Creatine kinase and prognosis in amyotrophic lateral sclerosis: a literature review and multi-centre cohort analysis.

Creatine kinase and prognosis in amyotrophic lateral sclerosis: a literature review and multi-centre cohort analysis.
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DOI:
10.1007/s00415-022-11195-8
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发表时间:
2022-10
影响因子:
6
通讯作者:
Thompson, Alexander G.
Thompson, Alexander G.
中科院分区:
医学2区
文献类型:
--
作者:
Gao, Jiali;Dharmadasa, Thanuja;Malaspina, Andrea;Shaw, Pamela J.;Talbot, Kevin;Turner, Martin R.;Thompson, Alexander G.

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肌萎缩性侧索硬化症(ALS)是一种预后异质性的神经退行性疾病。血肌酸激酶(CK)水平作为预后生物标志物的报道不一致,一些ALS患者的水平升高被认为反映了肌肉萎缩,这也是可变的。MEDLINE系统检索了CK在ALS中的相关论文,并对相关研究进行了综述。使用222名ALS患者的多中心、前瞻性、纵向队列数据,使用Kaplan-Meier和Cox比例风险模型进行与CK和其他预后因素相关的生存分析。有25项研究调查了CK在ALS中的作用,其中10项专门研究了CK与生存之间的关系。五项研究没有发现任何关联,四项研究发现较高的CK水平与较长的生存有关,一项研究则相反。在我们的队列中(n = 222), 39%的患者CK水平高于实验室参考范围。男性的水平高于女性(p < 0.001),肢体症状发作的患者高于球部症状发作的患者(p < 0.001),以及运动神经元负荷较高的患者(p < 0.001)。纵向CK值变化趋势不显著。虽然在单因素分析中,首次就诊时较高的标准化对数(CK)与较长的生存期相关(风险比0.75,p = 0.003),但在调整其他预后协变量后,没有显著相关性。虽然在ALS中升高的CK水平在很大程度上确实反映了运动神经元去神经支配的降低,但当在疾病的症状期测量时,它们与生存并没有独立的关联。在线版本包含补充材料,可在10.1007/s00415-022-11195-8获得。
Amyotrophic lateral sclerosis (ALS) is a prognostically heterogeneous neurodegenerative disease. Blood creatine kinase (CK) level has been inconsistently reported as a prognostic biomarker and raised levels in some ALS patients have been presumed to reflect muscle wasting, which is also variable. MEDLINE was systematically searched for papers related to CK in ALS and the relevant studies were reviewed. Using data from 222 ALS patients in a multi-centre, prospective, longitudinal cohort, survival analyses using Kaplan–Meier and Cox proportional hazards models were undertaken in relation to CK and other prognostic factors. Twenty-five studies investigating CK in ALS were identified, of which 10 specifically studied the link between CK and survival. Five studies observed no association, four found that higher CK levels were associated with longer survival and one, the opposite. In our cohort (n = 222), 39% of patients had a CK level above the laboratory reference range. Levels were higher in males compared to females (p < 0.001), in patients with limb versus bulbar onset of symptoms (p < 0.001) and in patients with higher lower motor neuron burden (p < 0.001). There was no significant trend in longitudinal CK values. Although a higher standardised log (CK) at first visit was associated with longer survival in univariate analysis (hazard ratio 0.75, p = 0.003), there was no significant association after adjusting for other prognostic covariates. While raised CK levels in ALS do reflect lower motor neuron denervation to a large extent, they are not independently associated with survival when measured in the symptomatic phase of the disease. The online version contains supplementary material available at 10.1007/s00415-022-11195-8.
多中心对肌萎缩性侧索硬化生物流体生物标志物的评估显示出血液神经丝轻链的至高无上。
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期刊: Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
影响因子: --
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