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亚铁调素/铁蛋白比值作为β-地中海贫血患者铁过载的早期诊断指标。

Hepcidin-to-Ferritin Ratio as an Early Diagnostic Index of Iron Overload in β-Thalassemia Major Patients.

机构信息

Department of Basic Sciences, College of Pharmacy, University of Duhok, Duhok City, Iraq.

Department of Physiology and Pharmacology, College of Medicine, University of Duhok, Duhok City, Iraq.

出版信息

Hemoglobin. 2022 Mar;46(2):106-113. doi: 10.1080/03630269.2022.2083969. Epub 2022 Aug 5.

Abstract

Hepcidin (HEPC) hormone production is expected to be elevated in cases accompanying iron overload, but the opposite impact of ineffective erythropoiesis in β-thalassemia major (β-TM) patients overrides this effect. The role of the HEPC-to-ferritin (FER) ratio and its components in iron metabolism along with their diagnostic cutoff values, sensitivity, specificity, and accuracy in β-TM patients with iron overload, were examined in this study. This was a 1:1 case-control study with 120 participants, ages ranging from 2 to 30 years of both sexes, who were assigned into two groups: 60 β-TM patients with iron overload, and a control group, comprising 60 healthy individuals matched by gender and age. In the present study, we found slightly elevated serum HEPC concentration (21.9 ng/mL) compared to the controls (9.9 ng/mL), which was not statistically significant ( =0.1), and the median HEPC-to-FER ratio of the cases was significantly lower than the controls, with the median case-control difference of (-0.366;  < 0.001). Our results revealed a statistically significant impact ( < 0.001) of mean age on the serum HEPC level with the inverse linear correlation of (-0.487,  < 0.001). The area under the curve of the HEPC-to-FER ratio was 0.999 and the optimum cutoff value was 0.046 ng/mL ( < 0.001) with 100.0% sensitivity and 98.3% specificity. In conclusion, we found that serum HEPC-to-FER ratio, with an accuracy of 99.2%, may serve as an excellent index for the diagnosis of iron overload in β-TM patients differentiating them from nonthalassemic controls.

摘要

预计铁过载时会升高铁调素 (HEPC) 激素的产生,但β-地中海贫血 (β-TM) 患者无效红细胞生成的反作用会抵消这种作用。本研究检查了 HEPC-铁蛋白 (FER) 比值及其成分在铁代谢中的作用及其在铁过载的β-TM 患者中的诊断截断值、敏感性、特异性和准确性。这是一项 1:1 的病例对照研究,共有 120 名年龄在 2 至 30 岁之间的男女参与者,他们被分为两组:60 名铁过载的β-TM 患者和对照组,由 60 名性别和年龄匹配的健康个体组成。在本研究中,我们发现与对照组(9.9ng/mL)相比,血清 HEPC 浓度(21.9ng/mL)略有升高,但无统计学意义(=0.1),且病例的 HEPC-FER 比值中位数明显低于对照组,病例对照差异中位数为 (-0.366;  < 0.001)。我们的结果表明,平均年龄对血清 HEPC 水平有统计学显著影响( < 0.001),呈负线性相关(-0.487, < 0.001)。HEPC-FER 比值的曲线下面积为 0.999,最佳截断值为 0.046ng/mL( < 0.001),灵敏度为 100.0%,特异性为 98.3%。总之,我们发现血清 HEPC-FER 比值的准确性为 99.2%,可作为诊断β-TM 患者铁过载的优秀指标,将其与非地中海贫血对照组区分开来。

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