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法国肌萎缩侧索硬化症患者队列中的铁代谢紊乱

Iron metabolism disturbance in a French cohort of ALS patients.

作者信息

Veyrat-Durebex Charlotte, Corcia Philippe, Mucha Aleksandra, Benzimra Simon, Mallet Cindy, Gendrot Chantal, Moreau Caroline, Devos David, Piver Eric, Pagès Jean-Christophe, Maillot François, Andres Christian R, Vourc'h Patrick, Blasco Hélène

机构信息

Unité Mixte de Recherche U930, Institut National de la Santé et de la Recherche Médicale, Université François-Rabelais, Equipe "Neurogénétique et Neurométabolomique", 10 boulevard Tonnellé, 37032 Tours, France ; Laboratoire de Biochimie et de Biologie Moléculaire, Hôpital Bretonneau, Centre Hospitalier Régional Universitaire de Tours, 37044 Tours, France.

Unité Mixte de Recherche U930, Institut National de la Santé et de la Recherche Médicale, Université François-Rabelais, Equipe "Neurogénétique et Neurométabolomique", 10 boulevard Tonnellé, 37032 Tours, France ; Centre SLA, Service de Neurologie, Centre Hospitalier Régional Universitaire de Tours, 37044 Tours, France.

出版信息

Biomed Res Int. 2014;2014:485723. doi: 10.1155/2014/485723. Epub 2014 Jul 2.

DOI:10.1155/2014/485723
PMID:25101285
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4101961/
Abstract

OBJECTIVE

The aim of this study was to assess iron status in a cohort of amyotrophic lateral sclerosis (ALS) patients compared to controls in order to evaluate these parameters as a risk factor or a modifying factor of ALS.

METHODS

We collected serum iron, ferritin, transferrin, total iron-binding capacity, and transferrin saturation coefficient (TSC) from 104 ALS patients at the time of diagnosis and from 145 controls. We reported phenotypic characteristics and evolution parameters such as ALSFRS-R and forced vital capacity at diagnosis and after one year of follow-up. In a first step we compared iron status between ALS patients and controls, and then we evaluated the relation between iron status and disease evolution of ALS patients using univariate and multivariate analysis.

RESULTS

We observed increased concentrations of serum iron (P = 0.002) and ferritin (P < 0.0001) and increased TSC (P = 0.017) in ALS patients. We also showed an association between markers of iron status and high body weight loss in ALS patients. The multivariate analysis of survival highlighted a significant relation between ferritin level and disease duration (P = 0.038).

CONCLUSION

This is the first study showing a higher concentration of serum iron in ALS patients, strengthening the involvement of a deregulation of iron metabolism in ALS.

摘要

目的

本研究旨在评估一组肌萎缩侧索硬化症(ALS)患者相较于对照组的铁状态,以评估这些参数作为ALS的危险因素或调节因素。

方法

我们在诊断时收集了104例ALS患者以及145例对照者的血清铁、铁蛋白、转铁蛋白、总铁结合力和转铁蛋白饱和度系数(TSC)。我们报告了表型特征以及诸如诊断时和随访一年后的ALSFRS-R和用力肺活量等病情进展参数。第一步,我们比较了ALS患者和对照者之间的铁状态,然后我们使用单变量和多变量分析评估了铁状态与ALS患者疾病进展之间的关系。

结果

我们观察到ALS患者的血清铁浓度升高(P = 0.002)、铁蛋白浓度升高(P < 0.0001)以及TSC升高(P = 0.017)。我们还表明,ALS患者的铁状态标志物与高体重减轻之间存在关联。生存的多变量分析突出显示铁蛋白水平与疾病持续时间之间存在显著关系(P = 0.038)。

结论

这是第一项显示ALS患者血清铁浓度较高的研究,强化了铁代谢失调与ALS有关的观点。

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PLoS One. 2012;7(9):e45034. doi: 10.1371/journal.pone.0045034. Epub 2012 Sep 14.
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