Reda Shereen M, Afifi Hanaa M, Amine Mai M
Department of Pediatric Allergy and Immunology, Children's Hospital, Faculty of Medicine, Ain Shams University, 110 El-Merghany Street, Heliopolis, Cairo 11341, Egypt.
J Clin Immunol. 2009 May;29(3):343-51. doi: 10.1007/s10875-008-9260-x. Epub 2008 Nov 11.
Sixty-four primary immunodeficiency patients were registered at the Pediatric Allergy and Immunology Department, Children's Hospital, Ain Shams University, Cairo, Egypt.
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%).
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.
在埃及开罗艾因夏姆斯大学儿童医院的儿科过敏与免疫学部门登记了64例原发性免疫缺陷患者。
主要的抗体缺陷是最常见的类型（35.9%），其次是T细胞和B细胞联合免疫缺陷（29.7%）、其他明确的免疫缺陷综合征（18.7%）、吞噬细胞数量、功能或两者的先天性缺陷（12.5%）以及免疫失调疾病（3.1%）。最常见的疾病是普通可变免疫缺陷（18.7%）。诊断时的平均年龄为29.9个月。近亲结婚率为62.5%。所有类型中均可见反复严重感染。15例患者死于感染（23.4%），其中T细胞和B细胞联合免疫缺陷的死亡率最高（15.6%）。
原发性免疫缺陷疾病在埃及儿童中并不罕见。在我们的队列中观察到的T细胞和B细胞联合免疫缺陷的频率相对高于其他国家。在埃及建立原发性免疫缺陷国家登记册是一项先决条件。
