肺功能在原发性纤毛功能障碍的自然变异性:从PROVALF-PCD队列的纵向分析
Kewei Zhang1,2, Avni Kant1,2, Mieke Boon3,4
1Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.
ERJ open research
|June 24, 2025
概括
在原发性纤维动力障碍 (PCD) 中,个体内肺功能变异性是显著的. 在稳定的PCD患者中,1秒内强迫呼气体积 (FEV1) 超过25%的变化在生理上是重要的.
科学领域:
- 肺部医学 肺部医学
- 临床研究 临床研究
- 患者变性研究研究.
背景情况:
- 原发性状动力障碍 (PCD) 肺功能变异性不太清楚.
- 对稳定的PCD患者来说,评估螺旋测量中的自然变化至关重要.
研究的目的:
- 在临床稳定的PCD患者中评估1秒内强迫呼气体积 (FEV1) 的个体内变异性.
- 为了区分测试变异性和肺功能的生理变化.
主要方法:
- 关于稳定PCD患者 (PROVALF-PCD) 队列 (2017-2019) 肺功能变异性的前性观察多中心研究.
- 包括252名临床稳定的PCD患者 (≥5年) 与螺旋计数据.
- 使用多层模型和重复性系数 (CR) 分析了FEV1的变异性.
主要成果:
- 相对FEV1变化的正常 (ULN) 上限为25%.
- 在所有灵敏度分析中,测试变异性仍然很高.
- 对于FEV1z分数的重复性系数 (CR) 是1.88.
结论:
- 在稳定的PCD患者中,个体内FEV1变化>25%表明生理相关性.
- 在PCD临床试验中,FEV1可能不是监测肺部健康的敏感终点.
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