Treating Other Symptoms of Wilson Disease: The Liver

  • Michelle Camarata
  • Michael L. SchilskyEmail author
Part of the Clinical Gastroenterology book series (CG)


Wilson disease results in the pathological accumulation of copper, particularly in the liver where it causes cellular damage. Features of liver disease can be extremely variable. Patients may be asymptomatic, particularly when found by family screening or routine laboratory testing while others can have features of jaundice from an inflamed liver or more severe symptoms resulting from end-stage liver disease with cirrhosis. In patients with cirrhosis, complications related to portal hypertension can be life-threatening, and prompt initiation of treatment is essential. Liver cancer, although rare in Wilson disease patients, can occur when cirrhosis and chronic inflammation are present. Screening for liver cancer in those at risk may improve detection at an early stage. Timely initiation of the appropriate therapy for Wilson disease is necessary to prevent disease progression. In those without cirrhosis at the time of diagnosis, the prognosis is excellent. A minority of patients present with acute liver failure. In this situation transplantation is lifesaving.


Wilson disease Fibrosis Portal hypertension Cirrhosis Jaundice Varices Ascites Spontaneous bacterial peritonitis TIPS Sepsis Hepatic encephalopathy Hepatocellular carcinoma Cholangiocarcinoma 


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Gastroenterology and HepatologyRoyal Surrey County HospitalGuildford, SurreyUK
  2. 2.Department of Clinical and Experimental MedicineUniversity of SurreyGuildford, SurreyUK
  3. 3.Department of Surgery, Section of Transplant and ImmunologyYale School of MedicineNew HavenUSA
  4. 4.Division of Digestive Diseases and Transplant and Immunology, Departments of Medicine and SurgeryYale UniversityNew HavenUSA

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