Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran, and the University of Medical Science, Tehran, Iran.
Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran, and the University of Medical Science, Tehran, Iran; Medical Genetics Network (Megene), Universal Scientific Education and Research Network (USERN), Tehran, Iran.
Allergol Immunopathol (Madr). 2020 Nov-Dec;48(6):729-737. doi: 10.1016/j.aller.2020.04.004. Epub 2020 Oct 25.
Bacille Calmette-Guerin (BCG) vaccination has a great impact on the prevention of severe complications of tuberculosis. However, in patients with primary immunodeficiencies (PID), it can lead to severe complications such as severe combined immunodeficiency, chronic granulomatous disease, and Mendelian susceptibility to mycobacterial disease. This study highlights the demographics, clinical complications and laboratory parameters among PID patients associated with BCG vaccination side effects.
One hundred and thirty-seven PID patients with BCGosis were evaluated in this study, based on the complications following BCG vaccination.
The mean age of the patients with BCG complications at the time of the first visit was five years. The within-group comparison of patients showed a highly significant incidence of pneumonia and hepatomegaly in severe combined immunodeficiency patients. Furthermore, the immunologic data showed an increase in the overall rates of lymphocytes such as CD3+, CD4+ and CD8 + T cells in Mendelian susceptibility to mycobacterial disease patients. The level of immunoglobulins has also increased in chronic granulomatous disease patients.
The high rate of undiagnosed PIDs predisposes individuals to a high risk of severe side effects as a result of BCG vaccination, as well as infants that are less than one month of age. Therefore, there is a need for early screening and diagnosis of PIDs before exposing unknown PID status patients to BCG vaccination. The benefits of screening and early diagnosis of PID cannot be overemphasized, especially in patients with a previous family history of immunodeficiency.
卡介苗（BCG）接种对预防结核病的严重并发症有很大影响。然而，在原发性免疫缺陷病（PID）患者中，它可能导致严重并发症，如严重联合免疫缺陷、慢性肉芽肿病和孟德尔易感性分枝杆菌病。本研究强调了与卡介苗接种副作用相关的 PID 患者的人口统计学、临床并发症和实验室参数。
本研究基于卡介苗接种后的并发症，评估了 137 例 PID 患者的 BCG 病。
发生 BCG 并发症的患者首次就诊时的平均年龄为 5 岁。组内比较显示，严重联合免疫缺陷患者肺炎和肝肿大的发生率极高。此外，免疫数据显示孟德尔易感性分枝杆菌病患者的总淋巴细胞（如 CD3+、CD4+和 CD8+T 细胞）比率增加。慢性肉芽肿病患者的免疫球蛋白水平也增加。
未确诊 PID 的高发病率使个体因 BCG 接种而面临严重副作用的高风险，以及不到一个月大的婴儿。因此，在将未知 PID 状态的患者暴露于 BCG 接种之前，需要对 PID 进行早期筛查和诊断。强调筛查和早期诊断 PID 的益处是不过分的，特别是在有免疫缺陷家族史的患者中。
