Wilson’s Disease

Wilson’s disease is an inherited autosomal-recessive disease typically associated with increased intestinal uptake of copper and subsequent deposition in the liver, basal ganglia and other organs. There may be an acute or even fulminant hepatitis, chronic inflammation or cirrhosis. In contrast to hemochromatosis, these patients do not demonstrate an increased risk of developing HCC. Wilson’s disease should be considered when a low level of coeruloplasmin (less than 1.3 mmol/l) and an increased quantity of copper is present in the liver (greater than 250 mg/g dry weight) [159].
Clinical symptoms of patients suffering from Wilson’s disease seem to be directly related to the accumulation of copper in the brain, cornea, liver and kidneys. Liver cirrhosis induced by Wilson’s disease is normally macronodular. However, a mixed type or a micronodular type can also be observed.
Histology reveals nodules of variable size separated by fibrous septa with minimal cholangiocellular proliferation and varying signs of inflammation. However, the distribution of copper deposition does not correlate with the pattern of nodules The current treatment of choice is D-Penicillamin, which chelates unbound copper for urinary excretion. However, liver transplantation can also be considered as an ultimate therapeutic option.

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