%0杂志文章%A Mark R. Janse van Mantgem %A Wouter van Rheenen %A Anemone V. Hackeng %A Michael A. van Es %A Jan H. Veldink %A Leonard H. van den Berg %A Ruben P.A. van Eijk %T肌萎缩性侧索硬化症患者血脂与生存的关系%B A荟萃分析和基于人群的研究%D 2023 %R 10.1212/WNL。背景与目的探讨肌萎缩性侧索硬化症(ALS首页)患者脂质、脂代谢生物标志物多基因谱评分(PPS)、疾病严重程度标志物与生存之间的关系。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。使用个体患者数据(N = 1324)，我们发现高密度脂蛋白(HDL)胆固醇升高与较差的生存率相关(HR为1.33 (95% CI 1.14-1.55, p < 0.001))。BMI与HDL胆固醇(Pearson r−0.26,95% CI−0.32至−0.20)之间呈负相关，BMI与甘油三酯(TG)之间呈正相关(Pearson r 0.18, 95% CI 0.12-0.24)。在较晚期的临床阶段，总胆固醇和低密度脂蛋白胆固醇的血清浓度较低(均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 %U //www.ez-admanager.com/content/neurology/100/10/e1062.full.pdf