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在肌萎缩侧索硬化症患者中,与用力肺活量(FVC)类似,上腔静脉(SVC)是呼吸功能衰退的一个指标。

SVC Is a Marker of Respiratory Decline Function, Similar to FVC, in Patients With ALS.

作者信息

Pinto Susana, de Carvalho Mamede

机构信息

Faculdade de Medicina, Instituto de Fisiologia e Instituto de Medicina Molecular, Universidade de Lisboa, Lisbon, Portugal.

Department of Community and Rehabilitation, Rehabilitation Medicine, Umeå University, Umeå, Sweden.

出版信息

Front Neurol. 2019 Feb 28;10:109. doi: 10.3389/fneur.2019.00109. eCollection 2019.

Abstract

Respiratory function is a critical predictor of survival in amyotrophic lateral sclerosis (ALS). We aimed to determine if slow vital capacity (SVC) is a predictor of functional loss in ALS as compared to forced vital capacity (FVC). Consecutive ALS patients in whom respiratory tests were performed at baseline and 6 months later were included. All patients were evaluated with revised ALS functional rating scale (ALSFRS-R) and the respiratory tests, SVC, and FVC. Significant independent variables of functional decay were assessed by univariate Kaplan-Meier log-rank test and multivariate Cox proportional hazards model. A monthly decay not exceeding 0.92 in ALSFRS was considered as the time event. We included 232 patients (134 men; mean onset-age 59.1 ± 11.23 years; mean disease duration from first symptoms to first visit: 14.5 ± 12.9 months; 166 spinal and 66 bulbar onset). All variables studied declined significantly between the two evaluations ( < 0.001). FVC and SVC were strongly correlated at study entry ( = 0.98, < 0.001) and FVC and SVC decays between first evaluation and 6 months after were the only significant prognostic variables of functional decay ( < 0.001). FVC and SVC decay are inter-changeable in predicting functional decay in ALS. Pharmacological interventions reducing the decline rate of FVC and SVC can have a positive impact on the global functional impairment, with relevant implications for clinical trials' design and interpretation.

摘要

呼吸功能是肌萎缩侧索硬化症(ALS)患者生存的关键预测指标。我们旨在确定与用力肺活量(FVC)相比,慢肺活量(SVC)是否为ALS功能丧失的预测指标。纳入在基线和6个月后进行呼吸测试的连续性ALS患者。所有患者均接受修订的ALS功能评定量表(ALSFRS-R)以及呼吸测试、SVC和FVC评估。通过单因素Kaplan-Meier对数秩检验和多因素Cox比例风险模型评估功能衰退的显著独立变量。将ALSFRS中每月衰退不超过0.92视为时间事件。我们纳入了232例患者(134例男性;平均发病年龄59.1±11.23岁;从首发症状到首次就诊的平均病程:14.5±12.9个月;166例脊髓型发病和66例延髓型发病)。两次评估之间所有研究变量均显著下降(<0.001)。研究开始时FVC和SVC高度相关(=0.98,<0.001),首次评估与6个月后FVC和SVC的衰退是功能衰退仅有的显著预后变量(<0.001)。在预测ALS功能衰退方面,FVC和SVC的衰退具有互换性。降低FVC和SVC下降速率的药物干预可对整体功能损害产生积极影响,这对临床试验的设计和解读具有重要意义。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70ce/6403463/4f72ad55ba67/fneur-10-00109-g0001.jpg

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