Lo Coco Daniele, Marchese Santino, La Bella Vincenzo, Piccoli Tommaso, Lo Coco Albino
ALS Research Center, Dipartimento Universitario di Neuroscienze Cliniche, Università di Palermo, Via G La Loggia 1, 90129 Palermo, Italy.
Chest. 2007 Jul;132(1):64-9. doi: 10.1378/chest.06-2712. Epub 2007 May 2.
To determine whether the amyotrophic lateral sclerosis functional rating scale (ALSFRS), which is a validated instrument that assesses the functional status and the disease progression in patients with amyotrophic lateral sclerosis (ALS), predicts hospital length of stay and survival time in ALS patients treated with tracheostomy-intermittent positive-pressure ventilation (TIPPV).
Thirty-three consecutive ALS patients with acute respiratory failure who received therapy with TIPPV were prospectively followed up from their admission to the hospital until death. The association of ALSFRS score at hospital admission with length of hospital stay and survival after TIPPV were examined using Cox proportional hazard models, adjusting for age at baseline, sex, and symptom duration.
The median ALSFRS score of the ALS patients at hospital admission was 11 (range, 4 to 22). The median length of hospital stay was 55 days (range, 7 to 124 days), with a hospital mortality rate of 9%. For the 30 patients (91%) discharged from the hospital, the median survival time was 37 months (range, 2 to 64 months). The total ALSFRS score (above or below the median score) was a significant predictor of length of hospital stay (hazard ratio [HR], 2.86; 95% confidence interval [CI], 1.2 to 6.5; p = 0.003) and survival after TIPPV (HR, 3.76; 95% CI, 1.4 to 9.7; p = 0.002). The total ALSFRS score at hospital admission was also associated with length of hospital stay (HR, 2.1; 95% CI, 1.1 to 5.1; p = 0.005) and survival (HR, 0.52; 95% CI, 0.1 to 0.8; p = 0.002) when included in a Cox multivariable model together with the other demographic and clinical variables.
In ALS patients with acute respiratory failure who have been treated with TIPPV, the total ALSFRS score may predict length of hospital stay and long-term survival after invasive mechanical ventilation.
肌萎缩侧索硬化功能评定量表（ALSFRS）是一种用于评估肌萎缩侧索硬化（ALS）患者功能状态和疾病进展的有效工具，本研究旨在确定该量表能否预测接受气管切开-间歇性正压通气（TIPPV）治疗的ALS患者的住院时间和生存时间。
对33例接受TIPPV治疗的急性呼吸衰竭ALS患者进行前瞻性随访，从入院直至死亡。采用Cox比例风险模型，对基线年龄、性别和症状持续时间进行校正，研究入院时ALSFRS评分与TIPPV治疗后的住院时间和生存情况之间的关联。
ALS患者入院时ALSFRS评分中位数为11分（范围4至22分）。住院时间中位数为55天（范围7至124天），住院死亡率为9%。30例（91%）出院患者的生存时间中位数为37个月（范围2至64个月）。ALSFRS总分（高于或低于中位数）是住院时间（风险比[HR]，2.86；95%置信区间[CI]，1.2至6.5；p = 0.003）和TIPPV治疗后生存情况（HR，3.76；95%CI，1.4至9.7；p = 0.002）的显著预测因素。当与其他人口统计学和临床变量一起纳入Cox多变量模型时，入院时ALSFRS总分也与住院时间（HR，2.1；95%CI，1.1至5.1；p = 0.005）和生存情况（HR，0.52；95%CI，0.1至0.8；p = 0.002）相关。
在接受TIPPV治疗的急性呼吸衰竭ALS患者中，ALSFRS总分可能预测侵入性机械通气后的住院时间和长期生存情况。
