Ljubljana ALS Centre, Institute of Clinical Neurophysiology, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Department of Neurology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Amyotroph Lateral Scler Frontotemporal Degener. 2020 May;21(3-4):203-208. doi: 10.1080/21678421.2020.1746809. Epub 2020 Apr 6.
The Ljubljana ALS Centre, established in 2002, is the only tertiary center for amyotrophic lateral sclerosis (ALS) in Slovenia. The aim of our study was to evaluate the impact of therapeutic interventions and improvements in the multidisciplinary care on the survival of our patients. All patients diagnosed with ALS at our center during years 2003-2005 and 2011-2012 were included in this retrospective cohort study ( = 124). Kaplan-Meier survival analysis and multiple regression analysis with Cox proportional hazards model were performed to compare survival and to evaluate the differences between the two cohorts. Median survival from the time of diagnosis was 13.0 (95% CI 10.2-15.8) months in the early group and 21.8 (95% CI 17.2-26.4) months in the late group ( = 0.005). In the Cox proportional hazards analysis, the late group of patients was associated with better survival independently of all other prognostic factors (hazard ratio (HR)=0.51, 95% CI = 0.32-0.81, = 0.004). Survival was also associated with patients' age, use of noninvasive ventilation (NIV) and gastrostomy. The model fit significantly improved when the interaction between the NIV use and the observed time period was added to the model (HR = 0.34, 95% CI = 0.12-0.96, = 0.041). Our findings suggest that improvements in the multidisciplinary care were beneficial for survival of our patients with ALS. The survival benefit in the late group of our patients could be partially explained by the improvements in the NIV use at our center.
卢布尔雅那肌萎缩侧索硬化症中心成立于 2002 年，是斯洛文尼亚唯一的肌萎缩侧索硬化症（ALS）三级中心。我们的研究目的是评估治疗干预措施和多学科护理的改进对我们患者生存的影响。本回顾性队列研究纳入了 2003-2005 年和 2011-2012 年期间在我们中心诊断为 ALS 的所有患者（ = 124）。使用 Kaplan-Meier 生存分析和 Cox 比例风险模型进行多变量回归分析，以比较两组患者的生存率并评估两组之间的差异。早期组诊断后中位生存期为 13.0 个月（95%CI 10.2-15.8），晚期组为 21.8 个月（95%CI 17.2-26.4）（ = 0.005）。在 Cox 比例风险分析中，晚期组患者的生存时间优于其他所有预后因素（风险比（HR）=0.51，95%CI = 0.32-0.81， = 0.004）。生存时间还与患者年龄、使用无创通气（NIV）和胃造口术有关。当将 NIV 使用和观察时间的交互作用添加到模型中时，模型拟合显著改善（HR = 0.34，95%CI = 0.12-0.96， = 0.041）。我们的研究结果表明，多学科护理的改进有利于 ALS 患者的生存。我们患者晚期组的生存获益部分可以通过我们中心 NIV 使用的改善来解释。
