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What are causes of Wilson’s disease?

Wilson’s disease is also known medically as hepatolenticular degeneration which is an autosomal recessive genetic disorder. In this disorder copper accumulates in tissues.

Overview
– This manifests as neurological or psychiatric symptoms and liver disease.
– It is treated with medication that reduces copper absorption
– Medication that removes the excess copper from the body also can be used.
– Occasionally a liver transplant is required for this disorder.
– The condition is due to mutations in the Wilson disease protein (ATP7B) gene.
– A single abnormal copy of the gene is present in 1 in 100 people.
– If a child inherits the gene from both parents, the child may develop Wilson’s disease.
– Symptoms usually appear between the ages of 6 and 20 years.
– Wilson’s disease occurs in 1 to 4 per 100,000 people.
– Wilson’s disease is named after Samuel Alexander Kinnier Wilson (1878–1937) who was the British neurologist who first described this condition in 1912.
– This condition is most common in eastern Europeans, Sicilians, and southern Italians.
– A small amount of copper obtained from food is needed to stay healthy, but excess copper is poisonous.
– High copper levels can cause life-threatening organ damage.

In Wilson disease, copper builds up in:
– the liver
– brain
– kidneys
– eyes
– other organs

Who gets Wilson disease?
– Patients who inherit two abnormal copies of the ATP7B gene, one from each parent.
– Who have only one copy of the abnormal gene, do not have symptoms.
– Most people with Wilson disease have no known family history of the disease.
– A person’s chances of having Wilson disease increase if one or both parents have it.
– It equally affects men and women.
– Symptoms usually appear between ages 5 to 35.
– New cases have been reported in people aged 2 to 72 years.

What causes Wilson disease?
– Wilson disease is caused by a buildup of copper in the body.
– Normally, copper from the diet is filtered out by the liver.
– It is then released into bile.
– This then flows out of the body through the gastrointestinal tract.
– People who have Wilson disease cannot release copper from the liver at a normal rate.
– This is due to a mutation of the ATP7B gene.
– When the copper storage capacity of the liver is exceeded, copper is released into the bloodstream.
– It travels to other organs—including the brain, kidneys, and eyes.

The deposits of copper causes:
– tissue damage.
– death of the tissues.
– scarring of the tissue.

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