Wilson Disease

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Definition & Facts

Wilson disease is a genetic disorder that prevents the body from removing extra copper, causing copper to build up in the liver, brain, eyes, and other organs. Without treatment, high copper levels can cause life-threatening organ damage.

Image of an eye with a Kayser-Fleischer ring

Symptoms & Causes

The symptoms of Wilson disease vary. The symptoms may be related to your liver, nervous system and mental health, eyes, or other organs. Gene mutations cause Wilson disease.

Diagnosis

Doctors diagnose Wilson disease based on your medical and family history, a physical exam, an eye exam, blood tests, and urine tests. Doctors may also use a liver biopsy and imaging tests.

Treatment

Doctors treat Wilson disease with chelating agents and zinc. People who have Wilson disease need lifelong treatment to manage symptoms and reduce or prevent organ damage. If Wilson disease causes acute liver failure or cirrhosis with liver failure, you may need a liver transplant.

Eating, Diet, & Nutrition

If you have Wilson disease, talk with your doctor about your diet and foods that contain copper. When you start treatment for Wilson disease, your doctor may recommend avoiding foods that are high in copper, such as chocolate, liver, mushrooms, nuts, and shellfish.

Clinical Trials

The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and other components of the National Institutes of Health (NIH) conduct and support research into many diseases and conditions.

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Last Reviewed October 2018

This content is provided as a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the National Institutes of Health. NIDDK translates and disseminates research findings to increase knowledge and understanding about health and disease among patients, health professionals, and the public. Content produced by NIDDK is carefully reviewed by NIDDK scientists and other experts.

The NIDDK would like to thank:
Valentina Medici, M.D., University of California, Davis