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Diagnostic Approach to the Patients With Suspected Primary Immunodeficiency Publisher Pubmed



Tavakol M1, 2 ; Jamee M3 ; Azizi G1, 4 ; Sadri H1, 2 ; Bagheri Y5 ; Zakidizaji M6 ; Mahdavi FS3 ; Jadidiniaragh F7 ; Tajfirooz S8 ; Kamali AN9 ; Aghamahdi F1, 10 ; Noorian S10 ; Kojidi HT10 ; Mosavian M1, 11 Show All Authors
Authors
  1. Tavakol M1, 2
  2. Jamee M3
  3. Azizi G1, 4
  4. Sadri H1, 2
  5. Bagheri Y5
  6. Zakidizaji M6
  7. Mahdavi FS3
  8. Jadidiniaragh F7
  9. Tajfirooz S8
  10. Kamali AN9
  11. Aghamahdi F1, 10
  12. Noorian S10
  13. Kojidi HT10
  14. Mosavian M1, 11
  15. Matani R11
  16. Dolatshahi E12
  17. Porrostami K8
  18. Elahimehr N8
  19. Fatemiabhari M8
  20. Sharifi L13
  21. Arjmand R14
  22. Haghi S15
  23. Zainaldain H4
  24. Yazdani R4
  25. Shaghaghi M16
  26. Abolhassani H17
  27. Aghamohammadi A4
Show Affiliations
Authors Affiliations
  1. 1. Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
  2. 2. Department of Allergy and Clinical Immunology, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran
  3. 3. Student Research Committee, Alborz University of Medical Sciences, Karaj, Iran
  4. 4. Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  5. 5. Clinical Research Development Unit (CRDU), Golestan University of Medical Sciences, 5 azar Hospital, Gorgan, Iran
  6. 6. Legal Medicine Research Center, Legal Medicine Organization, Tehran, Iran
  7. 7. Immunology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
  8. 8. Department of Pediatrics, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran
  9. 9. CinnaGen Medical Biotechnology Research Center, Alborz University of Medical Sciences, Karaj, Iran
  10. 10. Department of Pediatric Endocrinology, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran
  11. 11. Department of Gastroenterology and Hepatology, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran
  12. 12. Department of Rheumatology, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran
  13. 13. Uro-Oncology Research Center, Tehran University of Medical Sciences, Tehran, Iran
  14. 14. Department of Infectious Disease, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran
  15. 15. Department of Hematology & Oncology, School of Medicine, Alborz university of medical sciences, Karaj, Iran
  16. 16. Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins Hospital, Baltimore, MD, United States
  17. 17. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden

Source: Endocrine# Metabolic and Immune Disorders - Drug Targets Published:2020


Abstract

Background and Objective: Primary immunodeficiency diseases (PIDs) are a group of more than 350 disorders affecting distinct components of the innate and adaptive immune systems. In this review, the classic and advanced stepwise approach towards the diagnosis of PIDs are simplified and explained in detail. Results: Susceptibility to recurrent infections is the main hallmark of almost all PIDs. However, non-infectious complications attributable to immune dysregulation presenting with lymphoproliferative and/or autoimmune disorders are not uncommon. Moreover, PIDs could be associated with misleading presentations including allergic manifestations, enteropathies, and malignancies. Conclusion: Timely diagnosis is the most essential element in improving outcome and reducing the morbidity and mortality in PIDs. This wouldn’t be possible unless the physicians keep the diagnosis of PID in mind and be sufficiently aware of the approach to these patients. © 2020 Bentham Science Publishers.
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