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Wilson’s Disease: Pathophysiology and Therapy

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Hepatobiliary Diseases

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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.

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Authors
  1. S. D. Zucker
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  2. J. L. Gollan
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Editors and Affiliations

  1. Department of Medicine and Liver Unit, Clinica Universitaria and School of Medicine University of Navarra, Pamplona, Spain

    Jesús Prieto M.D. (Professor of Medicine) (Professor of Medicine)

  2. Liver Unit, Hospital Clinic i Provincial, University of Barcelona, Barcelona, Spain

    Joan Rodés M.D. (Professor of Medicine) (Professor of Medicine)

  3. Liver Research Center, Albert Einstein College of Medicine, Bronx, NY, USA

    David A. Shafritz M.D. (Professor of Medicine and Cell Biology and Director Marion Bessin) (Professor of Medicine and Cell Biology and Director Marion Bessin)

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© 1992 Springer-Verlag Berlin Heidelberg

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Zucker, S.D., Gollan, J.L. (1992). Wilson’s Disease: Pathophysiology and Therapy. In: Prieto, J., Rodés, J., Shafritz, D.A. (eds) Hepatobiliary Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-76802-6_23

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  • DOI: https://doi.org/10.1007/978-3-642-76802-6_23

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