Abstract
Very few population-based studies have examined the epidemiology of Wilson’s disease (WD). We investigated the epidemiology of WD using the National Health Insurance Service (NHIS) database in South Korea. We analyzed not only the statistical variables of WD, but also those of WD-related diseases. WD patients were identified with the relevant International Classification of Diseases-10 code out of 50.5 million people. We used the NHIS database from 2009 to 2016 and analyzed the incidence rate, prevalence, and clinical symptoms of WD. A total of 1,333 patients were identified. The average annual incidence rate was 3.8 per million person-years. The prevalence was 38.7 per million people. The mean diagnostic age was 26.1 ± 17.2 with earlier diagnosis in men (P = 0.0003). Among the patients, 988 (74.1%) had hepatic symptoms, 510 (38.3%) had neurologic symptoms, and 601 (45.1%) had psychiatric symptoms. Before the diagnosis of WD, 350 (26.3%) had neurologic symptoms, and 427 (32%) had psychiatric symptoms. The annual mortality rate was 0.7%. Age, liver cirrhosis, and liver failure correlated with a fatal prognosis (P < 0.05). Many patients showed neurologic and psychiatric symptoms before they were diagnosed with WD. Prognosis correlated with age, liver cirrhosis, and liver failure.
Highlights
Very few population-based studies have examined the epidemiology of Wilson’s disease (WD)
Approximately 50.5 million people, must be enrolled in the health insurance provided by the National Health Insurance Service (NHIS), and all types of medical institutions must register with the NHIS
WD patients were defined as people who had an outpatient visit or admission history with the principal diagnostic code of E830 in the Korean Standard Classification of Diseases 2015 (KCD), which is the Korean version of the International Classification of Disease, 10th Revision
Summary
Very few population-based studies have examined the epidemiology of Wilson’s disease (WD). We used the NHIS database from 2009 to 2016 and analyzed the incidence rate, prevalence, and clinical symptoms of WD. Many patients showed neurologic and psychiatric symptoms before they were diagnosed with WD. Wilson’s disease (WD) is an autosomal-recessive disorder associated with copper metabolism that produces abnormal accumulation of copper in the liver, brain, kidneys, and other organs[1] It is caused by mutations in the ATP7B gene, which is involved in transporting copper across cell m embranes[2,3]. Studies have reported that neurologic and psychiatric manifestations are present in up to 40% and 10–25% of patients respectively, at the time they are diagnosed with WD6,7. Our aim in this study was to evaluate the incidence rate, prevalence, treatment efficacy, and symptoms of WD in South Korea using the National Health Insurance Service (NHIS) database
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