免疫的先天性错误的全球研究格局:一个文献计量分析 (1991-2025)
Qibin Wu1, Jingxian Gao2, Yinglin Yuan3
1Organ Transplant Center, Translational Clinical Immunology key Laboratory of Sichuan Province, Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China, No. 32, West Second Section, 1st Ring Road, Qingyang District, Chengdu, 610072, China.
Orphanet journal of rare diseases
|January 24, 2026
概括
免疫的先天性错误 (IEI) 研究已经显著扩大,美国在全球输出方面处于领先地位. 维持IEI的进展可能需要基因编辑的进步.
科学领域:
- 免疫学和遗传学
- 医学研究 医学研究
背景情况:
- 免疫的先天性错误 (IEI) 单独罕见,但集体显著,分类疾病从数十个增长到559个实体.
- 治疗方面的进步,包括免疫球蛋白替代和基因疗法,推动了这种扩张.
研究的目的:
- 为全球免疫的先天性错误 (IEI) 研究环境提供全面的文献分析.
- 绘制35年来研究趋势,重点和治疗前沿的地图.
主要方法:
- 从1991年到2025年使用Web of Science核心集合对7455篇出版物的图书统计分析.
- 使用Bibliometrix,VOSviewer和CiteSpace进行数据分析和可视化.
主要成果:
- 年刊增长10.27%,H指数高 (173).
- 美国在IEI的研究中占主导地位,贡献了36.3%的出版物.
- 关键的研究领域包括免疫缺陷机制,诊断,SCID,CVID,APDS以及HSCT和基因疗法等疗法.
结论:
- 国际工业研究所的研究领域显示出高H指数和广泛的范围,但在过去五年中增长趋于稳定.
- 持续增长可能需要变革性的进步,特别是基因编辑技术.
- 在新生儿查实施和生活质量指标方面发现了关键差距.
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