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Wilson's disease |
An inherited metabolic disorder characterized by excessive amounts of copper in the liver, brain, kidneys, and corneas. It can lead to tissue necrosis and fibrosis, which in turn can cause hepatic disease and neurologic changes. Without treatment, it leads to fatal hepatic failure.
CAUSES: •genetic
TREATMENT
•avoid copper-rich foods •penicillamine, potassium sulfide, pyridoxine, zinc acetate as alternative to penicillamine
SYNONYMS: •progressive lenticular degeneration •Westphal-Struempell pseudosclerosis |
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