呼吸功能,存活率和NIV患病率随着时间的推移在ALS - 一个精确的ALS研究
Stefan Sennfält1,2, Ammar Al-Chalabi3,4, Alejandro Caravaca Puchades5
1Department of Neurology, Karolinska University Hospital, Stockholm, Sweden.
概括
随着肌缩侧面硬化症 (ALS) 的进展,呼吸功能下降,影响生存. 非侵入性通风 (NIV) 使用随着呼吸功能恶化而增加,但其引入在患者之间有所不同.
科学领域:
- 神经学 神经学
- 肺部病理学 肺部病理学
- 流行病学 流行病学
背景情况:
- 肌缩侧面硬化症 (ALS) 是一种进展性神经退行性疾病.
- 呼吸功能下降是ALS患者生存的主要决定因素.
研究的目的:
- 描述ALS中的呼吸功能轨迹.
- 为了确定非侵入性通风 (NIV) 在ALS患者中使用的流行率.
- 分析呼吸功能,NIV和存活率之间的关系.
主要方法:
- 来自6个欧洲队列中的3449名ALS患者的前性数据.
- 预测的生命能力百分比 (VC%) 和ALS功能评级尺度修订 (ALSFRS-R) 的分析.
- 调查遗传状态 (C9orf72,SOD1,FUS,TARDBP) 和与生存有关的NIV使用情况.
主要成果:
- 随着时间的推移,中位数VC%显著下降,平均每年下降24个百分点.
- 与一般功能障碍相比,呼吸道症状增加 (ALSFRS-R).
- 与VC%相关的NIV患病率:>80% (3%),50-80% (15%),<50% (25%).NIV患病率与VC%相关的是:>80% (3%),50-80% (15%),<50% (25%).NIV患病率与VC%相关的是:>80% (3%),50-80% (15%),<50% (25%).
结论:
- 呼吸功能恶化是ALS进展的关键特征.
- 呼吸功能与患者的生存率密切相关.
- 对于呼吸功能受损的患者,NIV的实施存在显著的变化.
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