Jiang Qirui, Guo Yuan, Yang Tianmi, Li Shirong, Hou Yanbing, Lin Junyu, Xiao Yi, Ou Ruwei, Wei Qianqian, Shang Huifang
Department of Neurology, Laboratory of Neurodegenerative Disorders, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Outpatient Department, West China School of Nursing, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Front Neurosci. 2024 Jan 5;17:1309568. doi: 10.3389/fnins.2023.1309568. eCollection 2023.
Cystatin C (CysC) levels in amyotrophic lateral sclerosis (ALS) have been found changes, however, the associations between serum CysC levels and the progression and survival of ALS remain largely unknown.
A total of 1,086 ALS patients and 1,026 sex-age matched healthy controls (HCs) were enrolled in this study. Serum CysC, other renal function, and metabolic parameters were measured. Correlation analysis and binary logistic regression were used to explore the factors related to serum CysC. Kaplan-Meier curve and Cox regression model were used for survival analysis.
CysC levels were significantly higher in ALS patients compared to HCs (0.94 vs. 0.85 mg/L, < 0.001). Compared with ALS patients with lower CysC levels, those with higher CysC levels had an older age of onset, significantly lower ALSFRS-R scores (40.1 vs. 41.3, < 0.001), a faster disease progression rate (0.75 vs. 0.67, = 0.011), and lower frontal lobe function scores (15.8 vs. 16.1, = 0.020). In the correlation analysis, CysC levels were significantly negatively correlated with ALSFRS-R scores ( = -0.16, < 0.001). Additionally, ALS patients with higher CysC levels had significantly shorter survival time (40.0 vs. 51.8, < 0.001) compared to patients with lower CysC levels. Higher CysC levels were associated with a higher risk of death in Cox analysis (HR: 1.204, 95% CI: 1.012-1.433). However, when treatment was included in the model, the result was no longer significant.
CysC levels in ALS patients were higher compared to HCs. Higher CysC levels were associated with greater disease severity, faster progression rate and shorter survival, needing early intervention.
已发现肌萎缩侧索硬化症（ALS）患者的胱抑素C（CysC）水平发生变化，然而，血清CysC水平与ALS进展及生存之间的关联仍大多未知。
本研究共纳入1086例ALS患者和1026例年龄和性别匹配的健康对照（HCs）。检测血清CysC、其他肾功能及代谢参数。采用相关性分析和二元逻辑回归探索与血清CysC相关的因素。采用Kaplan-Meier曲线和Cox回归模型进行生存分析。
与HCs相比，ALS患者的CysC水平显著更高（0.94 vs. 0.85mg/L，<0.001）。与CysC水平较低的ALS患者相比，CysC水平较高的患者发病年龄更大，ALSFRS-R评分显著更低（40.1 vs. 41.3，<0.001），疾病进展速度更快（0.75 vs. 0.67，=0.011），额叶功能评分更低（15.8 vs. 16.1，=0.020）。在相关性分析中，CysC水平与ALSFRS-R评分显著负相关（=-0.16，<0.001）。此外，与CysC水平较低的患者相比，CysC水平较高的ALS患者生存时间显著更短（40.0 vs. 51.8，<0.001）。在Cox分析中，较高的CysC水平与更高的死亡风险相关（HR：1.204，95%CI：1.012-1.433）。然而，当模型中纳入治疗因素时，结果不再显著。
与HCs相比，ALS患者的CysC水平更高。较高的CysC水平与更严重的疾病、更快的进展速度和更短生存相关，需要早期干预。
