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Primary Immunodeficiency Diseases in Egyptian Children: A Single-Center Study

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Abstract

Introduction

Sixty-four primary immunodeficiency patients were registered at the Pediatric Allergy and Immunology Department, Children’s Hospital, Ain Shams University, Cairo, Egypt.

Data

Predominantly antibody deficiencies were the most common category (35.9%) followed by combined T- and B-cell immunodeficiencies (29.7%), other well defined immunodeficiency syndromes (18.7%), congenital defects of phagocyte number, function or both (12.5%), and diseases of immune dysregulation (3.1%). The most frequent disorder was common variable immunodeficiency (18.7%). The mean age at diagnosis was 29.9 months. The consanguinity rate was 62.5%. Recurrent severe infections were seen in all categories. Fifteen patients died (23.4%) from infections with the highest mortality for combined T- and B-cell immunodeficiencies (15.6%).

Conclusions

Primary immunodeficiency disorders are not rare in Egyptian children. The observed frequency of combined T- and B-cell immunodeficiencies in our cohort is relatively higher than other countries. It is a prerequisite to establish a national registry of primary immunodeficiency in Egypt.

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Authors and Affiliations

  1. Department 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

  2. Department of Clinical Pathology, Faculty of Medicine, Ain Shams University, Cairo, Egypt

    Hanaa M. Afifi

  3. Department of Pediatric Allergy and Immunology, Children’s Hospital, Faculty of Medicine, Ain Shams University, Cairo, Egypt

    Mai M. Amine

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  1. Shereen M. Reda
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  3. Mai M. Amine
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Correspondence to Shereen M. Reda.

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Reda, S.M., Afifi, H.M. & Amine, M.M. Primary Immunodeficiency Diseases in Egyptian Children: A Single-Center Study. J Clin Immunol 29, 343–351 (2009). https://doi.org/10.1007/s10875-008-9260-x

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  • DOI: https://doi.org/10.1007/s10875-008-9260-x

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