TY - JOUR T1 -肌萎缩性侧索硬化症患者血脂与生存的关系JF -神经学JO -神经学SP - e1062 LP - e1071 DO - 10.1212/WNL.0000000000201657首页六世- 100 - 10 AU -马克。r . Janse van Mantgem盟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 Y1 - 2023/03/07 UR - //www.ez-admanager.com/content/100/10/e1062.abstract N2 -首页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,标记的疾病严重程度,以及肌萎缩性侧索硬化症(ALS)患者的生存率。方法:我们对目前有关肌萎缩性侧索硬化症患者血脂预后价值的文献进行荟萃分析。随后，我们评估了在荷兰诊断的所有连续患者的诊断、临床疾病阶段和生存之间的血脂水平的关系。我们确定了每种脂类的总生存风险比(HR)，定义为任何原因导致的死亡。一部分患者与之前的全基因组关联研究相匹配;数据用于计算PPS的脂代谢生物标志物，并确定诊断时观察到的脂质水平与生存之间的关系。结果4项研究的meta分析显示，脂代谢生物标志物均与总生存期无统计学意义;然而，研究结果之间存在相当大的异质性。 Using individual patient data (N = 1,324), we found that increased high-density lipoprotein (HDL) cholesterol was associated with poorer survival (HR of 1.33 (95% CI 1.14–1.55, p < 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 ER -