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Wilson's Disease (Clinical Presentation (Reduced memory, Liver disease…
Wilson's Disease
Clinical Presentation
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Liver disease varies from episodes of acute hepatitis, especially in children, to chronic hepatitis to cirrhosis
Young adults tend to present with more CNS problems e.g. tremor, dysarthria (slurred or slowed speech), involuntary movements, dysphagia, dyskinesia, and eventual dementia
Kayser-Fleischer ring - caused by copper deposition in cornea, results in a greenish-brown pigment at the corneoscleral junction
Children usually present with hepatic problems e.g. hepatitis, cirrhosis and fulminant liver failure
Diagnosis
Liver biopsy shows increased hepatic copper, hepatitis and cirrhosis - but note high copper levels are also found in chronic cholestasis
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Treatment
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Avoid foods that are high in copper e.g. liver, chocolate, nuts, mushrooms & shellfish
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Pathophysiology
A very rare inborn error of copper metabolism that results in copper deposition in various organs, including the liver, the basal ganglia of the brain and the cornea
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