Mohammadzadeh I, Moazzami B, Ghaffari J, Aghamohammadi A, Rezaei N
Noncommunicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Babol, Iran.
Department of Pediatrics, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.
Allergol Immunopathol (Madr). 2017 May-Jun;45(3):244-250. doi: 10.1016/j.aller.2016.11.001. Epub 2017 Feb 22.
Primary immunodeficiency diseases (PID) are a heterogeneous group of inherited disorders, characterised by recurrent severe infections, autoimmunity and lymphoproliferation. Despite impressive progress in identification of novel PID, there is an unfortunate lack of awareness among physicians in identification of patients with PID, especially in non-capital cities of countries worldwide.
This study was performed in a single-centre paediatric hospital in Northern Iran during a 21-year period (1994-2015). Ninety-four patients were included in this study. The majority of cases had antibody deficiencies (37.23%), followed by well-defined syndromes with immunodeficiency in 16 (17.02%), phagocytic disorders in 15 patients (15.95%), complement deficiencies in 15 patients (15.95%), immunodeficiencies affecting cellular and humoral immunity in nine patients (9.57%), disease of immune dysregulation in three (3.19%), and defects in intrinsic and innate immunity in one (1.06%).
It seems that there are major variations in frequency of different types of PID in different regions of a country. Therefore, reporting local data could provide better ideas to improve the local health care system strategists and quality of care of PID patients.
原发性免疫缺陷病（PID）是一组遗传性疾病，具有异质性，其特征为反复严重感染、自身免疫和淋巴细胞增殖。尽管在新型PID的识别方面取得了显著进展，但不幸的是，全球各国尤其是非首都城市的医生对PID患者的识别缺乏认识。
本研究在伊朗北部的一家单中心儿科医院进行，为期21年（1994 - 2015年）。本研究纳入了94例患者。大多数病例存在抗体缺陷（37.23%），其次是明确的免疫缺陷综合征16例（17.02%）、吞噬细胞疾病15例（15.95%）、补体缺陷15例（15.95%）、影响细胞免疫和体液免疫的免疫缺陷9例（9.57%）、免疫失调疾病3例（3.19%）以及固有免疫和先天免疫缺陷1例（1.06%）。
一个国家不同地区不同类型PID的发病率似乎存在很大差异。因此，报告本地数据可为改善当地医疗保健系统策略制定者和PID患者的护理质量提供更好的思路。
