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评估伊拉克依赖输血的β-地中海贫血重型患者的红细胞生成素、铁调素和铁过载状态。

Evaluation of Erythroferrone, Hepcidin, and Iron Overload Status in Iraqi Transfusion-Dependent β-Thalassemia Major Patients.

机构信息

Department of Chemistry, College of Science, University of Kufa, Kufa, Iraq.

Protein Research Center, Shahid Beheshti University, G.C., Tehran, Iran.

出版信息

Hemoglobin. 2020 Jul;44(4):272-277. doi: 10.1080/03630269.2020.1794888. Epub 2020 Jul 27.

Abstract

Patients with β-thalassemia major (β-TM) show ineffective erythropoiesis and iron overload, which is the leading cause of mortality and organ injury. The present study aimed to investigate the relationships between two iron regulatory hormones, hepcidin and erythroferrone (ERFE) levels, and iron status parameters in Iraqi patients with β-TM. Iron status parameters and hormones were measured in 60 patients and compared with 30 healthy controls. The results indicated significant changes in different iron status parameters, while ferritin with the ∼11-fold increase showed the most change. Significant reduction in hepcidin and an increase in ERFE levels were detected in patients when compared to the control group, while no direct correlation was identified with the other measured iron status parameters. The receiver operating characteristic (ROC) analysis showed that the z-score of the composite of ERFE + ferritin has a full diagnostic ability for β-TM. In conclusion, our findings indicated the correlation between different iron status parameters and ferritin as the leading predictor of iron overload and two main iron regulatory hormones.

摘要

重型β地中海贫血(β-TM)患者表现出无效造血和铁过载,这是导致死亡和器官损伤的主要原因。本研究旨在探讨两种铁调节激素铁调素和红细胞生成素(ERFE)水平与伊拉克β-TM 患者铁状态参数之间的关系。在 60 名患者和 30 名健康对照者中测量了铁状态参数和激素。结果表明,不同的铁状态参数发生了显著变化,而铁蛋白增加了约 11 倍,变化最为显著。与对照组相比,患者的铁调素显著降低,ERFE 水平升高,但与其他测量的铁状态参数之间没有直接相关性。受试者工作特征(ROC)分析表明,ERFE+铁蛋白的 z 评分对β-TM 具有完全的诊断能力。总之,我们的研究结果表明,不同的铁状态参数与铁蛋白之间存在相关性,铁蛋白是铁过载的主要预测因子,而两种主要的铁调节激素也是如此。

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