{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T05:35:33Z","timestamp":1792992933625,"version":"4.5.0"},"reference-count":0,"publisher":"The Korean Association for the Study of the Liver","issue":"4","license":[{"start":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T00:00:00Z","timestamp":1786406400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/3.0\/"}],"content-domain":{"domain":["e-cmh.org"],"crossmark-restriction":true},"short-container-title":["Clin Mol Hepatol"],"published-print":{"date-parts":[[2026,10,1]]},"abstract":"<jats:p>Background\/Aims: Wilson\u2019s disease (WD) is an inherited and treatable disorder of copper metabolism with hepatic or neurological manifestations. It continues to pose challenges in clinical management, and long-term outcome data remain limited. This study investigated hepatic involvement and clinical outcomes in cohorts from Taiwan.Methods: Longitudinal data from 146 patients with WD, retrieved from the medical database of a tertiary referral center, were analyzed (mean age at diagnosis 20.7\u00b112.4 years; mean follow-up 16.8\u00b110.8 years; maximum 51.8 years). All patients received standard treatment and care.Results: Nine patients (6.2%) underwent liver transplantation, 6 patients (4.1%) died, and 3 patients (2.1%) developed hepatocellular carcinoma (HCC). Thrombocytopenia &lt;100 K\/\u03bcL at diagnosis was associated with reduced survival (&lt;i&gt;P&lt;\/i&gt;=0.0001). Univariate Cox regression identified thrombocytopenia at diagnosis as a strong predictor of liver transplantation or death (&lt;i&gt;P&lt;\/i&gt;=0.0001; hazard ratio 8.41; 95% confidence interval [CI] 2.97\u201323.78). Consistently, thrombocytopenia &lt;100 K\/\u03bcL was associated with reduced survival (&lt;i&gt;P&lt;\/i&gt;&lt;0.0001) in an external cohort of 155 patients. In the original cohort, the estimated annual HCC risk was 0.12% (95% CI 0.03\u22120.36), increasing to 0.86% (95% CI 0.18\u22122.5) in those with thrombocytopenia at diagnosis (&lt;i&gt;P&lt;\/i&gt;=0.029). An exploratory Firth penalized logistic regression showed that older age at diagnosis was associated with higher odds of developing HCC (odds ratio 1.096; 95% CI 1.009\u20131.191; &lt;i&gt;P&lt;\/i&gt;=0.031).Conclusions: WD patients have a high rate of liver fibrosis, regardless of whether they present with neurological and hepatic symptoms, and are at risk of developing HCC, warranting lifelong hepatic surveillance.<\/jats:p>","DOI":"10.3350\/cmh.2026.0402","type":"journal-article","created":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T07:42:17Z","timestamp":1786434137000},"page":"e2026.0402","update-policy":"https:\/\/doi.org\/10.3350\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Long-term liver outcomes in Wilson\u2019s disease: predictors of survival and hepatocellular carcinoma"],"prefix":"10.3350","volume":"32","author":[{"given":"Chien-Ting","family":"Hsu","sequence":"first","affiliation":[{"name":"Department of Pediatrics, National Taiwan University Hospital Hsin-Chu Branch Biomedical Park Hospital, Hsinchu County, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sung-Pin","family":"Fan","sequence":"additional","affiliation":[{"name":"Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jia-Feng","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, National Taiwan University Children\u2019s Hospital, Taipei, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chin-Chang","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Neurology, Chang Gung Memorial Hospital-Linkou Medical Center, Taoyuan, Taiwan"},{"name":"Department of Neurology, Chang Gung University, Taoyuan, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tung-Hung","family":"Su","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan"},{"name":"Department of Medical Research, National Taiwan University Hospital Hsin-Chu Branch Biomedical Park Hospital, Hsinchu County, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tai-Chung","family":"Tseng","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ni-Chung","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, National Taiwan University Children\u2019s Hospital, Taipei, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chin-Hsien","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4074-5838","authenticated-orcid":true,"given":"Huey-Ling","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, National Taiwan University Children\u2019s Hospital, Taipei, Taiwan"},{"name":"Department of Medical Education and Bioethics, National Taiwan University College of Medicine, Taipei, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1158-5249","authenticated-orcid":true,"given":"Yen-Hsuan","family":"Ni","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, National Taiwan University Children\u2019s Hospital, Taipei, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1902","container-title":["Clinical and Molecular Hepatology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/e-cmh.org\/upload\/pdf\/cmh-2026-0402.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/e-cmh.org\/journal\/view.php?doi=10.3350\/cmh.2026.0402","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/e-cmh.org\/upload\/pdf\/cmh-2026-0402.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T05:02:41Z","timestamp":1792990961000},"score":1,"resource":{"primary":{"URL":"http:\/\/e-cmh.org\/journal\/view.php?doi=10.3350\/cmh.2026.0402"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10,1]]},"references-count":0,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,10]]}},"URL":"https:\/\/doi.org\/10.3350\/cmh.2026.0402","relation":{},"ISSN":["2287-2728","2287-285X"],"issn-type":[{"value":"2287-2728","type":"print"},{"value":"2287-285X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10,1]]},"assertion":[{"value":"2026-04-01","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-04","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-11","order":2,"name":"published","label":"Published online","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-10-01","order":3,"name":"published","label":"Print publication","group":{"name":"publication_history","label":"Publication History"}}]}}