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

Journal of Clinical Immunology volume 36, pages 649–655 (2016)Cite this article

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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Acknowledgments

The authors wish to acknowledge the following scientists for their valuable support and provision of expertise to aid the diagnosis of our children:

M. Baker, J. Bustamanate, J.L. Casanova, T. Freiberger, R. Geha, B. Grimbacher, J. Litzman, M. Masaad, A. Villa

Author information

Authors and Affiliations

  1. Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt

    Nermeen Galal, Dalia Abd ElAziz, Radwa Alkady, Sohilla Lotfy, Jeanette Boutros & Aisha Elmarsafy

  2. Department of Clinical and Chemical Pathology, Faculty of Medicine, Cairo University, Cairo, Egypt

    Safa Meshaal, Rabab Elhawary & Alia Eldash

Authors
  1. Nermeen Galal
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  2. Safa Meshaal
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  3. Rabab Elhawary
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  4. Dalia Abd ElAziz
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  5. Radwa Alkady
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  6. Sohilla Lotfy
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  7. Alia Eldash
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  8. Jeanette Boutros
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  9. Aisha Elmarsafy
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Corresponding author

Correspondence to Nermeen Galal.

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The authors declare that they have no conflict of interest.

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Cite this article

Galal, N., Meshaal, S., Elhawary, R. et al. Patterns of Primary Immunodeficiency Disorders Among a Highly Consanguineous Population: Cairo University Pediatric Hospital’s 5-Year Experience. J Clin Immunol 36, 649–655 (2016). https://doi.org/10.1007/s10875-016-0314-1

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  • DOI: https://doi.org/10.1007/s10875-016-0314-1

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