•Wilson disease (WD) is an
inherited disease of copper metabolism characterized by cirrhosis and
degenerative central nervous system disorder first described an American
neurologist Samuel Alexander Kinnier Wilson in 1912
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•WD is inherited as an
autosomal recessive disorder linked to a locus on the long arm of chromosome 13.
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•The
condition is characterized by excessive deposition of copper in the liver,
brain, and other tissues. The major physiologic aberration is excessive
absorption of copper from the small intestine and decreased excretion of
copper by the liver.
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