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02.08.2016 | Original Article

Patterns of Primary Immunodeficiency Disorders Among a Highly Consanguineous Population: Cairo University Pediatric Hospital’s 5-Year Experience

verfasst von: Nermeen Galal, Safa Meshaal, Rabab Elhawary, Dalia Abd ElAziz, Radwa Alkady, Sohilla Lotfy, Alia Eldash, Jeanette Boutros, Aisha Elmarsafy

Erschienen in: Journal of Clinical Immunology | Ausgabe 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.

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Titel: Patterns of Primary Immunodeficiency Disorders Among a Highly Consanguineous Population: Cairo University Pediatric Hospital’s 5-Year Experience
verfasst von: Nermeen Galal
Safa Meshaal
Rabab Elhawary
Dalia Abd ElAziz
Radwa Alkady
Sohilla Lotfy
Alia Eldash
Jeanette Boutros
Aisha Elmarsafy
Publikationsdatum: 02.08.2016
Verlag: Springer US
Erschienen in: Journal of Clinical Immunology / Ausgabe 7/2016
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-016-0314-1

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Patterns of Primary Immunodeficiency Disorders Among a Highly Consanguineous Population: Cairo University Pediatric Hospital’s 5-Year Experience