免疫的先天性错误:表现,治疗和结果 - - 一份ESID注册表1994-2024年关于30628名患者的报告
Gerhard Kindle1,2,3, Mickaël Alligon4,3, Michael H Albert5
1Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
欧洲免疫缺陷病学会 (ESID-R) 患者登记处是免疫缺陷 (IEI) 遗传错误的主要全球资源. 它提供了关于IEI诊断,治疗和生存趋势的关键现实数据,推动了研究和患者护理.
科学领域:
- 免疫学 免疫学 免疫学
- 遗传学 遗传学 是一个
- 临床医学 临床医学
背景情况:
- 欧洲免疫缺陷病患者登记处 (ESID-R) 成立于1994年.
- 它是世界上最大的免疫天生的错误 (IEI) 数据库之一.
- IEI是与感染,自身免疫,炎症,过敏和恶性瘤有关的遗传性疾病.
研究的目的:
- 审查ESID-R的结构,数据内容和影响.
- 突出注册机构在推进IEI研究和患者护理方面的作用.
- 在跨国背景下展示注册表对基于证据的发现的贡献.
主要方法:
- 从33个国家的194个中心收集数据.
- 包括30628名患者数据集,包括全面的临床和治疗信息.
- 分析了15个子研究中的13550个病例的数据.
主要成果:
- ESID-R包含有关IEI诊断,遗传原因和临床表现的广泛数据.
- 分析揭示了IEI患者的现实治疗结果和生存趋势.
- 该注册表已经支持了84个同行评审的出版物,其引用率很高.
结论:
- 欧洲兴业开发中心-R是IEI研究的重要全球合作平台.
- 它对理解和改善患有天生的免疫错误的个体的护理作出了重大贡献.
- 该注册表的数据推动了IEI管理和治疗的基于证据的进步.
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