Liver Assessment in Patients with Ataxia-Telangiectasia: Transient Elastography Detects Early Stages of Steatosis and Fibrosis.

IF 2.7 4区医学 Q2 Medicine Canadian Journal of Gastroenterology and Hepatology Pub Date : 2023-01-01 DOI:10.1155/2023/2877350

H Donath, S Wölke, V Knop, U Heß, R P Duecker, J Trischler, T Poynard, R Schubert, S Zielen

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Abstract

Background: Ataxia-telangiectasia (A-T) is a rare autosomal-recessive multisystem disorder characterized by pronounced cerebellar ataxia, telangiectasia, cancer predisposition, and altered body composition. Liver diseases with steatosis, fibrosis, and hepatocellular carcinoma are frequent findings in older patients but sensitive noninvasive diagnostic tools are lacking.

Objectives: To determine the sensitivity of transient elastography (TE) as a screening tool for early hepatic tissue changes and serum biomarkers for liver disease.

Methods: Thirty-one A-T patients aged 2 to 25 years were examined prospectively from 2016-2018 by TE. In addition, we evaluated the diagnostic performance of liver biomarkers for steatosis and necroinflammatory activity (SteatoTest and ActiTest, Biopredictive, Paris) compared to TE. For calculation and comparison, patients were divided into two groups (<12, >12 years of age).

Results: TE revealed steatosis in 2/21 (10%) younger patients compared to 9/10 (90%) older patients. Fibrosis was present in 3/10 (30%) older patients as assessed by TE. We found a significant correlation of steatosis with SteatoTest, alpha-fetoprotein (AFP), HbA1c, and triglycerides. Liver stiffness correlated significantly with SteatoTest, ActiTest, HbA1c, and triglycerides.

Conclusion: Liver disease is a common finding in older A-T patients. TE is an objective measure to detect early stages of steatosis and fibrosis. SteatoTest and ActiTest are a good diagnostic assessment for steatosis and necroinflammatory activity in patients with A-T and confirmed the TE results.

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失调性毛细血管扩张患者的肝脏评估:瞬时弹性成像检测早期脂肪变性和纤维化。

背景:共济失调-毛细血管扩张症(a-t)是一种罕见的常染色体隐性多系统疾病，其特征是小脑共济失调、毛细血管扩张、癌症易感性和身体成分改变。肝脏疾病伴脂肪变性、纤维化和肝细胞癌是老年患者的常见发现，但缺乏敏感的无创诊断工具。目的:确定瞬变弹性成像(TE)作为早期肝组织变化和肝脏疾病血清生物标志物筛查工具的敏感性。方法:对2016-2018年31例2 ~ 25岁的A-T患者进行TE前瞻性检查。此外，与TE相比，我们评估了肝脏脂肪变性和坏死炎症活性生物标志物(SteatoTest和ActiTest, Biopredictive, Paris)的诊断性能。为了计算和比较，将患者分为两组(12岁)。结果:TE显示2/21(10%)的年轻患者有脂肪变性，而9/10(90%)的老年患者有脂肪变性。通过TE评估，3/10(30%)的老年患者存在纤维化。我们发现脂肪变性与脂肪测试、甲胎蛋白(AFP)、糖化血红蛋白(HbA1c)和甘油三酯有显著相关性。肝硬度与脂肪测试、活动测试、糖化血红蛋白和甘油三酯显著相关。结论:肝病是老年a - t患者的常见发现。TE是检测早期脂肪变性和纤维化的客观指标。脂肪测试(SteatoTest)和活动测试(ActiTest)是a - t患者脂肪变性和坏死炎症活动的良好诊断评估，并证实了TE的结果。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Canadian Journal of Gastroenterology and Hepatology GASTROENTEROLOGY & HEPATOLOGY-

CiteScore

4.80

自引率

0.00%

发文量

审稿时长

37 weeks

期刊介绍： Canadian Journal of Gastroenterology and Hepatology is a peer-reviewed, open access journal that publishes original research articles, review articles, and clinical studies in all areas of gastroenterology and liver disease - medicine and surgery. The Canadian Journal of Gastroenterology and Hepatology is sponsored by the Canadian Association of Gastroenterology and the Canadian Association for the Study of the Liver.