Aim: To investigate the epidemiology and natural history of Wilson's disease in the Chinese.
Methods: Data were retrieved via electronic search of hospital medical registry of the Hong Kong Hospital Authority, which covers all the public healthcare services. We identified cases of Wilson's disease between 2000 and 2016 by the International Classification of Diseases (ICD)-9 code. We analyzed the incidence rate, prevalence and adverse outcomes of Wilson's disease.
Results: We identified 211 patients (male cases 104; female cases 107; median age 27.2 years, IQR: 17.1-38.6 years; duration of follow-up 8.0 years, IQR: 5.0-14.0 years). The average annual incidence rate was 1.44 per million person-years while the prevalence was 17.93 per million. Between 2000 and 2016, there was a decrease in the annual incidence rate from 1.65 to 1.23 per million person-years (P = 0.010), whereas there was an increase in the annual prevalence from 7.80 to 25.20 per million (P < 0.001). Among the 176 cases with hepatic involvement, 38 (21.6%) had cirrhosis, three (1.7%) developed hepatocellular carcinoma, 24 (13.6%) underwent liver transplantations, and 26 (14.8%) died. Seven patients had concomitant chronic viral hepatitis B or C. The 5-year and 10-years rates of overall survival were 92.6% and 89.5%, and for transplant-free survival rates 91.8% and 87.4%, respectively. Cirrhosis and possibly chronic viral hepatitis were associated with poorer overall survival.
Conclusion: There was a significant increase in the prevalence of Wilson's disease in Hong Kong. The prognosis was favorable except for those with cirrhosis or concomitant viral hepatitis.
Keywords: Cirrhosis; Hepaticolenticular degeneration; Hepatocellular carcinoma; Transplantation.