Wilson's disease


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Definition

By Mayo Clinic staff

Wilson'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.

Symptoms
References
  1. Cox DW, et al. Wilson disease. In: Feldman M, et al. Sleisenger & Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2010. http://www.mdconsult.com/books/page.do?eid=4-u1.0-B978-1-4160-6189-2..00075-5&isbn=978-1-4160-6189-2&uniqId=270505025-3#4-u1.0-B978-1-4160-6189-2..00075-5. Accessed Aug. 2, 2011.
  2. Ferri FF. Wilson's disease. In: Ferri FF. Ferri's Clinical Advisor 2011: Instant Diagnosis and Treatment. Philadelphia, Pa.: Mosby Elsevier; 2011. http://www.mdconsult.com/books/about.do?eid=4-u1.0-B978-0-323-05610-6..C2009-0-38600-6--TOP&isbn=978-0-323-05610-6&about=true&uniqId=230100505-53. Accessed Aug. 2, 2011.
  3. Huster D. Wilson disease. Best Practice & Research Clinical Gastroenterology. 2010;24:531.
  4. Wilson disease. National Institute for Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/wilson/. Accessed Aug. 2, 2011.
  5. Kaplan MM. Treatment of Wilson disease. http://www.uptodate.com/home/index.html. Accessed Aug. 2, 2011.
  6. Kaplan MM. Diagnosis of Wilson disease. http://www.uptodate.com/home/index.html. Accessed Aug. 2, 2011.
  7. Picco MF (expert opinion). Mayo Clinic, Rochester, Minn. Aug. 11, 2011.
DS00411 Sept. 23, 2011

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