Primary immunodeficiency diseases in Northern Iran

I. Mohammadzadeh; Nima Rezaei; B. Moazzami; J. Ghaffari; A. Aghamohammadi

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Primary immunodeficiency diseases in Northern Iran

I. Mohammadzadeh ^[1] ; N. Rezaei ^[2] ; B. Moazzami ^[1] ; J. Ghaffari ^[3] ; A. Aghamohammadi ^[2]
1. [1] Babol University of Medical Sciences
  
  Babol University of Medical Sciences
  
  Irán
2. [2] Tehran University of Medical Sciences
  
  Tehran University of Medical Sciences
  
  Irán
3. [3] Mazandaran University of Medical Sciences
  
  Mazandaran University of Medical Sciences
  
  Irán
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Localización: Allergologia et immunopathologia: International journal for clinical and investigate allergology and clinical immunology, ISSN-e 1578-1267, ISSN 0301-0546, Vol. 45, Nº. 3, 2017, págs. 244-250
Idioma: inglés
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Resumen
- Introduction Primary immunodeficiency diseases (PID) are a heterogeneous group of inherited disorders, characterised by recurrent severe infections, autoimmunity and lymphoproliferation. Despite impressive progress in identification of novel PID, there is an unfortunate lack of awareness among physicians in identification of patients with PID, especially in non-capital cities of countries worldwide.
  
  Result This study was performed in a single-centre paediatric hospital in Northern Iran during a 21-year period (1994–2015). Ninety-four patients were included in this study. The majority of cases had antibody deficiencies (37.23%), followed by well-defined syndromes with immunodeficiency in 16 (17.02%), phagocytic disorders in 15 patients (15.95%), complement deficiencies in 15 patients (15.95%), immunodeficiencies affecting cellular and humoral immunity in nine patients (9.57%), disease of immune dysregulation in three (3.19%), and defects in intrinsic and innate immunity in one (1.06%).
  
  Conclusion It seems that there are major variations in frequency of different types of PID in different regions of a country. Therefore, reporting local data could provide better ideas to improve the local health care system strategists and quality of care of PID patients.