Department of Medicine, The University of Hong Kong, Queen Mary Hospital, Hong Kong 999077, China.
World J Gastroenterol. 2017 Nov 21;23(43):7716-7726. doi: 10.3748/wjg.v23.i43.7716.
To investigate the epidemiology and natural history of Wilson's disease in the Chinese.
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.
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 ( = 0.010), whereas there was an increase in the annual prevalence from 7.80 to 25.20 per million ( < 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.
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.
研究中国人威尔逊病的流行病学和自然史。
通过检索香港医院管理局医院病历电子检索，获取 2000 年至 2016 年间的威尔逊病病例。我们通过国际疾病分类（ICD-9）代码识别威尔逊病病例。我们分析了威尔逊病的发病率、患病率和不良结局。
我们共确定了 211 例患者（男性 104 例，女性 107 例；中位年龄 27.2 岁，IQR：17.1-38.6 岁；随访时间 8.0 年，IQR：5.0-14.0 年）。年平均发病率为 1.44/百万人年，患病率为 17.93/百万人。2000 年至 2016 年，年发病率从 1.65 降至 1.23/百万人年（=0.010），而年患病率从 7.80 增至 25.20/百万人（<0.001）。在 176 例有肝脏受累的患者中，38 例（21.6%）有肝硬化，3 例（1.7%）发生肝细胞癌，24 例（13.6%）接受了肝移植，26 例（14.8%）死亡。7 例患者同时患有慢性乙型或丙型病毒性肝炎。总的 5 年和 10 年生存率分别为 92.6%和 89.5%，无移植生存率分别为 91.8%和 87.4%。肝硬化和可能的慢性病毒性肝炎与较差的总生存率相关。
香港威尔逊病的患病率显著增加。除肝硬化或合并病毒性肝炎患者外，预后良好。
