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All about Wilson's disease causes of Wilson's disease symptoms of Wilson's disease diagnosis of Wilson's disease treatment for Wilson's disease

What causes Wilson's disease?

Wilson's disease is a rare inherited disorder, passed in families as a recessive trait (meaning that an individual must receive a defective gene from both his or her mother and father in order to actually have symptoms of the disease). The

gene defect is believed to result in an abnormal protein within liver cells that binds to copper, retaining it in the liver. Patients with Wilson's disease also frequently have decreased amounts of a protein, called ceruloplasmin, that carries copper through the bloodstream. If both parents carry an abnormal gene for Wilson's disease, there is a 25% chance that each of their children will develop the disorder (i.e., it is an autosomal recessive disease).

Wilson's disease causes the body to absorb and retain excessive amounts of copper. The copper deposits in the liver, brain, kidneys, and the eyes. The deposits of copper cause tissue damage, death of the tissues, and scarring, which causes the affected organs to stop functioning properly. Liver failure and damage to the central nervous system (brain, spinal cord) are the most predominant, and the most dangerous, effects of the disorder.

It is most common in eastern Europeans, Sicilians, and southern Italians, but may occur in any group. The disorder most commonly appears in people under 40 years old. In children, the symptoms begin to be expressed by around 4 years old.


More information on Wilson's disease

What is Wilson's disease? - Wilson's disease is an inherited disorder where there is excessive amounts of copper in the body leading to dementia and muscle control loss.
What causes Wilson's disease? - Wilson's disease causes the body to absorb and retain excessive amounts of copper. The deposits of copper cause tissue damage, death of the tissues, and scarring.
What're the symptoms of Wilson's disease? - Symptoms of Wilson's disease are dementia, drooling, slurred speech, temper outbursts and violent tremors.
How is Wilson's disease diagnosed? - Diagnosis of Wilson's disease begins with a medical history and physical exam. Other tests are also useful.
What's the treatment for Wilson's disease? - The goals of treatment of Wilson's disease are to reduce the amount of copper in the tissues and to manage the symptoms of the disorder.
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All information is intended for reference only. Please consult your physician for accurate medical advices and treatment. Copyright 2005,, all rights reserved. Last update: July 18, 2005