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Primary immunodeficiency diseases in Egyptian children: a single-center study
Shereen M Reda1, Hanaa M Afifi, Mai M Amine
1Department of Pediatric Allergy and Immunology, Children's Hospital, Faculty of Medicine, Ain Shams University, 110 El-Merghany Street, Heliopolis, Cairo 11341, Egypt. shereen.m.reda@gmail.com
Journal of Clinical Immunology
|November 13, 2008
Summary
Primary immunodeficiency disorders are common in Egyptian children, with antibody deficiencies most frequent. Combined T- and B-cell deficiencies showed a higher prevalence than in other countries, indicating a need for a national registry.
Area of Science:
- Pediatric Allergy and Immunology
- Clinical Immunology
Background:
- Study conducted at Children's Hospital, Ain Shams University, Cairo, Egypt.
- 64 pediatric patients with primary immunodeficiency disorders (PIDs) were registered.
Purpose of the Study:
- To determine the frequency and characteristics of PIDs in Egyptian children.
- To highlight the need for a national PID registry in Egypt.
Main Methods:
- Retrospective analysis of registered pediatric PID patients.
- Classification of PIDs based on established categories.
- Analysis of demographic data, clinical presentation, and outcomes.
Main Results:
- Predominantly antibody deficiencies (35.9%) were most common, followed by combined T- and B-cell immunodeficiencies (29.7%).
- Common variable immunodeficiency was the most frequent specific disorder (18.7%).
- High consanguinity rate (62.5%) and significant mortality (23.4%) due to infections were observed.
Conclusions:
- Primary immunodeficiency disorders are not rare in Egyptian children.
- The observed frequency of combined T- and B-cell immunodeficiencies is relatively higher than in international cohorts.
- Establishing a national registry for PIDs in Egypt is a prerequisite for better management and research.
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