Background/Aims
Wilson’s 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 ± 12.4 years; mean follow-up 16.8 ± 10.8 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 <100 K/μL at diagnosis was associated with reduced survival (P=0.0001). Univariate Cox regression identified thrombocytopenia at diagnosis as a strong predictor of liver transplantation or death (P = 0.0001; hazard ratio (HR): 8.41; 95% CI: 2.97–23.78). Consistently, thrombocytopenia <100 K/μL was associated with reduced survival (P < 0.0001) in an external cohort of 155 patients. In the original cohort, the estimated annual HCC risk was 0.12% (95% CI: 0.03−0.36), increasing to 0.86% (95% CI: 0.18−2.5) in those with thrombocytopenia at diagnosis (P=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: 1.009–1.191; P = 0.031).
Conclusion
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.

It is our great pleasure to announce that the Taehan Kan Hakhoe Chi (The Korean Journal of Hepatology) was approved for listing, from 2002, in the Index Medicus, Medline/PubMed of the National Library of Medicine, NIH of USA. Herein, I review the searching tools employing a Medical Subject Heading (MeSH) such as liver disease and liver neoplasm or an author index for this Journal in the PubMed at a website. Of course, The Korean Journal of Hepatology should be continually striving to be upgraded. Dream comes true.(Korean J Hepatol 2003; 9:35-41)