Wilson's disease

Wilson’s disease, also known as hepatolenticular degeneration, is an inherited defect of copper metabolism. Copper is an essential element involved in the function of a number of enzymes. Usually excess copper in the diet is excreted in the bile. In Wilson's disease this mechanism fails and copper is deposited in the liver cells. Once the liver stores are full it is deposited in other cells, particularly brain cells where it manifests itself by producing symptoms either of a psychiatric nature or by causing abnormalities of gait or movement. Treatment aimed at increasing copper excretion is effective provided it is started early enough.
 
 
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