St. James's Hospital and Trinity College Dublin, Dublin, Republic of Ireland.
Belfast Health and Social Care Trust, Belfast, Northern Ireland.
J Clin Immunol. 2022 Aug;42(6):1293-1299. doi: 10.1007/s10875-022-01274-w. Epub 2022 May 23.
The epidemiology of inborn errors of immunity (IEI) in the Republic of Ireland was first published in 2005 but has not been updated since. IEI prevalence data from Northern Ireland was last published in 2018. Using data from the United Kingdom Primary Immune Deficiency (UKPID) and European Society for Immunodeficiencies (ESID) registries, we reviewed all registered cases of IEI affecting adult patients ≥ 18 years of age from the two largest immunology specialist centres in Northern Ireland and the Republic of Ireland, respectively and calculated the combined minimum adult prevalence of IEI on the island of Ireland for the first time. We also recorded data pertaining to presenting symptoms of IEI, diagnostic delay, immunoglobulin data, and genetic testing, as well as briefly reporting data pertaining to secondary immunodeficiency in both countries. As of 1 May 2020, we identified a minimum adult IEI prevalence in Ireland of 8.85/100,000 population.
爱尔兰共和国的先天性免疫缺陷（IEI）流行病学情况于 2005 年首次公布，但此后并未更新。北爱尔兰的 IEI 患病率数据最后一次公布于 2018 年。利用来自英国原发性免疫缺陷（UKPID）和欧洲免疫缺陷学会（ESID）登记处的数据，我们分别对北爱尔兰和爱尔兰共和国两个最大的免疫学专家中心登记的所有成年患者（≥18 岁）的 IEI 病例进行了审查，并首次计算了爱尔兰岛的 IEI 合并最低成年患病率。我们还记录了与 IEI 表现症状、诊断延迟、免疫球蛋白数据和基因检测相关的数据，以及简要报告了两国的继发性免疫缺陷数据。截至 2020 年 5 月 1 日，我们确定爱尔兰的成人 IEI 患病率最低为 8.85/100000 人。
