Vaccination for Patients with Inborn Errors of Immunity: a Nationwide Survey in Japan
Vaccination for Patients with Inborn Errors of Immunity: a Nationwide Survey in Japan
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DOI：
10.1007/s10875-021-01160-x
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发表时间：
2021-10-27
影响因子：
9.1
通讯作者：
Takada, Hidetoshi
中科院分区：
文献类型：
--
作者：
Hosaka, Sho;Kido, Takahiro;Takada, Hidetoshi
我们在日本进行了10年来第二次全国性的先天性免疫缺陷（IEI）调查，重点是IEI患者对传染病的保护措施。向全国各医疗部门发送了调查表，共报告了1307名患者。IEI的患病率为2.2例/100，000人，与之前的全国性研究相当。最常见的疾病类别是自身炎症性疾病（25%），其次是抗体缺陷（24%）和吞噬细胞数量或功能的先天性缺陷（16%）。我们发现，相当数量的患者接种了禁忌疫苗，主要是因为患者在接种疫苗时未被诊断为IEI。关于卡介苗接种禁忌的疾病，43%的患者实际接受了卡介苗接种，其中14%发生了与卡介苗相关的感染。BCG相关感染主要见于CGD和MSMD患者。为了防止IEI患者接受不适当的疫苗，需要对父母和医生进行持续教育，沿着新生儿筛查的扩大，但在疫苗接种现场筛查IEI的努力仍然很重要。
We conducted a nationwide survey of inborn errors of immunity (IEI) in Japan for the second time in 10 years, focusing on protective measures for IEI patients against infectious diseases. Questionnaires were sent to various medical departments nationwide, and a total of 1307 patients were reported. The prevalence of IEI was 2.2 patients per 100,000 population, which was comparable with the previous nationwide study. The most common disease category was autoinflammatory disorders (25%), followed by antibody deficiencies (24%) and congenital defects of phagocyte number or function (16%). We found that a significant number of patients received contraindicated vaccines, principally because the patients were not diagnosed with IEI by the time of the vaccination. Regarding diseases for which BCG vaccination is contraindicated, 43% of patients had actually received BCG, of which 14% developed BCG-related infections. BCG-related infections were mainly observed among patients with CGD and MSMD. In order to prevent IEI patients from receiving inadequate vaccines, continuous education to parents and physicians is needed, along with the expansion of newborn screening, but efforts to screen IEI at the site of vaccination also remain important.