Population-based epidemiologic study of Wilson's disease in Taiwan

Background: The purpose of the study is to investigate the epidemiology and medical expenses of Wilson's disease in Taiwan. Methods: Cases of Wilson's disease were identified from the National Health Insurance Research Database with corresponding International Classification of Diseases, Ninth Revision (ICD-9) code 275.1 from January 2000 to December 2005. Age and sex-specific incidences were estimated by dividing the incidence number by population data obtained from the Department of Statistics, Ministry of the Interior. Results: During the study period, 495 cases of Wilson's disease were identified. The male to female ratio was 1.28. The average annual incidence rate was 0.27 per 100 000, and the incidence rate peaked in the age group of 15-19 years. Most cases were juvenile and young adults. The prevalence increased steadily during the study period. Cirrhosis of the liver was the most common co-existing condition. Conclusions: This is the first population-based epidemiologic study of Wilson's disease in Taiwan. Because of the effective and affordable treatment, the prevalence of Wilson's disease in Taiwan is expected to increase continuously.