{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,6]],"date-time":"2026-01-06T17:31:54Z","timestamp":1767720714786,"version":"build-2065373602"},"reference-count":25,"publisher":"S. Karger AG","license":[{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["GE Port J Gastroenterol"],"abstract":"<jats:p>Introduction: Wilson\u2019s disease is a rare autosomal recessive inherited disorder of human copper metabolism. The spectrum of clinical manifestations of liver disease ranges from asymptomatic to cirrhosis and acute liver failure. Unlike the increased frequency of hepatobiliary tumours in other chronic liver diseases, the incidence of these tumours in Wilson\u2019s disease is an uncommon event, even in cirrhotic patients. Case Presentation: We describe two clinical cases of cirrhotic patients with Wilson disease diagnosed with cholangiocarcinoma. Discussion: The occurrence of hepatobiliary malignancies in Wilson\u2019s disease is rare, even in cirrhotic patients. The exact reasons for this low prevalence are unclear, with some conflicting data. Copper\u2019s role in chromatin stabilization and angiogenesis, along with copper deficiency due to Wilson\u2019s disease and its treatment, may reduce tumour growth. The current guidelines do not have specific directives regarding cholangiocarcinoma screening in patients with cirrhosis secondary to Wilson\u2019s disease. Despite the lower incidence of malignancies in Wilson\u2019s disease, vigilant monitoring is advised. <\/jats:p>","DOI":"10.1159\/000548202","type":"journal-article","created":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T16:00:59Z","timestamp":1756742459000},"page":"1-5","source":"Crossref","is-referenced-by-count":1,"title":["Intrahepatic Cholangiocarcinoma in Wilson\u2019s Disease: 2 Case Reports"],"prefix":"10.1159","author":[{"given":"Joana","family":"Reis Aguiar","sequence":"first","affiliation":[]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2072-8763","authenticated-orcid":false,"given":"Helena","family":"Pessegueiro Miranda","sequence":"additional","affiliation":[]},{"given":"Cl\u00e1udia","family":"Pereira","sequence":"additional","affiliation":[]}],"member":"127","published-online":{"date-parts":[[2025,9,1]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(07)60196-2"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0676.2003.00824.x"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2011.11.007"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1016\/j.cld.2017.06.011"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.aohep.2019.04.016"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1093\/qjmed\/hcg114"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v19.i13.2110"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1111\/liv.12727"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2006.00562.x"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1007\/s00535-004-1466-y"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1016\/s0016-5085(19)30524-4"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1136\/gut.24.8.767"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1093\/carcin\/14.7.1303"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1016\/j.mrfmmm.2012.03.010"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.1537"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2008.03.016"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1016\/s1542-3565(05)00181-3"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2023.03.010"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2024.08.028"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1097\/HEP.0000000000000466"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1136\/gutjnl-2023-331695"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.1136\/gutjnl-2023-330029"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.1002\/hep.32801"},{"key":"ref24","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(92)90053-s"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/531640"}],"container-title":["GE - Portuguese Journal of Gastroenterology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/article-pdf\/doi\/10.1159\/000548202","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T16:01:23Z","timestamp":1759334483000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000548202"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9,1]]},"references-count":25,"URL":"https:\/\/doi.org\/10.1159\/000548202","archive":["Portico"],"relation":{"is-supplemented-by":[{"id-type":"doi","id":"10.6084\/m9.figshare.30021760","asserted-by":"subject"}]},"ISSN":["2341-4545","2387-1954"],"issn-type":[{"type":"print","value":"2341-4545"},{"type":"electronic","value":"2387-1954"}],"subject":[],"published":{"date-parts":[[2025,9,1]]}}}