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Definition
By Mayo Clinic staffWilson's disease is an inherited disorder that causes too much copper to accumulate in your liver, brain and other vital organs. Another term for Wilson's disease is hepatolenticular degeneration.
Copper plays a key role in the development of healthy nerves, bones, collagen and the skin pigment melanin. Normally, copper is absorbed from your food, and any excess is excreted through bile — a substance produced in your liver.
But in people with Wilson's disease, copper isn't eliminated properly and instead accumulates, possibly to a life-threatening level. Left untreated, Wilson's disease is fatal. When diagnosed early, Wilson's disease is treatable, and many people with the disorder live normal lives.
- Cox DW, et al. Wilson disease. In: Feldman M, et al. Sleisenger & Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 8th ed. Philadelphia, Pa.: Saunders Elsevier; 2006. http://www.mdconsult.com/das/book/body/153549812-5/0/1389/0.html. Accessed Aug. 11, 2009.
- Roberts EA, et al. Diagnosis and treatment of Wilson disease: An update. Hepatology. 2008;47:2089.
- Wilson disease. National Institute for Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/wilson. Accessed Aug. 11, 2009.
- ATP7B. Genetics Home Reference. http://ghr.nlm.nih.gov/gene=atp7b. Accessed Aug. 11, 2009.
- Copper, Cu (mg) content of selected foods. USDA National Nutrient Database for Standard Reference, Release 21. http://www.nal.usda.gov/fnic/foodcomp/Data/SR21/nutrlist/sr21w312.pdf. Accessed Aug. 18, 2009.
- Picco MF (expert opinion). Mayo Clinic, Jacksonville, Fla. Sept. 14, 2009.
