Shereen M Reda1, Hanaa M Afifi, Mai M Amine. 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@gmail.com
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.
INTRODUCTION: Sixty-four primary immunodeficiencypatients 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.
Authors: A S Grumach; A J Duarte; R Bellinati-Pires; A C Pastorino; C M Jacob; C L Diogo; A Condino-Neto; M Kirschfink; M M Carneiro-Sampaio Journal: J Clin Immunol Date: 1997-07 Impact factor: 8.317
Authors: M Ballow; L Notarangelo; B Grimbacher; C Cunningham-Rundles; M Stein; M Helbert; B Gathmann; G Kindle; A K Knight; H D Ochs; K Sullivan; J L Franco Journal: Clin Exp Immunol Date: 2009-12 Impact factor: 4.330