分枝杆菌感染相关儿童原发性免疫缺陷病

Primary immunodeficiency diseases susceptible to mycobacteria in children

原发性免疫缺陷病(PIDs)是一类由单基因突变引起免疫细胞或免疫分子缺陷,最终导致免疫功能降低、缺如或免疫调节功能失衡的疾病。PIDs患儿更易发生感染性疾病,由于不同病原菌感染宿主的免疫机制有所差异,不同类型的PIDs相对易感的病原学种类也不尽相同。目前多项研究指出,某些PIDs患儿更易发生卡介苗感染、重症结核病及非结核分枝杆菌重症感染。现就与分枝杆菌易感密切相关的主要PIDs进行概述,包括慢性肉芽肿病、严重联合免疫缺陷病、孟德尔遗传易感分枝杆菌病及高IgM综合征等,以期为临床医师早期识别此病提供理论基础。

Primary immunodeficiency diseases(PIDs)refer to immune function decrease and deficiency or immune regulation function imbalance resulted from immune cell or immunomolecular defects caused by single gene mutation.Children with PIDs are prone to develop infectious diseases.Because the immune mechanisms of hosts infected by different pathogens infecting vary,different types of PIDs are relatively susceptible to different pathogens.A number of studies have indicated that some PIDs children are more likely to develop Bacilles Calmétte-Guerin infection,severe tuberculosis and non-Tuberculous mycobacteria severe infection.In this review,several major primary immunodeficiency diseases closely related to the susceptibility of mycobacteria were summarized,including chronic granulomatous disease,severe combined immunodeficiency disease,mendelian susceptibility to mycobacterial disease,and high IgM syndrome,in order to provide a theoretical basis for early identification of such diseases.