Premium
Wilson's Disease in Finland: A Nationwide Population‐Based Study
Author(s) -
Sipilä Jussi O.T.,
Hietala Marja,
Kytö Ville,
Kaasinen Valtteri
Publication year - 2020
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.28207
Subject(s) - medicine , interquartile range , confidence interval , hazard ratio , asymptomatic , epidemiology , pediatrics , incidence (geometry) , population , disease , environmental health , physics , optics
Background Data on the epidemiology and prognosis of Wilson's disease are scarce, and no clinical data are available from Finland. Methods All persons diagnosed and treated for Wilson's disease in Finnish hospitals in 1998 to 2017 were identified. Data were collected from national registries and patient charts. Results The point prevalence was 0.45/100,000 (95% confidence interval, 0.29–0.67) on December 31, 2017, but no more than 0.35/100,000 (95% confidence interval, 0.21–0.55) among native Finns. Annual incidence was 0.016/100,000 (95% confidence interval, 0.0093–0.026). Median age at diagnosis was 15.8 years (interquartile range, 8.3–32.2; range, 3.8–48.1 years). Upon presentation, liver damage was observed in 58%, neurological signs and symptoms (most often tremor and dysarthria) in 40%, and 32% of patients were asymptomatic. Patients had poorer long‐term survival (hazard ratio, 2.92 for death; P = 0.005) compared with matched controls. Conclusions Wilson's disease is very rare in Finland. Patients have an increased risk of death indicating an unmet treatment need. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society