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Diseases of the Liver and Gallbladder01:26

Diseases of the Liver and Gallbladder

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Wilson disease: symptomatic liver therapy.

Jan Pfeiffenberger1, Karl-Heinz Weiss1, Wolfgang Stremmel1

  • 1Department of Gastroenterology and Hepatology, University Hospital of Heidelberg, Heidelberg, Germany.

Handbook of Clinical Neurology
|April 24, 2017
PubMed
Summary

Wilson disease can cause liver cirrhosis, requiring lifelong decoppering therapy and monitoring for complications like portal hypertension and hepatic encephalopathy. Liver transplantation is an option for advanced disease.

Keywords:
Wilson diseaseacute liver failureascitesesophageal variceshepatic encephalopathyliver cirrhosis

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Area of Science:

  • Hepatology
  • Internal Medicine
  • Genetics

Background:

  • Wilson disease is a genetic disorder causing copper accumulation, leading to liver damage and cirrhosis.
  • Symptomatic Wilson disease necessitates strict adherence to decoppering agents.
  • Secondary prevention strategies are crucial to prevent further hepatic damage.

Purpose of the Study:

  • To outline the management of liver complications in Wilson disease.
  • To detail screening protocols for cirrhotic Wilson disease patients.
  • To discuss treatment options for Wilson disease-related liver decompensation.

Main Methods:

  • Review of current medical literature and clinical guidelines.
  • Emphasis on routine examinations for portal hypertension, ascites, and hepatic encephalopathy.
  • Discussion of therapeutic interventions including pharmacotherapy, procedural treatments, and liver transplantation.

Main Results:

  • Decoppering therapy is essential for Wilson disease patients.
  • Screening for portal hypertension, ascites, and hepatic encephalopathy is vital in cirrhotic patients.
  • Management strategies include beta-blockers, diuretics, antibiotics, laxatives, and potentially liver transplantation.

Conclusions:

  • Wilson disease management requires lifelong decoppering therapy and vigilant monitoring of liver function.
  • Complications of cirrhosis in Wilson disease, such as portal hypertension and hepatic encephalopathy, require specific treatments.
  • Liver transplantation is a critical option for patients with decompensated liver cirrhosis due to Wilson disease.