在先天性免疫错误中死亡率和死亡原因:系统性审查和元分析
Saba Fekrvand1, Zahra Hamidi Esfahani1, Mohammadmehdi Yarahmadi2
1Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran.
Mutation research. Reviews in mutation research
|September 28, 2025
概括
天生的免疫错误 (IEI) 患者面临着24%的死亡率,明显高于一般人群. 感染和移植并发症是导致死亡的主要原因,强调需要更好地管理IEI.
科学领域:
- 免疫学 免疫学 免疫学
- 遗传学 是一个遗传学.
- 临床医学 临床医学
背景情况:
- 免疫的先天性错误 (IEI) 与严重的并发症和增加的死亡率有关.
- 延迟诊断和错误诊断加剧IEI患者的死亡风险.
研究的目的:
- 系统地审查和对IEI患者的死亡原因进行元分析.
- 为了确定不同类型的IEI的死亡率.
主要方法:
- 在Embase,科学网,PubMed和Scopus的综合文献搜索到2024年7月.
- 包括12581名已故IEI患者进行元分析.
主要成果:
- 整体IEI死亡率为24.0%,大约是全球平均水平的27倍.
- 严重的综合免疫缺陷,慢性颗粒状体疾病和心动性-脑膜异位症是死者最多的病例.
- 家庭血细胞性淋巴细胞细胞瘤显示出最高的死亡率 (49.0%).
- 感染 (34.3%),移植相关死亡率 (27.5%) 和肺部并发症 (11.4%) 是主要的死亡原因.
- COVID-19占感染相关死亡人数的10.8%.
结论:
- 特定的IEI类型,包括免疫调节失调,细胞免疫缺陷和细胞功能障碍,与更高的死亡率有关.
- 迫切需要改善IEI高风险人群的管理策略.
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