Organ system: The digestive system
Disease class: Liver diseases
Also known as hepatolenticular degeneration.
This is a familial disease in which there is an increased accumulation of copper in the liver, brain, and other tissues including the kidneys.
Its main manifestation is the development of tremor and rigidity, with difficulty in speech.
In many cases there is improvement following treatment with dimercaprol, penicillamine, or trientine dihydrochloride; these substances cause an increased excretion of copper.
Signs
- A brown ring on the edge of the cornea (Kayser–Fleischer ring)
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