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Wilson's disease
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Wilson's disease

Rare inherited defect of copper metabolism. If untreated it leads to liver failure and brain damage. It is managed by daily use of penicillamine, a drug known as a chelating agent, to aid excretion of copper.



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These include: * Hemochromatosis, the abnormal accumulation of iron in the liver * Wilson disease, the abnormal accumulation of copper in the liver * Alpha 1-antitrypsin deficiency, the absence of a specific enzyme in the liver * Glycogen storage diseases, which prevent the body from properly using sugars * Autoimmune hepatitis, an abnormality of the body's immune system that leads to inflammation of the liver What are the symptoms and complications of cirrhosis?
The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene.
Rosenfield N, Grand RJ, Watkins JB, et al: Cholelithiasis and Wilson disease.
 
 
 
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