Amyotrophic lateral sclerosis: early predictors of prolonged survival
Amyotrophic lateral sclerosis: early predictors of prolonged survival
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DOI：
10.1007/s00415-006-0226-8
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发表时间：
2006-11-01
影响因子：
6
通讯作者：
Appel, Stanley H.
中科院分区：
文献类型：
--
作者：
Czaplinski, Adam;Yen, Albert A.;Appel, Stanley H.
目的为了确定首次检查时可获得的延长生存期的预测因素，我们对过去20年来在我们的ALS诊所转诊的肌萎缩性硬化症（ALS）患者进行了一项历史队列研究。方法采用Kaplan-Meier生命表法对1034例确诊或可能确诊的ALS患者的无气管切开生存率进行分析。采用单变量和多变量考克斯比例风险分析估计每个因素的预后价值。结果中位生存期为3.45年（95%CI 3.27-3.74）。单变量和多变量考克斯模型均表明，年龄较小、肢体发病部位、诊断延迟时间较长、首次检查时阿佩尔ALS评分（AALSS）较低、首次症状与首次检查之间AALSS变化率（斜坡前）较低、基线用力肺活量（FVC）较高与生存期较长相关。此外，四个因素：年龄、诊断延迟、基线FVC和AALSS前斜率已被确定为患者人群生存的独立预测因素。结论：年龄小、肢体发病部位和诊断延迟时间长是ALS临床人群生存期延长的预测因素，这支持了几项早期研究的结果，这些研究是基于较小的患者群体。更重要的是，在首次检查时评估的几个其他变量预测了更长的生存期：较低的基线AALSS、较低的AALSS-前斜率和较高的基线FVC。所有这些参数都在患者管理和临床试验开发中具有价值。
Objective In order to define the predictors of prolonged survival available at the time of first examination we performed a historical cohort study of amyotrophis sclerosis (ALS) patients referred to our ALS Clinic over the last 20 years. Methods In a group of 1034 patients with the diagnosis of definite or probable ALS the effects of individual prognostic factors on tracheostomy-free survival were assessed with the Kaplan-Meier life-table method. The prognostic value of each factor was estimated using univariate and multivariate Cox proportional hazard analyses. Results The median survival time was 3.45 years, (95%CI 3.27-3.74). Both the univariate and multivariate Cox models indicated that younger age, limb site of onset, longer diagnostic delay, lower Appel ALS score (AALSS) at first examination, lower AALSS-rate of change between first symptom and first exam (preslope), and higher baseline forced vital capacity (FVC) were associated with longer survival. In addition, four factors: age, diagnostic delay, baseline FVC and AALSS preslope have been identified as independent predictors of survival in our patient population. Conclusions The identification of younger age, limb site of onset and longer diagnostic delay as predictors of prolonged survival in ALS clinic population supports the findings of several, earlier studies that were based on smaller groups of patients. More significantly, several additional variables assessed at the first examination predict longer survival: lower baseline AALSS, lower AALSS- preslope and higher baseline FVC. All of these parameters are of value in patient management and in clinical trial development.