Evidence map›Paper›PMID 34381985›Full record

ArticleJHEP reports : innovation in hepatology2021

Estimating the clinical prevalence of Wilson's disease in the UK.

Pramudi Wijayasiri, Jatinder Hayre, Edward S Nicholson, Philip Kaye, Emilie A Wilkes, Jonathan Evans, Guruprasad P Aithal, Gabriela Jones, Fiona Pearce, Aloysious D Aravinthan

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Article in JHEP reports : innovation in hepatology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

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7citing papers in PubMed
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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

Who cites it

7 citing papers in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

10 authors.

Pramudi WijayasiriNIHR Nottingham Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, UK.
Jatinder HayreNottingham Digestive Diseases Centre, Translational Medical Sciences, School of Medicine, University of Nottingham, UK.
Edward S NicholsonDepartment of Pharmacy, Nottingham University Hospitals NHS Trust, UK.
Philip KayeDepartment of Pathology, Nottingham University Hospitals NHS Trust, UK.
Emilie A WilkesNIHR Nottingham Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, UK.
Jonathan EvansDepartment of Neurology, Nottingham University Hospitals NHS Trust, UK.
Guruprasad P AithalNIHR Nottingham Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, UK.
Gabriela JonesDepartment of Clinical Genetics, Nottingham University Hospitals NHS Trust, UK.
Fiona PearceNIHR Nottingham Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, UK.
Aloysious D AravinthanNIHR Nottingham Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, UK.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

BACKGROUND &

aimsThe clinical prevalence of Wilson's disease (WD) in the UK remains unknown. The estimated genetic prevalence in the UK, 142/million, is higher than the clinical prevalence (15/million) reported in other European studies. The aim of this study was to estimate the clinical prevalence of WD utilising readily available laboratory and clinical data.

methodPatients with WD who attended Nottingham University Hospital NHS Trust (NUH) between 2011 and 2018 were identified using multiple sources of case ascertainment: serum ceruloplasmin, 24-hour urinary copper, 'Wilson' in liver biopsy report, hospital prescription for penicillamine/trientine/zinc and admission coded with ICD-10 Code E83.0 (disorder of copper metabolism). Potential cases were identified using the Leipzig score, diagnosis was confirmed in hospital records and the point prevalence was calculated using the Office for National Statistics mid-2017 population estimates.

resultsA total of 1,794 patients were identified from ≥1 source; 19 patients had WD, of whom 11 were from within the study catchment area and alive at the time of point prevalence estimation. Twenty-nine patients had a Leipzig score ≥2 without a diagnosis of WD, but none had WD on screening (n = 16). The overall prevalence of WD was 15.5/million; males 16.9/million and females 14.1/million.

conclusionThis is the first UK population-based study to assess the clinical prevalence of WD. The reported clinical prevalence is lower than the UK genetic prevalence, but comparable to the clinical prevalence reported in Europe. The case ascertainment approach used in this study may be cost-effective, and similar practises could be adopted nationally. LAY SUMMARY: Our study estimates the clinical prevalence of Wilson's disease, a rare genetic disorder of copper metabolism, in the UK. The estimated clinical prevalence is this study is markedly lower than the estimated UK genetic prevalence.

Indexed as

CCG, clinical commissioning groupClinical prevalenceCopper metabolism disorderMultiple sources of case ascertainmentWD, Wilson’s diseaseWilson’s disease

Identifiers

PMID34381985
PMCPMC8335649

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