Primary Immunodeficiency Disorders: Awareness Survey of Physicians in Iran
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Abstract
Background/objectives: Primary immunodeficiency disorders (PID) are a group of hereditary disorders characterized by various complications. Many patients with PID are undiagnosed, underdiagnosed, or misdiagnosed due to lack of physicians’ awareness, which culminates in increased rates of morbidity and mortality.
Method: Nine states of Iran were chosen for evaluating physicians’ awareness of PID. The population study consisted of pediatricians (specialties and subspecialties), pediatric residents, and general practitioners. A valid and reliable questionnaire was prepared for awareness scoring assessment. We provided physicians with continuing medical education (CME) and evaluated the effect on physicians’ awareness of PID.
Results: Among 794 physicians, 466 general practitioners (GP), 90 pediatric residents, 124 pediatric specialists, and 20 pediatric subspecialists were included in this study. The mean age of participants was 40.96±10.63 years. The mean period of practicing medicine was 12±9.53 years. The mean total knowledge score of participants was 51.30 with a standard deviation of 18.76. Only 161 participants (20.4%) answered more than 2/3 of all questions correctly. The mean scores in the management of PIDs was 66.25±54.55, followed by laboratory findings as 49.57±25.07, clinical symptoms as 54.42 ± 17.85, and associated syndromes as 42.32 ± 28.57. Only 207 physicians completed the CME curricula. Significant improvements were observed in physician’s knowledge after the programs (P <0.0001).
Conclusion: This survey demonstrated that there is a lack of both the knowledge and practice of pediatricians in the field of PID in Iran. Implementation of strategies to raise pediatricians’ awareness and assure the earliest diagnosis, appropriate treatment, and proper care management is critical.
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2. Aghamohammadi A, Farhoudi A, Moin M, Rezaei N, Kouhi A, Pourpak Z, et al. Clinical and immunological features of 65 Iranian patients with common variable immunodeficiency. Clin Diagn Lab Immunol. 2005;12(7):825-32.
3. Yazdani R, Abolhassani H, Asgardoon M, Shaghaghi M, Modaresi M, Azizi G, et al. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders. J Investig Allergol Clin Immunol. 2017;27(4):213-24.
4. Al-Herz W, Bousfiha A, Casanova JL, Chatila T, Conley ME, Cunningham-Rundles C, et al. Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency. Frontiers in immunology. 2014;5:162.
5. Sharifi L, Mirshafiey A, Rezaei N, Azizi G, Magaji Hamid K, Amirzargar AA, et al. The role of toll-like receptors in B-cell development and immunopathogenesis of common variable immunodeficiency. Expert review of clinical immunology. 2016;12(2):195-207.
6. Geha RS, Notarangelo LD, Casanova JL, ChapelH, Conley ME, Fischer A, et al. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. The Journal of allergy and clinical immunology. 2007;120(4):776-94.
7. Kumar A, Teuber SS, Gershwin ME. Current perspectives on primary immunodeficiency diseases. Clinical & developmental immunology. 2006;13(2-4):223-59.
8. Aghamohammadi A, Moein M, Farhoudi A, Pourpak Z, Rezaei N, Abolmaali K, et al. Primary immunodeficiency in Iran: first report of the National Registry of PID in Children and Adults. J Clin Immunol. 2002;22(6):375-80.
9. Rezaei N, Aghamohammadi A, Moin M, Pourpak Z, Movahedi M, Gharagozlou M, et al. Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian Primary Immunodeficiency Registry. J Clin Immunol. 2006;26(6):519-32.
10. Aghamohammadi A, Mohammadinejad P, Abolhassani H, Mirminachi B, Movahedi M, Gharagozlou M, et al. Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry. J Clin Immunol. 2014;34(4):478-90.
11. Aghamohammadi A, Allahverdi A, Abolhassani H, Moazzami K, Alizadeh H, Gharagozlou M, et al. Comparison of pulmonary diseases in common variable immunodeficiency and X-linked agammaglobulinaemia. Respirology. 2010;15(2):289-95.
12. Khodadad A, Aghamohammadi A, Parvaneh N, Rezaei N, Mahjoob F, Bashashati M, et al. Gastrointestinal manifestations in patients with common variable immunodeficiency. Dig Dis Sci. 2007;52(11):2977-83.
13. Abolhassani H, Amirkashani D, Parvaneh N, Mohammadinejad P, Gharib B, Shahinpour S, et al. Autoimmune phenotype in patients with common variable immunodeficiency. J Investig Allergol Clin Immunol. 2013;23(5):323-9.
14. Abolhassani H, Aghamohammadi A, Imanzadeh A, Mohammadinejad P, Sadeghi B, Rezaei N. Malignancy phenotype in common variable immunodeficiency. J Investig Allergol Clin Immunol. 2012;22(2):133-4.
15. Aghamohammadi A, Pouladi N, Parvaneh N, Yeganeh M, Movahedi M, Gharagolou M, et al. Mortality and morbidity in common variable immunodeficiency. J Trop Pediatr. 2007;53(1):32-8.
16. Aghamohammadi A, Montazeri A, Abolhassani H, Saroukhani S, Pourjabbar S, Tavassoli M, et al. Health-related quality of life in primary antibody deficiency. Iran J Allergy Asthma Immunol. 2011;10(1):47-51.
17. Abolhassani H, Asgardoon MH, Rezaei N, Hammarstrom L, Aghamohammadi A. Different brands of intravenous immunoglobulin for primary immunodeficiencies: how to choose the best option for the patient? Expert review of clinical immunology. 2015;11(11):1229-43.
18. Boyle JM, Buckley RH. Population prevalence of diagnosed primaryimmunodeficiency diseases in the United States. J Clin Immunol. 2007;27(5):497-502.
19. Lee PP, Lau YL. Endemic infections in Southeast Asia provide new insights to the phenotypic spectrum of primary immunodeficiency disorders. Asian Pacific journal of allergy and immunology / launched by the Allergy and Immunology Society of Thailand. 2013;31(3):217-26.
20. Matamoros Flori N, Mila Llambi J, Espanol Boren T, Raga Borja S, Fontan Casariego G. Primary immunodeficiency syndrome in Spain: first report of the National Registry in Children and Adults. J Clin Immunol. 1997;17(4):333-9.
21. Al-Herz W, Zainal ME, Salama M, Al-Ateeqi W, Husain K, Abdul-Rasoul M, et al. Primary immunodeficiency disorders: survey of pediatricians in Kuwait. J Clin Immunol. 2008;28(4):379-83.
22. Nourijelyani K, Aghamohammadi A, Salehi Sadaghiani M, Behniafard N, Abolhassani H, Pourjabar S, et al. Physicians awareness on primary immunodeficiency disorders in Iran. Iran J Allergy Asthma Immunol. 2012;11(1):57-64.
23. Notarangelo L, Casanova JL, Conley ME, Chapel H, Fischer A, Puck J, et al. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005. The Journal of allergy and clinical immunology. 2006;117(4):883-96.
24. Yuksek M, Ikinciogullari A, Dogu F, Elhan A, Yuksek N, Reisli I, et al. Primary immune deficiency disease awareness among a group of Turkish physicians. Turkish J Pediatr. 2010;52(4):372-7.
25. Available from: http://www.cdc.gov/genomics/info/conference/P Isynop.htm.
26. Thacker SB, Stroup DF. Future directions for comprehensive public health surveillance and health information systems in the United States. American journal of epidemiology. 1994;140(5):383-97.
27. Brown AS, Gwinn M, Cogswell ME, Khoury MJ. Hemochromatosis-associated morbidity in the United States: an analysis of the National Hospital Discharge Survey, 1979-1997. Genetics in medicine : official journal of the American College of Medical Genetics. 2001;3(2):109-11.
28. McDonnell SM, Witte DL, Cogswell ME, McIntyre R. Strategies to increase detection of hemochromatosis. Annals of internal medicine. 1998;129(11):987-92.
29. Wetterhall SF, Cogswell ME, Kowdley KV. Public health surveillance for hereditary hemochromatosis. Annals of internal medicine. 1998;129(11):980-6.
2. Aghamohammadi A, Farhoudi A, Moin M, Rezaei N, Kouhi A, Pourpak Z, et al. Clinical and immunological features of 65 Iranian patients with common variable immunodeficiency. Clin Diagn Lab Immunol. 2005;12(7):825-32.
3. Yazdani R, Abolhassani H, Asgardoon M, Shaghaghi M, Modaresi M, Azizi G, et al. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders. J Investig Allergol Clin Immunol. 2017;27(4):213-24.
4. Al-Herz W, Bousfiha A, Casanova JL, Chatila T, Conley ME, Cunningham-Rundles C, et al. Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency. Frontiers in immunology. 2014;5:162.
5. Sharifi L, Mirshafiey A, Rezaei N, Azizi G, Magaji Hamid K, Amirzargar AA, et al. The role of toll-like receptors in B-cell development and immunopathogenesis of common variable immunodeficiency. Expert review of clinical immunology. 2016;12(2):195-207.
6. Geha RS, Notarangelo LD, Casanova JL, ChapelH, Conley ME, Fischer A, et al. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. The Journal of allergy and clinical immunology. 2007;120(4):776-94.
7. Kumar A, Teuber SS, Gershwin ME. Current perspectives on primary immunodeficiency diseases. Clinical & developmental immunology. 2006;13(2-4):223-59.
8. Aghamohammadi A, Moein M, Farhoudi A, Pourpak Z, Rezaei N, Abolmaali K, et al. Primary immunodeficiency in Iran: first report of the National Registry of PID in Children and Adults. J Clin Immunol. 2002;22(6):375-80.
9. Rezaei N, Aghamohammadi A, Moin M, Pourpak Z, Movahedi M, Gharagozlou M, et al. Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian Primary Immunodeficiency Registry. J Clin Immunol. 2006;26(6):519-32.
10. Aghamohammadi A, Mohammadinejad P, Abolhassani H, Mirminachi B, Movahedi M, Gharagozlou M, et al. Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry. J Clin Immunol. 2014;34(4):478-90.
11. Aghamohammadi A, Allahverdi A, Abolhassani H, Moazzami K, Alizadeh H, Gharagozlou M, et al. Comparison of pulmonary diseases in common variable immunodeficiency and X-linked agammaglobulinaemia. Respirology. 2010;15(2):289-95.
12. Khodadad A, Aghamohammadi A, Parvaneh N, Rezaei N, Mahjoob F, Bashashati M, et al. Gastrointestinal manifestations in patients with common variable immunodeficiency. Dig Dis Sci. 2007;52(11):2977-83.
13. Abolhassani H, Amirkashani D, Parvaneh N, Mohammadinejad P, Gharib B, Shahinpour S, et al. Autoimmune phenotype in patients with common variable immunodeficiency. J Investig Allergol Clin Immunol. 2013;23(5):323-9.
14. Abolhassani H, Aghamohammadi A, Imanzadeh A, Mohammadinejad P, Sadeghi B, Rezaei N. Malignancy phenotype in common variable immunodeficiency. J Investig Allergol Clin Immunol. 2012;22(2):133-4.
15. Aghamohammadi A, Pouladi N, Parvaneh N, Yeganeh M, Movahedi M, Gharagolou M, et al. Mortality and morbidity in common variable immunodeficiency. J Trop Pediatr. 2007;53(1):32-8.
16. Aghamohammadi A, Montazeri A, Abolhassani H, Saroukhani S, Pourjabbar S, Tavassoli M, et al. Health-related quality of life in primary antibody deficiency. Iran J Allergy Asthma Immunol. 2011;10(1):47-51.
17. Abolhassani H, Asgardoon MH, Rezaei N, Hammarstrom L, Aghamohammadi A. Different brands of intravenous immunoglobulin for primary immunodeficiencies: how to choose the best option for the patient? Expert review of clinical immunology. 2015;11(11):1229-43.
18. Boyle JM, Buckley RH. Population prevalence of diagnosed primaryimmunodeficiency diseases in the United States. J Clin Immunol. 2007;27(5):497-502.
19. Lee PP, Lau YL. Endemic infections in Southeast Asia provide new insights to the phenotypic spectrum of primary immunodeficiency disorders. Asian Pacific journal of allergy and immunology / launched by the Allergy and Immunology Society of Thailand. 2013;31(3):217-26.
20. Matamoros Flori N, Mila Llambi J, Espanol Boren T, Raga Borja S, Fontan Casariego G. Primary immunodeficiency syndrome in Spain: first report of the National Registry in Children and Adults. J Clin Immunol. 1997;17(4):333-9.
21. Al-Herz W, Zainal ME, Salama M, Al-Ateeqi W, Husain K, Abdul-Rasoul M, et al. Primary immunodeficiency disorders: survey of pediatricians in Kuwait. J Clin Immunol. 2008;28(4):379-83.
22. Nourijelyani K, Aghamohammadi A, Salehi Sadaghiani M, Behniafard N, Abolhassani H, Pourjabar S, et al. Physicians awareness on primary immunodeficiency disorders in Iran. Iran J Allergy Asthma Immunol. 2012;11(1):57-64.
23. Notarangelo L, Casanova JL, Conley ME, Chapel H, Fischer A, Puck J, et al. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005. The Journal of allergy and clinical immunology. 2006;117(4):883-96.
24. Yuksek M, Ikinciogullari A, Dogu F, Elhan A, Yuksek N, Reisli I, et al. Primary immune deficiency disease awareness among a group of Turkish physicians. Turkish J Pediatr. 2010;52(4):372-7.
25. Available from: http://www.cdc.gov/genomics/info/conference/P Isynop.htm.
26. Thacker SB, Stroup DF. Future directions for comprehensive public health surveillance and health information systems in the United States. American journal of epidemiology. 1994;140(5):383-97.
27. Brown AS, Gwinn M, Cogswell ME, Khoury MJ. Hemochromatosis-associated morbidity in the United States: an analysis of the National Hospital Discharge Survey, 1979-1997. Genetics in medicine : official journal of the American College of Medical Genetics. 2001;3(2):109-11.
28. McDonnell SM, Witte DL, Cogswell ME, McIntyre R. Strategies to increase detection of hemochromatosis. Annals of internal medicine. 1998;129(11):987-92.
29. Wetterhall SF, Cogswell ME, Kowdley KV. Public health surveillance for hereditary hemochromatosis. Annals of internal medicine. 1998;129(11):980-6.
| Files | ||
| Issue | Vol 2, No 2 (2019) | |
| Section | Review Article | |
| DOI | https://doi.org/10.22034/igj.2019.190165.1015 | |
| Keywords | ||
| Primary immunodeficiency physician Survey awareness | ||
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How to Cite
1.
Asgardoon MH, Rezwanifar MM, Ataeinia B, Bagheri Y. Primary Immunodeficiency Disorders: Awareness Survey of Physicians in Iran. Immunol Genet J. 2019;2(2):44-57.
