Published in:
01-10-2016 | Original Article
Patterns of Primary Immunodeficiency Disorders Among a Highly Consanguineous Population: Cairo University Pediatric Hospital’s 5-Year Experience
Authors:
Nermeen Galal, Safa Meshaal, Rabab Elhawary, Dalia Abd ElAziz, Radwa Alkady, Sohilla Lotfy, Alia Eldash, Jeanette Boutros, Aisha Elmarsafy
Published in:
Journal of Clinical Immunology
|
Issue 7/2016
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Abstract
Introduction
Primary immunodeficiency disorders (PIDs) are heterogeneous disorders that mainly present with severe, persistent, unusual, or recurrent infections in childhood. Reports from different parts of the world indicate a difference between Western and Eastern populations.
Aim
The aim of this study was to report on the different patterns of PIDs and identify subgroup characteristics in a highly consanguineous population in Egypt.
Methods
We performed a retrospective chart review for children below 18 years diagnosed with PID at Cairo University Pediatric Hospital from 2010 to 2014.
Results
Four hundred seventy-six children were diagnosed with PID disorders. Major categories included combined immunodeficiency disorders, which constituted a large proportion (30 %) of cases, along with predominantly antibody disorders (18 %) followed by syndromic combined disorders (16.8 %), phagocytic disorders (13.2 %), immune dysregulation disorders (10.5 %), and autoinflammatory disorders (9 %).
Conclusion
PIDs have different patterns within inbred populations with high consanguinity.