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Wilson's Disease

Wilson's disease is a rare inherited disorder marked by the body's inability to metabolize copper, an essential trace mineral that comes from such food sources as liver, shellfish and dried beans. It affects 1 in 30,000 with a carrier frequency of 1 in 90. The cause is unknown.

Unable to process copper, the body stores it in the liver until it reaches abnormally high levels. With age and capacity, the liver releases the mineral into the body, where it accumulates in the central nervous system, kidneys and skeleton, among other organs.

Although children may experience clinical manifestations before age five, the condition generally presents thereafter. It is most often diagnosed in early adulthood, where patients present with clinical symptoms that mimic acute or chronic hepatitis, cirrhosis or liver failure. Some patients complain of abdominal pain and fatigue. It should be assumed that children and young adults who experience liver failure have Wilson's disease unless proven otherwise. Treatment includes pencillamine and a reduced copper diet.