IR@PKUHSC  > 北京大学第三临床医学院  > 神经内科
学科主题临床医学
Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China
Chen, Lu1; Zhang, Bin1; Chen, Ru2; Tang, Lu1; Liu, Rong1; Yang, Yan1; Yang, Yi1; Liu, Xiaolu1; Ye, Shan1; Zhan, Siyan2; Fan, Dongsheng1
刊名JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
2015-10-01
DOI10.1136/jnnp-2015-310471
86期:10页:1075-1081
收录类别SCI
文章类型Review
WOS标题词Science & Technology
类目[WOS]Clinical Neurology ; Psychiatry ; Surgery
资助者National Natural Science Foundation of China ; National Natural Science Foundation of China
研究领域[WOS]Neurosciences & Neurology ; Psychiatry ; Surgery
关键词[WOS]PROGNOSTIC-FACTORS ; PESTICIDE EXPOSURE ; RISK-FACTOR ; FLAIL ARM ; ALS ; SURVIVAL ; SMOKING ; EPIDEMIOLOGY ; VENTILATION ; ONSET
英文摘要

Objectives To describe the natural history and clinical features of sporadic amyotrophic lateral sclerosis (ALS) in Chinese patients, and to report data on the prognostic factors for survival.

Methods All patients referred to our ALS centre between 2003 and 2012 were followed up every 3 months. Survival and tracheotomy were predefined as primary outcome measures. Group differences were analysed using parametric and non-parametric tests as appropriate. Survival was analysed using the Kaplan-Meier method and Cox regression analysis.

Results Of the 1624 patients with ALS, 75.1% had limb-onset, 14.0% had bulbar-onset, 7.8% had flail-arm syndrome (FAS), 2.6% had progressive muscular atrophy and 0.5% had primary lateral sclerosis. The male: female ratio was 1.7: 1, and the mean age at onset was 49.8 years. The median diagnostic delay was 14 months, and the median survival time after symptom onset was 71 months. Male gender, older age at symptom onset, lower body mass index, shorter diagnostic delay, bulbaronset ALS phenotype, higher Airlie House category at presentation, rural place of residence, use of traditional Chinese medicine and a history of contact with pesticides were associated with poorer survival, whereas female gender or an FAS phenotype may have a better prognosis.

Conclusions The clinical characteristics and outcomes of Chinese patients with sporadic ALS were different compared with patients from other countries. Compared with other studies, the age at onset of Chinese patients was earlier, the percentage of bulbar-onset ALS was lower and the prognosis was better. This study substantially advances the understanding of the clinical features and epidemiology of this rare disease.

语种英语
所属项目编号81030019
资助者National Natural Science Foundation of China ; National Natural Science Foundation of China
WOS记录号WOS:000361154800006
引用统计
被引频次:10[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/64692
专题北京大学第三临床医学院_神经内科
作者单位1.Peking Univ, Hosp 3, Dept Neurol, Beijing 100191, Peoples R China
2.Peking Univ, Sch Publ Hlth, Dept Epidemiol & Biostat, Hlth Sci Ctr, Beijing 100191, Peoples R China
推荐引用方式
GB/T 7714
Chen, Lu,Zhang, Bin,Chen, Ru,et al. Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China[J]. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY,2015,86(10):1075-1081.
APA Chen, Lu.,Zhang, Bin.,Chen, Ru.,Tang, Lu.,Liu, Rong.,...&Fan, Dongsheng.(2015).Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China.JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY,86(10),1075-1081.
MLA Chen, Lu,et al."Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China".JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY 86.10(2015):1075-1081.
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