Wilson's disease
an inborn defect of copper metabolism in which there is a deficiency of CAERULOPLASMIN (which normally forms a nontoxic complex with copper). The free copper may be deposited in the liver, causing jaundice and cirrhosis, or in the brain, causing mental retardation and symptoms resembling PARKINSONISM . There is a characteristic brown ring in the cornea (the Kayser-Fleischer ring). If the excess copper is removed from the body by regular treatment with PENICILLAMINE both mental and physical development may be normal.
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
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