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Wilson’s Disease: Pathophysiology and Therapy

  • S. D. Zucker
  • J. L. Gollan

Abstract

Wilson’s disease, or hepatolenticular degeneration, is an inherited disorder of copper metabolism, characterized by decreased biliary excretion of the metal. Copper accumulation and deposition in a variety of organ systems results in progressive tissue injury and leads inexorably to death unless appropriate therapy is initiated. Kinnear Wilson first described the familial syndrome of “progressive lenticular degeneration associated with cirrhosis of the liver” in 1912. This chapter will attempt to review the current understanding of Wilson’s disease, highlighting some of he recent advances and controversies in the pathogenesis and management of this disorder.

Keywords

Primary Biliary Cirrhosis Chronic Active Hepatitis Fulminant Hepatic Failure Serum Ceruloplasmin Hepatic Copper 
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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© Springer-Verlag Berlin Heidelberg 1992

Authors and Affiliations

  • S. D. Zucker
  • J. L. Gollan

There are no affiliations available

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