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肝铁或全身铁负荷在 HFE 血色病中评估晚期肝纤维化的效用。

Utility of hepatic or total body iron burden in the assessment of advanced hepatic fibrosis in HFE hemochromatosis.

机构信息

Department of Gastroenterology & Hepatology, Fiona Stanley Fremantle Hospital Group, Murdoch, Western Australia, Australia.

Faculty of Medicine, The University of Queensland, Herston, Brisbane, Queensland, Australia.

出版信息

Sci Rep. 2019 Dec 27;9(1):20234. doi: 10.1038/s41598-019-56732-0.

DOI:10.1038/s41598-019-56732-0
PMID:31882912
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6934689/
Abstract

Development of advanced hepatic fibrosis in HFE Hemochromatosis (HH) is influenced by hepatic iron concentration (HIC) and age. In patients with HH, it is important to assess the likelihood of cirrhosis and thus the need for confirmatory liver biopsy. Therapeutic phlebotomy also provides an estimate of mobilisable iron stores. We determined whether mobilisable iron stores may predict the presence of advanced fibrosis. Retrospective analysis of 137 male and 65 female HH subjects was undertaken. Biochemical, histological and phlebotomy data were available on all subjects. The mean values of HIC, HIC × [age], mobilisable iron, mobilisable iron × [age] and serum ferritin in the cohort were higher in the group with advanced fibrosis. HIC had an optimum sensitivity and specificity of 73% for the diagnosis of advanced liver fibrosis, with a cut-off HIC level of 200 µmol/g (AUROC 0.83, p < 0.0001). AUROC for HIC was greater in females (0.93) than males (0.79). Mobilisable iron had an optimum sensitivity and specificity both of 83% at a cut-off of 9.6 g for the prediction of advanced fibrosis in all subjects (AUROC 0.92, p < 0.0001). Mobilisable iron stores provide a simple, clinically useful indication of the risk of advanced fibrosis and should routinely be considered.

摘要

在遗传性血色病(HH)中,高级肝纤维化的发展受肝铁浓度(HIC)和年龄的影响。在 HH 患者中,评估肝硬化的可能性以及因此需要进行确认性肝活检非常重要。治疗性放血术还可提供可动员铁储存的估计值。我们确定了可动员的铁储存是否可以预测是否存在晚期纤维化。对 137 名男性和 65 名女性 HH 受试者进行了回顾性分析。所有受试者均具有生化、组织学和放血术数据。在纤维化程度较高的组中,队列的 HIC、HIC×[年龄]、可动员铁、可动员铁×[年龄]和血清铁蛋白的平均值较高。HIC 对晚期肝纤维化的诊断具有最佳的灵敏度和特异性,为 73%,最佳截止 HIC 水平为 200μmol/g(AUROC 0.83,p<0.0001)。HIC 的 AUROC 在女性(0.93)中大于男性(0.79)。在所有受试者中,可动员铁的最佳截止值为 9.6g 时,其灵敏度和特异性均为 83%,可用于预测晚期纤维化(AUROC 0.92,p<0.0001)。可动员的铁储存提供了一种简单、临床上有用的晚期纤维化风险指标,应常规考虑。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b83b/6934689/4962a10c97cf/41598_2019_56732_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b83b/6934689/81ed5adda0bf/41598_2019_56732_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b83b/6934689/4962a10c97cf/41598_2019_56732_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b83b/6934689/81ed5adda0bf/41598_2019_56732_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b83b/6934689/4962a10c97cf/41598_2019_56732_Fig2_HTML.jpg

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