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Wilson Disease

  • Peter Ferenci
Chapter

Abstract

Wilson disease is an autosomal recessive inherited disorder of copper metabolism resulting in pathological accumulation of copper in many organs and tissues. The hallmarks of the disease are the presence of liver disease, neurological symptoms and Kayser-Fleischer corneal rings.

The incidence of Wilson disease was estimated to be at least 1:30,000–50,000 with a gene frequency of 1:90–1:150. Among selected groups of patients Wilson disease is certainly more frequent. About 3–6% of patients transplanted for fulminant hepatic failure and 16% of young adults with chronic active hepatitis of unknown origin have Wilson disease.

Keywords

Orthotopic Liver Transplant Chronic Active Hepatitis Fulminant Hepatic Failure Wilson Disease Serum Ceruloplasmin 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • Peter Ferenci
    • 1
  1. 1.Department of Medicine III, Gastroenterology and HepatologyMedical University of ViennaViennaAustria

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