Yu Jixu, Wang Nian, Qi Faying, Wang Xianjun, Zhu Qiyi, Lu Yucheng, Zhang Huiling, Che Fengyuan, Li Wei
Department of Neurology, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.
Central Laboratory, Linyi People's Hospital, Linyi, Shandong 276003, P.R. China.
Biomed Rep. 2018 Oct;9(4):333-338. doi: 10.3892/br.2018.1138. Epub 2018 Aug 2.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. The mechanism that defines the loss of neurons in ALS is still not clearly understood, and there is no effective therapy to block its progression. Previous studies indicate that a disorder of iron homeostasis exists in ALS and based on this, the change of serum iron and ferritin and the association between iron metabolism and clinical features in Chinese Han patients with ALS was further investigated in the present study, in order to define its pathogenesis. Two cohorts were established: An ALS group consisting of 24 patients and a control group consisting of 38 healthy volunteers. Venous blood samples were collected for serum iron and ferritin analysis. The results indicated that the levels of serum iron were significantly decreased in patients with ALS (P<0.05), while there was no significant difference in serum ferritin between the ALS and control groups. However, the levels of serum ferritin were increased significantly in ALS patients with bulbar-onset (vs. limb-onset in females), dysphagia (vs. without dysphagia), longer disease duration (>12 months vs. ≤12 months in males) and lower ALS Functional Rating Scale-Revised score (<33 vs. ≥33; P<0.05). These results suggested that there was dysregulation of iron metabolism in Chinese Han patients with ALS and that serum ferritin may be a candidate biomarker of aggravation in these patients.
肌萎缩侧索硬化症(ALS)是一种神经退行性疾病。目前仍不清楚导致ALS患者神经元丧失的机制,并且尚无有效的治疗方法来阻止其进展。先前的研究表明,ALS患者存在铁稳态失调,基于此,本研究进一步调查了中国汉族ALS患者的血清铁和铁蛋白变化以及铁代谢与临床特征之间的关联,以明确其发病机制。建立了两个队列:一个由24例患者组成的ALS组和一个由38名健康志愿者组成的对照组。采集静脉血样本进行血清铁和铁蛋白分析。结果表明,ALS患者的血清铁水平显著降低(P<0.05),而ALS组和对照组之间的血清铁蛋白水平无显著差异。然而,延髓起病的ALS患者(女性中与肢体起病相比)、吞咽困难的患者(与无吞咽困难相比)、病程较长的患者(男性中>12个月与≤12个月相比)以及ALS功能评定量表修订版评分较低的患者(<33与≥33相比;P<0.05),其血清铁蛋白水平显著升高。这些结果表明,中国汉族ALS患者存在铁代谢失调,血清铁蛋白可能是这些患者病情加重的一个候选生物标志物。