PT -期刊文章盟马克。r . Janse van Mantgem AU - Wouter van Rheenen盟海葵诉Hackeng AU -迈克尔·a·范Es盟Jan h . Veldink AU -伦纳德·h . van den Berg盟鲁本P.A. van Eijk TI -协会之间的肌萎缩性脊髓侧索硬化症患者血清脂质和生存援助- 10.1212 / WNL。0000000000201657 DP - 2023年3月7日TA -神经病学首页PG e1062——//www.ez-admanager.com/content/100/10/e1062.short e1071 VI - 100 IP - 10 4099 - 4100——//www.ez-admanager.com/content/100/10/e1062.full——Neurology2023 3月7日;100 AB -背景和目的探讨脂质之间的关系,多基因档案分数(PPS)对脂质代谢的生物标记,标记疾病的严重性和肌萎缩性脊髓侧索硬化(ALS)患者的生存期。方法我们meta-analyzed当前文献对脂质ALS患者的预后价值。随后,我们在诊断评价血脂水平的关系,临床疾病阶段,连续和生存在所有患者诊断在荷兰。我们确定的风险比(人力资源)脂质整体生存,定义为死于任何原因。病人的一个子集匹配之前的全基因组关联研究;数据被用来计算PPS对脂质代谢的生物标志物和确定观察脂质水平诊断和生存之间的联系。4研究的荟萃分析结果表明,脂类代谢的生物标志物在统计学上都与总体存活率显著相关;然而,有相当大的研究结果之间的异质性。使用个别病人数据(N = 1324),我们发现,增加高密度脂蛋白(HDL)胆固醇与贫穷有关生存(HR 1.33 (95% CI 1.14 - -1.55, p & lt;0.001))。 The correlation between BMI and HDL cholesterol (Pearson r −0.26, 95% CI −0.32 to −0.20) was negative and between BMI and triglycerides (TG) positive (Pearson r 0.18, 95% CI 0.12–0.24). Serum concentrations of total cholesterol and LDL cholesterol were lower in more advanced clinical stages (both p < 0.001). PPS for biomarkers of lipid metabolism explained 1.2%–13.1% of their variance at diagnosis. None of the PPS was significantly associated with survival (all p > 0.50).Discussion Lipids may contain valuable information about disease severity and prognosis, but their main value may be driven as a consequence of disease progression. Our results underscore that gaining further insight into lipid metabolism and longitudinal data on serum concentrations of the lipid profile could improve the monitoring of patients and potentially further disentangle ALS pathogenesis.ALS=amyotrophic lateral sclerosis; ALSFRS=ALS functional rating scale; BMI=body mass index; EEC=El Escorial criteria; ENCALS=European Network to Cure ALS; FTD=frontotemporal dementia; HDL=high-density lipoprotein; HDL-C=HDL cholesterol; HR=hazard ratio; LDL-C=low-density lipoprotein cholesterol; PPS=polygenic profile score; TC=total cholesterol; TG=triglycerides

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