28 Wilson's Disease
28 Wilson's Disease
28 Wilson's Disease
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Pathophysiology<br />
• Mutation in gene ATP7A on Chromosome 13<br />
• Decreased incorporation of copper into<br />
apoceruloplasm<br />
– Decreased formation and secretion of ceruloplasm<br />
• Decreased copper excretion in bile<br />
– Loss of primary excretory mechanism<br />
• Both increased liver concentrations of Cu<br />
– Cu forms free radicals and injures hepatocytes
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