Abstract：
UNASSIGNED: To systematically assess decline in respiratory measures in amyotrophic lateral sclerosis (ALS) and to examine the impact of sex, disease onset type and baseline morbidity on progression.
UNASSIGNED: The REVEALS study (Registry of Endpoints and Validated Experiences in ALS) was conducted between April 2018 and February 2021 in six European ALS centers. Slow and forced vital capacity (S/FVC), sniff nasal inspiratory pressure (SNIP), peak cough flow, amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R), and respiratory morbidity were collected. Data were analyzed using a Bayesian multiple outcomes random effects model.
UNASSIGNED: Two hundred and eighty participants had a median of three assessments (IQR 2.0, 5.0) over a median of 8 months (IQR 2.3, 14.1). There were 974 data collection timepoints. Differences in respiratory measures and rates of decline between disease-onset and sex subgroups were identified. Females had lower scores in all respiratory measures and females with bulbar onset ALS had faster decline compared with other sub-groups. These differences were not detected by the ALSFRS-r respiratory subscale. Dyspnea, orthopnea, and a higher King\'s stage at baseline were associated with lower respiratory scores throughout follow-up, while having a regular productive cough at baseline was associated with lower peak cough flow scores.
UNASSIGNED: Respiratory function declines more quickly in females with ALS compared with males when measured by FVC, SVC, SNIP, or PCF, but not the ALSFRS-R respiratory sub-score. Higher baseline King\'s staging and the presence of clinical respiratory symptoms at baseline were associated with worse respiratory function. The ALSFRS-R respiratory sub-score is poorly correlated with objective respiratory measurements.
UNASSIGNED: The REVEALS study (Registry of Endpoints and Validated Experiences in ALS) was conducted between April 2018 and February 2021 in six European ALS centers. Slow and forced vital capacity (S/FVC), sniff nasal inspiratory pressure (SNIP), peak cough flow, amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R), and respiratory morbidity were collected. Data were analyzed using a Bayesian multiple outcomes random effects model.
UNASSIGNED: Two hundred and eighty participants had a median of three assessments (IQR 2.0, 5.0) over a median of 8 months (IQR 2.3, 14.1). There were 974 data collection timepoints. Differences in respiratory measures and rates of decline between disease-onset and sex subgroups were identified. Females had lower scores in all respiratory measures and females with bulbar onset ALS had faster decline compared with other sub-groups. These differences were not detected by the ALSFRS-r respiratory subscale. Dyspnea, orthopnea, and a higher King\'s stage at baseline were associated with lower respiratory scores throughout follow-up, while having a regular productive cough at baseline was associated with lower peak cough flow scores.
UNASSIGNED: Respiratory function declines more quickly in females with ALS compared with males when measured by FVC, SVC, SNIP, or PCF, but not the ALSFRS-R respiratory sub-score. Higher baseline King\'s staging and the presence of clinical respiratory symptoms at baseline were associated with worse respiratory function. The ALSFRS-R respiratory sub-score is poorly correlated with objective respiratory measurements.
摘要：
■为了系统地评估肌萎缩侧索硬化症（ALS）的呼吸指标下降，并检查性别的影响，疾病发作类型和进展的基线发病率。
REVEALS研究（ALS终点和验证经验注册）于2018年4月至2021年2月在六个欧洲ALS中心进行。缓慢和强迫肺活量（S/FVC），嗅鼻吸气压力（SNIP），咳嗽流量峰值,肌萎缩侧索硬化功能评定量表修订（ALSFRS-R），并收集呼吸道发病率。使用贝叶斯多结果随机效应模型分析数据。
■二百八十位参与者在8个月（IQR2.3，14.1）的中位数中进行了三项评估（IQR2.0，5.0）。有974个数据收集时间点。确定了疾病发作和性别亚组之间呼吸测量值和下降率的差异。与其他亚组相比，女性在所有呼吸指标中得分较低，而患有延髓性ALS的女性下降更快。ALSFRS-r呼吸亚量表未检测到这些差异。呼吸困难,端坐呼吸,基线时较高的King\'s分期与整个随访期间较低的呼吸评分相关，而在基线时有规律的生产性咳嗽与较低的咳嗽血流峰值评分相关.
■通过FVC测量，与男性相比，患有ALS的女性呼吸功能下降得更快，SVC,SNIP,或PCF，但不是ALSFRS-R呼吸评分。较高的基线King分期和基线时临床呼吸道症状的存在与较差的呼吸功能相关。ALSFRS-R呼吸子评分与客观呼吸测量结果的相关性较差。
REVEALS研究（ALS终点和验证经验注册）于2018年4月至2021年2月在六个欧洲ALS中心进行。缓慢和强迫肺活量（S/FVC），嗅鼻吸气压力（SNIP），咳嗽流量峰值,肌萎缩侧索硬化功能评定量表修订（ALSFRS-R），并收集呼吸道发病率。使用贝叶斯多结果随机效应模型分析数据。
■二百八十位参与者在8个月（IQR2.3，14.1）的中位数中进行了三项评估（IQR2.0，5.0）。有974个数据收集时间点。确定了疾病发作和性别亚组之间呼吸测量值和下降率的差异。与其他亚组相比，女性在所有呼吸指标中得分较低，而患有延髓性ALS的女性下降更快。ALSFRS-r呼吸亚量表未检测到这些差异。呼吸困难,端坐呼吸,基线时较高的King\'s分期与整个随访期间较低的呼吸评分相关，而在基线时有规律的生产性咳嗽与较低的咳嗽血流峰值评分相关.
■通过FVC测量，与男性相比，患有ALS的女性呼吸功能下降得更快，SVC,SNIP,或PCF，但不是ALSFRS-R呼吸评分。较高的基线King分期和基线时临床呼吸道症状的存在与较差的呼吸功能相关。ALSFRS-R呼吸子评分与客观呼吸测量结果的相关性较差。
