Retrospective Cohort Study
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Nov 21, 2017; 23(43): 7716-7726
Published online Nov 21, 2017. doi: 10.3748/wjg.v23.i43.7716
Epidemiology and natural history of Wilson’s disease in the Chinese: A territory-based study in Hong Kong between 2000 and 2016
Ka-Shing Cheung, Wai-Kay Seto, James Fung, Lung-Yi Mak, Ching-Lung Lai, Man-Fung Yuen
Ka-Shing Cheung, Wai-Kay Seto, James Fung, Lung-Yi Mak, Ching-Lung Lai, Man-Fung Yuen, Department of Medicine, The University of Hong Kong, Queen Mary Hospital, Hong Kong 999077, China
Wai-Kay Seto, James Fung, Ching-Lung Lai, Man-Fung Yuen, State Key Laboratory for Liver Research, The University of Hong Kong, Hong Kong 999077, China
Author contributions: Cheung KS designed and performed the study, were involved in statistical analysis and interpretation of the data, and wrote the manuscript; Seto WK and Mak LY performed the study, statistical analysis and interpretation of the data; Fung J, Lai CL and Yuen MF revised and edited the manuscript.
Institutional review board statement: This study was approved by The Institutional Review Board of The University of Hong Kong/Hospital Authority, Hong Kong West Cluster.
Informed consent statement: The Institutional Review Board of The University of Hong Kong/Hospital Authority waived the need for written informed consent as there were no direct contact with eligible subjects and no additional blood taking.
Conflict-of-interest statement: The authors have no conflicts of interest.
Data sharing statement: No additional data are available.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Man-Fung Yuen, Professor, Department of Medicine, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong 999077, China. mfyuen@hkucc.hku.hk
Telephone: +852-22553984 Fax: +852-28162863
Received: July 28, 2017
Peer-review started: July 30, 2017
First decision: August 29, 2017
Revised: September 25, 2017
Accepted: September 28, 2017
Article in press: September 28, 2017
Published online: November 21, 2017
Processing time: 114 Days and 22.7 Hours
Abstract
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: Hepaticolenticular degeneration; Cirrhosis; Hepatocellular carcinoma; Transplantation

Core tip: There are few studies on the epidemiology and natural history of Wilson’s disease in Asia. The present territory-based study was the first to describe both the epidemiology and natural history of Wilson’s disease over a long period of time (a span of 17 years from 2000 to 2016) in the Chinese. There was a significant increase in the number of cases of Wilson’s disease in Hong Kong. The prognosis was favorable except for those with cirrhosis or concomitant viral hepatitis.