Alternate Names : Hepatolenticular Degeneration
Wilson's disease is an inherited inborn error of
metabolism in which the body cannot process copper.
What is going on in the body?
Wilson's disease is caused by a defect in an enzyme, or special protein. This
enzyme normally helps the body get rid of extra copper. The amount of copper in
a normal diet is more than the body needs. In people with Wilson's disease, the
extra copper cannot be excreted from the body. The copper can damage the liver,
brain, kidney, cornea, and other organs.
What are the causes and risks of the disease?
Wilson's disease is an inherited disorder. The children of parents who carry
the gene for the disorder are at risk. The liver disease usually starts between
ages 8 and 12, while the brain symptoms occur later in life.