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Review
Wilson disease in children and adolescents
  1. Meranthi Fernando1,
  2. Indra van Mourik1,
  3. Evangeline Wassmer2,
  4. Deirdre Kelly1
  1. 1 Liver Unit, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, West Midlands, UK
  2. 2 Department of Neurology, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, West Midlands, UK
  1. Correspondence to Dr Indra van Mourik, Liver Unit, Birmingham Women's and Children's NHS Foundation Trust, Birmingham B4 6NH, UK; indra.mourik{at}nhs.net

Abstract

Wilson disease (WD) is a rare, recessively inherited disorder of copper metabolism mainly affecting liver and brain. In childhood, it is known to have a predominant hepatic phenotype. It is likely that the low awareness for WD-associated neuropsychiatric signs and symptoms in this age group means that neurological Wilson’s disease is underdiagnosed in children and young people. Practitioners should be alert for this complication in children with or without liver disease. Management of children with WD requires a dedicated multidisciplinary approach involving hepatologists, geneticists, neurologists and psychiatrists to ensure subtle neuropsychiatric symptoms are identified early and addressed appropriately. This review highlights recent advances in hepatic and neuropsychiatric symptoms of WD in childhood, specific diagnostic tools and pitfalls and summarises existing and potential future treatment options.

  • hepatology
  • neurology
  • paediatric practice

https://doi.org/10.1136/archdischild-2018-315705

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    Footnotes

    • Contributors IvM, MF and DK planned, researched and wrote the main body of the article. EW planned, researched and wrote the neurology section of the article. IvM and DK edited and finalised the various drafts to produce the final manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Not required.

    • Provenance and peer review Commissioned; externally peer reviewed.

    • Data availability statement Data sharing not applicable as no datasets generated and/or analysed for this study. No data are available.