1. Patterns of Primary Immunodeficiency Disorders Among a Highly Consanguineous Population: Cairo University Pediatric Hospital's 5-Year Experience.
- Author
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Galal, Nermeen, Meshaal, Safa, Elhawary, Rabab, ElAziz, Dalia, Alkady, Radwa, Lotfy, Sohilla, Eldash, Alia, Boutros, Jeanette, and Elmarsafy, Aisha
- Subjects
IMMUNOLOGICAL deficiency syndromes ,CHILDREN'S hospitals ,JUVENILE diseases ,CONSANGUINITY ,DISEASE relapse ,RETROSPECTIVE studies ,DIAGNOSIS - 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. [ABSTRACT FROM AUTHOR]
- Published
- 2016
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