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Published on: May 29, 2020
Primary immunodeficiency diseases in Northern Iran
I Mohammadzadeh1, B Moazzami1, J Ghaffari2
1Noncommunicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Babol, Iran.
This study highlights significant variations in primary immunodeficiency diseases (PID) types across regions. Understanding local PID prevalence is crucial for improving healthcare strategies and patient care globally.
Area of Science:
- Immunology
- Genetics
- Pediatrics
Background:
- Primary immunodeficiency diseases (PID) are inherited disorders causing recurrent infections, autoimmunity, and lymphoproliferation.
- Physician awareness of PID identification remains low, particularly in non-capital regions worldwide.
Purpose of the Study:
- To analyze the frequency and types of PID diagnosed over a 21-year period in a pediatric hospital in Northern Iran.
- To inform local healthcare strategies by reporting regional PID data.
Main Methods:
- Retrospective analysis of 94 pediatric patients diagnosed with PID between 1994 and 2015.
- Classification of PID based on affected immune system components (antibody, cellular, phagocytic, complement, etc.).
Main Results:
- Antibody deficiencies were the most common PID type (37.23%).
- Other significant categories included well-defined syndromes (17.02%), phagocytic disorders (15.95%), and complement deficiencies (15.95%).
- Defects in intrinsic/innate immunity and immune dysregulation were less frequent.
Conclusions:
- Significant regional variations in PID types necessitate local data reporting.
- Local data is essential for enhancing healthcare system strategies and improving the quality of care for PID patients.
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