{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,2]],"date-time":"2025-07-02T01:51:04Z","timestamp":1751421064510,"version":"3.40.5"},"reference-count":68,"publisher":"Wiley","license":[{"start":{"date-parts":[[2023,3,11]],"date-time":"2023-03-11T00:00:00Z","timestamp":1678492800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Canadian Journal of Gastroenterology and Hepatology"],"published-print":{"date-parts":[[2023,3,11]]},"abstract":"<jats:p>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\u2009years were examined prospectively from 2016\u20132018 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 (&lt;12, &gt;12\u2009years 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.<\/jats:p>","DOI":"10.1155\/2023\/2877350","type":"journal-article","created":{"date-parts":[[2023,3,11]],"date-time":"2023-03-11T20:20:07Z","timestamp":1678566007000},"page":"1-9","source":"Crossref","is-referenced-by-count":2,"title":["Liver Assessment in Patients with Ataxia-Telangiectasia: Transient Elastography Detects Early Stages of Steatosis and Fibrosis"],"prefix":"10.1155","volume":"2023","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1563-946X","authenticated-orcid":true,"given":"H.","family":"Donath","sequence":"first","affiliation":[{"name":"Department for Children and Adolescents, Division of Allergology, Pulmonology and Cystic Fibrosis, University Hospital Frankfurt, Goethe University, Frankfurt, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"W\u00f6lke","sequence":"additional","affiliation":[{"name":"Department for Children and Adolescents, Division of Allergology, Pulmonology and Cystic Fibrosis, University Hospital Frankfurt, Goethe University, Frankfurt, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V.","family":"Knop","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine 1, University Hospital Frankfurt, Goethe University, Frankfurt, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"U.","family":"He\u00df","sequence":"additional","affiliation":[{"name":"Department for Children and Adolescents, Division of Allergology, Pulmonology and Cystic Fibrosis, University Hospital Frankfurt, Goethe University, Frankfurt, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. 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