Survival of patients with amyotrophic lateral sclerosis in a population-based registry
Survival of patients with amyotrophic lateral sclerosis in a population-based registry
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DOI：
10.1159/000086353
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发表时间：
2005-01-01
影响因子：
5.7
通讯作者：
Zardi, A
中科院分区：
文献类型：
--
作者：
Millul, A;Beghi, E;Zardi, A
目的：评估意大利人群中肌萎缩侧索硬化症(ALS)患者的存活率，并评估选定的预后指标对存活率的影响。背景：据报道，ALS患者从确诊到出现症状的中位生存期为12至23个月，中位生存期为23至36个月。尽管已经确定了几个负面预后因素，但总体情况仍需澄清。方法：我们纳入了1998年意大利ALS地区登记的患者(人口4,529,003人)。这一诊断得到了一个特设委员会的确认，该委员会使用了最初的El Ecorial标准。对每个病例进行定期随访，直到死亡或2002年12月31日，以发生时间较早者为准。采用Kaplan-Meier方法在整个样本中通过诊断确定性水平和选定的预后指标(年龄、性别、延髓或脊柱发病以及病程)来评估生存率。多因素分析采用COX比例风险函数。结果：受试者79例，女性33例，男性46例，年龄28~85岁，平均64.4岁。30%的病例以延髓症状起病。确诊时平均症状持续时间为13.3个月。ALS确诊的占43%，可能的占29%，可能的占6%，可疑的占22%。截至2002年12月31日，已死亡56例(71%)。确诊后的累积存活概率在12个月时为78%，24个月时为56%，48个月时为32%。发病后中位生存期为39.2个月，确诊后中位生存期为30.6个月。多因素分析证实确诊时ALS和老年ALS是不良预后因素。结论：本样本中ALS患者的生存期比以前报道的略长。更好的姑息治疗和支持性治疗可能解释了这种差异。确诊时年龄较大和存在明确的肌萎缩侧索硬化症是预后不良的预测因素。版权所有(C)2005 S.Karger AG，巴塞尔。
Objective: To evaluate the survival of patients with a myotrophic lateral sclerosis (ALS) in an Italian population and to assess the effects of selected prognostic indicators on survival. Background: Median survival of ALS patients has been reported to range between 12 and 23 months from diagnosis and between 23 and 36 months from onset of symptoms. Although several negative prognostic factors have been identified, the overall picture still needs clarification. Methods: We included patients enrolled in an Italian ALS Regional Register (population 4,529,003) during the calendar year 1998. The diagnosis was confirmed by an ad hoc committee using the original El Escorial criteria. Each case was regularly followed up until death or December 31, 2002, whichever came first. Survival was assessed with the Kaplan-Meier method in the whole sample, by level of diagnostic certainty, and by selected prognostic indicators (age, sex, bulbar or spinal onset, and disease duration). Multivariate analysis was done with the Cox proportional hazard function. Results: The sample comprised 79 patients (33 female; 46 male) aged 28-85 years (mean age 64.4 years). Onset of symptoms was bulbar in 30% of cases. Mean symptom duration at diagnosis was 13.3 months. ALS was definite in 43%, probable in 29%, possible in 6%, and suspected in 22%. By December 31, 2002, 56 cases (71%) had died. The cumulative probability of surviving after diagnosis was 78% at 12 months, 56% at 24 months, and 32% at 48 months. Median survival from onset was 39.2 months and from diagnosis 30.6 months. Multivariate analysis confirmed definite ALS at diagnosis and older age as adverse prognostic factors. Conclusions: Survival of ALS patients in the present sample was slightly longer than previously reported. Better palliative care and supportive treatment may explain the difference. Older age and the presence of definite ALS at diagnosis are poor prognostic predictors. Copyright (C) 2005 S. Karger AG, Basel.