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Wilson Disease
URL of this page: https://medlineplus.gov/wilsondisease.html

Wilson Disease

Also called: Copper storage disease, Hepatolenticular degeneration
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Summary

Wilson disease is a rare inherited disorder that prevents your body from getting rid of extra copper. You need a small amount of copper from food to stay healthy. Too much copper is poisonous.

Normally, your liver releases extra copper into bile, a digestive fluid. With Wilson disease, the copper builds up in your liver, and it releases the copper directly into your bloodstream. This can cause damage to your brain, kidneys, and eyes.

Wilson disease is present at birth, but symptoms usually start between ages 5 and 35. It first attacks the liver, the central nervous system or both. The most characteristic sign is a rusty brown ring around the cornea of the eye. A physical exam and laboratory tests can diagnose it.

Treatment is with drugs to remove the extra copper from your body. You need to take medicine and follow a low-copper diet for the rest of your life. Don't eat shellfish or liver, as these foods may contain high levels of copper. At the beginning of treatment, you'll also need to avoid chocolate, mushrooms, and nuts. Have your drinking water checked for copper content and don't take multivitamins that contain copper.

With early detection and proper treatment, you can enjoy good health.

NIH: National Institute of Diabetes and Digestive and Kidney Diseases

Start Here

  • Wilson Disease From the National Institutes of Health (National Institute of Diabetes and Digestive and Kidney Diseases)
  • Wilson Disease: Frequently Asked Questions (Wilson Disease Association)
  • Wilson's Disease (Mayo Foundation for Medical Education and Research) Also in Spanish

Diagnosis and Tests

  • Ceruloplasmin Test From the National Institutes of Health (National Library of Medicine) Also in Spanish
  • How Is Wilson Disease Diagnosed? (Wilson Disease Association)
  • Kayser-Fleischer Rings (Wilson Disease Association)

Living With

  • Diet and Nutrition: Copper Conscious Eating (Wilson Disease Association)
  • Monitoring Your Treatment (Wilson Disease Association)

Genetics

  • How Is Wilson Disease Inherited? (Wilson Disease Association)
  • Wilson disease: MedlinePlus Genetics From the National Institutes of Health (National Library of Medicine)

Clinical Trials

  • ClinicalTrials.gov: Hepatolenticular Degeneration From the National Institutes of Health (National Institutes of Health)

Journal Articles References and abstracts from MEDLINE/PubMed (National Library of Medicine)

  • Article: Assessment of neurological symptoms and associated factors in patients with Wilson's...
  • Article: Epidemiologic features and economic burden of Wilson's disease in South Korea:...
  • Article: CLSI Validation of Exchangeable Copper Determination in Serum by ICP-MS: A...
  • Wilson Disease -- see more articles

Find an Expert

  • National Institute of Diabetes and Digestive and Kidney Diseases From the National Institutes of Health
  • National Institute of Neurological Disorders and Stroke From the National Institutes of Health

Patient Handouts

  • 24-hour urine copper test (Medical Encyclopedia) Also in Spanish
  • Ceruloplasmin blood test (Medical Encyclopedia) Also in Spanish
  • Wilson disease (Medical Encyclopedia) Also in Spanish

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Wilson Disease

MEDICAL ENCYCLOPEDIA

  • 24-hour urine copper test
  • Ceruloplasmin blood test
  • Wilson disease

National Institutes of Health

The primary NIH organization for research on Wilson Disease is the National Institute of Diabetes and Digestive and Kidney Diseases

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The information on this site should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.

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