandkarimi M9
  23. Cherghi T10
  24. Fayezi A11
  25. Mohammadzadeh I12
  26. Amin R13
  27. Aleyasin S13
  28. Moghtaderi M13
  29. Ghaffari J14
  30. Bemanian M15
  31. Shafiei A3, 15
  32. Kalantari N16
  33. Ahmadiafshar A17
  34. Khazaei HA18
  35. Mohammadi J19
  36. Nabavi M20
  37. Rezaei N1, 21
  38. Aghamohammadi A1
Show Affiliations
Authors Affiliations
  1. 1. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at Karolinska University Hospital Huddinge, Stockholm, Sweden
  3. 3. Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran
  4. 4. Department of Allergy and Clinical Immunology, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  5. 5. Pediatric Infectious Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  6. 6. Pediatric Respiratory Disease Research Center, National Research Institute of Tuberculosis and Lung Disease, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  7. 7. Acquired Immunodeficiency Research Center, Al-Zahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran
  8. 8. Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran
  9. 9. Allergy Research Center, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
  10. 10. Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran
  11. 11. Department of Immunology and Allergy, Ahvaz University of Medical Sciences, Ahvaz, Iran
  12. 12. Noncommunicable Pediatric Diseases Research Center, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran
  13. 13. Department of Pediatric Immunology and Allergy, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
  14. 14. Department of Pediatrics, Mazandaran University of Medical Sciences, Sari, Iran
  15. 15. Department of Immunology and Allergy, Yazd University of Medical Sciences, Yazd, Iran
  16. 16. Department of Immunology and Allergy, Golestan University of Medical Sciences, Gorgan, Iran
  17. 17. Department of Immunology and Allergy, Ghazvin University of Medical Sciences, Ghazvin, Iran
  18. 18. Department of Immunology and Hematology, Zahedan Medical Sciences University, Zahedan, Iran
  19. 19. Department of Life Science, University of Tehran, Tehran, Iran
  20. 20. Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
  21. 21. Molecular Immunology Research Center and Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

Source: Journal of Investigational Allergology and Clinical Immunology Published:2015


Abstract

Background: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. Objectives: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. Materials and Methods: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children’s Medical Center, Tehran, Iran. Results: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. Conclusions: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively. © 2015 Esmon Publicidad.
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