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Definition


Wilson's Disease Definition
Common names:
  • Copper Storage Disease
  • Hepatolenticular Degeneration
  • Wilson's Disease
A person with Wilson's disease has a disorder that causes the body to collect copper from the diet. Wilson disease is a rare inherited disorder that affects approximately 1 in 30,000 individuals.

In Wilson's disease, copper can accumulate within the liver, leading to liver disease. Overtime, the diseased liver releases copper into the bloodstream, which carries it to other organs in the body. This can lead to collections of copper in the brain, eyes, and kidneys. The most characteristic sign of Wilson's disease is the Kayser-Fleischer ring, a rusty, greenish-brown ring around the cornea of the eye that can be seen on eye examination.

Wilson's disease can cause:
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Last Updated: Sep 2, 2008  References
Authors: Stephen J. Schueler, MDJohn H. Beckett, MDD. Scott Gettings, MD
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