ArticleUnited European gastroenterology journal2023
Four-fold increased mortality rate in patients with Wilson's disease: A population-based cohort study of 151 patients.
Article in United European gastroenterology journal, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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15 citing papers in PubMed, 19 citations in OpenAlex.
- Disorders Mimicking Wilson's Disease: Clinical, Biochemical, and Molecular Perspectives for Accurate Differential Diagnosis.Diagnostics (Basel, Switzerland) · 2026Review
- Global burden of Wilson disease: a comprehensive evidence synthesis.Orphanet journal of rare diseases · 2026Article
- Altered static and dynamic regional homogeneity in basal ganglia-thalamocortical circuits and their association with neuropsychiatric manifestations in Wilson's disease.Frontiers in neuroscience · 2026Article
- Altered Sleep Patterns in Wilson's Disease Including Shortened REM Latency.Diagnostics (Basel, Switzerland) · 2025Article
- Improvement in sleep following treatment initiation in newly diagnosed, treatment-naïve patients with Wilson's disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- Oxidative Stress and Psychiatric Symptoms in Wilson's Disease.International journal of molecular sciences · 2025Article
- Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry.Orphanet journal of rare diseases · 2025Article
- Diagnostic accuracy in the Swedish national patient register: a review including diagnoses in the outpatient register.European journal of epidemiology · 2025Review
- Diet quality and nutritional risk in patients with Wilson's disease: a cross-sectional study.Frontiers in nutrition · 2025Article
- Clinical and Genetic Profile of Pediatric and Adult Wilson's Disease in India.Gastro hep advances · 2025Article
- Psychiatric Symptoms in Wilson's Disease-Consequence ofInternational journal of molecular sciences · 2024Review
- Epidemiology of Wilson's Disease and Pathogenic Variants of theInternational journal of molecular sciences · 2024Review
- Wilson's disease-An early diagnosis to improve outcomes.United European gastroenterology journal · 2023Article
- Four-fold increased mortality rate in patients with Wilson's disease: A population-based cohort study of 151 patients.United European gastroenterology journal · 2023Article
- Challenges with neuropsychiatric Wilson's disease.United European gastroenterology journal · 2023Article
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Authors and funding
6 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
aimsFew studies have investigated mortality rates in patients with Wilson's disease and compared these to the general population. Here, we examined several clinical outcomes (including cardiovascular, psychiatric, neurologic conditions) in a population-based study of patients with Wilson's disease.
methodWe used nationwide registers to identify all patients with a first diagnosis of Wilson's disease between 2002 and 2020 in Sweden. Each patient was matched by age, sex, and municipality with up to 10 reference individuals from the general population. Validated registers were used to investigate outcomes up to 19 years after baseline in patients and reference individuals.
resultsWe identified 151 patients with Wilson's disease matched with 1441 reference individuals. Median age at baseline was 26 years (IQR 17-42) and 50% were males. During a median follow-up of 6.6 years (IQR 2.9-12.9), 10 (6.6%) patients with Wilson's disease died compared with 31 (2.2%) reference individuals. This translated to a hazard ratio (HR) of 3.8 (95%CI = 1.8-8.1). Mortality was higher among Wilson's disease patients with baseline neuropsychiatric diagnoses (HR 7.9, 95%CI = 2.9-21.8). Cumulative mortality over 10 years was 9.3% (95%CI = 5.0-16.8) in Wilson's disease, compared to 2.4% (95%CI = 1.6-3.6) in reference individuals. We observed significantly elevated risks in the Wilson's disease group for incident cardiovascular disease, and incident psychiatric and neurological conditions when considering liver transplantation or death from other causes as competing events.
conclusionIn this large population-based cohort study, patients with Wilson's disease had an almost four-fold increased mortality rate compared with matched individuals from the general population.
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