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肌萎缩侧索硬化症的预后因素:中德之间的比较。

Prognostic factors in ALS: a comparison between Germany and China.

机构信息

Department of Neurology, Universitätsklinik Ulm, RKU, Oberer Eselsberg 45, 89081, Ulm, Germany.

Department of Neurology, Peking University Third Hospital, Beijing, 100191, China.

出版信息

J Neurol. 2019 Jun;266(6):1516-1525. doi: 10.1007/s00415-019-09290-4. Epub 2019 Mar 28.

Abstract

OBJECTIVE

Several independent prognostic factors, such as age of onset, type of onset, body mass index (BMI), and progression rate have been identified for amyotrophic lateral sclerosis (ALS) in Caucasians. The aim of this study was to identify such factors in Chinese patients and to compare their impact with German patients.

METHODS

Comparison of prognostic factors was based on two hospital-based registries. The registry of the German Network for Motor Neuron Diseases contains 3100 patients with ALS. The Chinese registry comprises 2101 patients who were collected between 2003 and 2015 in the metropolitan area of Beijing.

RESULTS

Disease progression was slower in China [median loss of 0.50 points (IQR 0.26-0.87 points) versus 0.55 points (IQR 0.28-1.00 points) of ALS functional rating scale revised (ALS-FRS-R) score per month; p < 0.0001]. Survival of patients with ALS was similar in Germany and China (p > 0.05). We found that younger age of onset (p < 0.0001), spinal onset (p < 0.0001), high BMI (p < 0.0001) and low progression rate (p < 0.0001) were positive prognostic factors in China as well as in Germany.

INTERPRETATION

Prognostic factors, which are known to modify the course of disease in Caucasians, apply to Chinese patients as well. The results indicate that despite the apparent differences regarding genotype and clinical phenotype, findings from interventional studies in Caucasians aiming at disease-modifying prognostic factors (such as body weight) may be transferred to Chinese patients.

摘要

目的

几项独立的预后因素,如发病年龄、发病类型、体重指数(BMI)和进展速度,已被确定为白种人肌萎缩侧索硬化症(ALS)的预后因素。本研究的目的是确定中国患者的这些因素,并与德国患者进行比较。

方法

基于两个基于医院的登记处比较预后因素。德国运动神经元疾病网络的登记处包含 3100 名 ALS 患者。中国的登记处包括 2101 名患者,这些患者是在 2003 年至 2015 年间在北京大都市区收集的。

结果

中国的疾病进展速度较慢[中位数丢失 0.50 分(IQR 0.26-0.87 分)与 ALS 功能评定量表修订版(ALS-FRS-R)评分每月 0.55 分(IQR 0.28-1.00 分);p<0.0001]。德国和中国的 ALS 患者的生存率相似(p>0.05)。我们发现,中国和德国的发病年龄较小(p<0.0001)、脊髓发病(p<0.0001)、BMI 较高(p<0.0001)和进展速度较慢(p<0.0001)是阳性预后因素。

结论

已知会改变白种人疾病进程的预后因素也适用于中国患者。这些结果表明,尽管在基因型和临床表型方面存在明显差异,但针对疾病修饰预后因素(如体重)的干预性研究在白种人中的发现可能会被转移到中国患者身上。

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