Wilson’s Disease: Pathophysiology and Therapy

  • S. D. Zucker
  • J. L. Gollan


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.


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

Authors and Affiliations

  • S. D. Zucker
  • J. L. Gollan

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