Primary immunodeficiency diseases in Egyptian children: a single-center study.
BACKGROUND
Sixty-four primary immunodeficiency patients were registered at the Pediatric Allergy and Immunology Department, Children's Hospital, Ain Shams University, Cairo, Egypt.


METHODS
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.
Reda
Shereen M
SM
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@gmail.com
Afifi
Hanaa M
HM
Amine
Mai M
MM
D002175:Candida / Q000276:immunology* / Q000472:pathogenicity; D002177:Candidiasis / Q000150:complications / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003241:Consanguinity; D003967:Diarrhea; D004534:Egypt; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000453:epidemiology* / Q000276:immunology* / Q000503:physiopathology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009894:Opportunistic Infections / Q000150:complications / Q000276:immunology*; D010033:Otitis Media; D011014:Pneumonia; D015995:Prevalence; D012042:Registries; D016019:Survival Analysis; D014774:Virulence