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肝脏铁暴露的持续时间会增加遗传性血色素沉着症发生显著纤维化的风险:磁共振成像的新作用

Duration of hepatic iron exposure increases the risk of significant fibrosis in hereditary hemochromatosis: a new role for magnetic resonance imaging.

作者信息

Olynyk John K, St Pierre Timothy G, Britton Robert S, Brunt Elizabeth M, Bacon Bruce R

机构信息

School of Medicine and Pharmacology, University of Western Australia, Nedlands, Western Australia.

出版信息

Am J Gastroenterol. 2005 Apr;100(4):837-41. doi: 10.1111/j.1572-0241.2005.41287.x.

Abstract

OBJECTIVES

Hepatic fibrosis is a complication of hereditary hemochromatosis. The aim of this study was to determine whether the product of the magnitude and duration of hepatic iron exposure is related to the risk of significant fibrosis.

METHODS

Receiver-operating characteristic curve analysis to determine the utility of hepatic iron concentration (HIC) and age in the diagnosis of low- or high-grade fibrosis was undertaken retrospectively in 60 subjects who had undergone liver biopsy for assessment of hereditary hemochromatosis. A prospective pilot study was then conducted in 10 additional subjects to evaluate utility of magnetic resonance imaging (MRI) measurements of HIC to predict fibrosis.

RESULTS

Eighteen subjects had high-grade fibrosis while 42 subjects had low-grade fibrosis. Hepatic iron concentration alone was highly sensitive (100%) but of limited specificity (67%) in diagnosis of high-grade fibrosis. The product of [HIC x age] had a sensitivity and specificity of 100% and 86%, respectively, for diagnosis of high-grade fibrosis. Magnetic resonance imaging measurements also provided accurate assignment of subjects into fibrosis severity groups.

CONCLUSIONS

Duration of exposure to iron is important in the development of hepatic fibrosis in hereditary hemochromatosis. The product of HIC and age is highly sensitive and specific for diagnosis of high-grade fibrosis and can be obtained using MRI.

摘要

目的

肝纤维化是遗传性血色素沉着症的一种并发症。本研究的目的是确定肝脏铁暴露的量和持续时间的乘积是否与显著纤维化的风险相关。

方法

对60例因评估遗传性血色素沉着症而接受肝脏活检的受试者进行回顾性研究,采用受试者操作特征曲线分析来确定肝脏铁浓度(HIC)和年龄在诊断低度或高度纤维化中的效用。然后对另外10名受试者进行前瞻性初步研究,以评估磁共振成像(MRI)测量HIC预测纤维化的效用。

结果

18例受试者有高度纤维化,42例受试者有低度纤维化。单独的肝脏铁浓度在诊断高度纤维化时敏感性高(100%)但特异性有限(67%)。[HIC×年龄]的乘积在诊断高度纤维化时敏感性和特异性分别为100%和86%。MRI测量也能准确地将受试者分为不同纤维化严重程度组。

结论

铁暴露的持续时间在遗传性血色素沉着症肝纤维化的发生中很重要。HIC与年龄的乘积对诊断高度纤维化具有高度敏感性和特异性,并且可以通过MRI获得。

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