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Long-Term Follow-Up of Ninety Eight Iranian Patients With Primary Immune Deficiency in a Single Tertiary Centre Publisher Pubmed



Nabavi M1 ; Arshi S1 ; Bemanian MH1 ; Aghamohammadi A2 ; Mansouri D3 ; Hedayat M4 ; Nateghian A5 ; Noorbakhsh S6 ; Ehsanipour F6 ; Faranoush M7 ; Shakeri R8 ; Mesdaghi M9 ; Taghvaei B1 ; Ghalebaghi B1, 10 Show All Authors
Authors
  1. Nabavi M1
  2. Arshi S1
  3. Bemanian MH1
  4. Aghamohammadi A2
  5. Mansouri D3
  6. Hedayat M4
  7. Nateghian A5
  8. Noorbakhsh S6
  9. Ehsanipour F6
  10. Faranoush M7
  11. Shakeri R8
  12. Mesdaghi M9
  13. Taghvaei B1
  14. Ghalebaghi B1, 10
  15. Babaie D11
  16. Bahrami A1
  17. Fallahpour M1
  18. Esmaeilzadeh H12
  19. Ali Hamidieh A13
  20. Rekabi M1
  21. Ahmadian J1
  22. Eslami N1
  23. Shokri S1
  24. Afshar M14
  25. Jalali F1
  26. Akbarpour N1
  27. Molatefi R1
  28. Rezaei N2, 15, 16
Show Affiliations
Authors Affiliations
  1. 1. Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran
  2. 2. Research Center for Immunodeficiency, Children's Medical Center, Tehran University of Medical Sciences (TUMS), Tehran, Iran
  3. 3. Department of Internal Medicine, Division of Infectious Diseases and Clinical Immunology, National Research Institute of Tuberculosis and Lung Diseases, Masih-e-Daneshvari Hospital, Shaheed Beheshti University of Medical Sciences (SBUMS), Tehran, Iran
  4. 4. Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States
  5. 5. Department of Infectious Diseases, Ali-Asghar Hospital, IUMS, Tehran, Iran
  6. 6. Research Center of Pediatric Infectious Diseases, Rasool-e-Akram Hospital, IUMS, Tehran, Iran
  7. 7. Department of Pediatric Oncology, Rasool-e-Akram Hospital, IUMS, Tehran, Iran
  8. 8. Digestive Oncology Research Center, Digestive Disease Research Institute, TUMS, Tehran, Iran
  9. 9. Department of Immunology, Mofid Children's Hospital, SBUMS, Tehran, Iran
  10. 10. Research Center of Otolaryngology and Head & Neck Surgery, IUMS, Tehran, Iran
  11. 11. Department of Allergy and Clinical Immunology, Mofid Hospital, SBUMS, Tehran, Iran
  12. 12. Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
  13. 13. Hematology Oncology and Stem Cell Transplantation Research Center, TUMS, Iran
  14. 14. Antimicrobial Resistance Research Center, Rasool-e-Akram Hospital, IUMS, Tehran, Iran
  15. 15. Molecular Immunology Research Center, Department of Immunology, School of Medicine, TUMS, Tehran, Iran
  16. 16. Universal Scientific Education and Research Network (USERN), Tehran, Iran

Source: Allergologia et Immunopathologia Published:2016


Abstract

Purpose The aim was to describe the clinical manifestations, complications and long-term outcome of a cohort of Iranian patients with primary immune deficiency (PID). Method We retrospectively studied the demographic, clinical and immunological characteristics of the PID patients in a single tertiary centre, from January 1989 to July 2014. The patients were classified according to the International Union of Immunological Societies Expert Committee on PID. Results 98 patients were diagnosed with and followed-up for 15 disorders. The mean age at onset and diagnosis and the diagnostic delay were 8 ± 10, 14.2 ± 13.1 and 6.1 ± 7 years, respectively. Parental consanguinity rate was 57%. Predominantly Antibody Deficiency was the most common diagnosis (n = 63), followed by congenital defects of phagocytes (n = 16), combined immunodeficiencies (n = 12), well defined syndromes (n = 4) and defects in innate immunity (n = 3). Recurrent sinopulmonary infection was the most common presentation. Active infections were treated appropriately, in addition to prophylactic therapy with IVIG and antimicrobials. Not all the patients were compliant with prophylactic regimens due to cost and unavailability. One SCID patient underwent successful bone marrow transplantation. The total mortality rate was 19% during the follow-up period (7.8 ± 7.6 years). The mean age of living patients at the time of study was 23 ± 11.7 years. Conclusions Physicians awareness of PID has been rising dramatically in Iran, ensuring an increasing number of patients being diagnosed and treated. More effective treatment services, including health insurance coverage and drug availability are needed to improve the outcome of PID patients. © 2016 SEICAP
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