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How is Wilson’s disease treated?

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

– 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

Treatment of Wilson Disease
– The goal of treatment is to reduce the amount of copper in the tissues.
– This is done by a procedure called chelation.
– This process is where certain medications can bind to copper and help remove it through the kidneys or gut.
– Treatment must be done for lifelong.

The following medications may be used:
– Penicillamine (Cuprimine, Depen) binds to copper and leads to increased release of copper in the urine.
– Trientine (Syprine) binds (chelates) the copper and increases its release through the urine.
– Zinc acetate (Galzin) blocks copper from being absorbed in the intestinal tract.
Vitamin E supplements may also be used.

– Medications that chelate copper (especially penicillamine) can affect the function of the brain and nervous system (neurological function).
– Other medications under investigation may bind copper without affecting neurological function.
– Lifelong treatment is needed to control Wilson’s disease.
– The disorder may cause fatal effects.
– Loss of liver function and toxic effects of copper on the nervous system are some of the side effects.
– In cases where the disorder is not fatal, symptoms may be disabling.

Dietary Recommendations
A low-copper diet may also be recommended.
Foods to avoid include:
– Chocolate
– Dried fruit
– Liver
– Mushrooms
– Nuts
– Shellfish

Other things to care about:
– You may want to drink distilled water.
– Most tap water flows through copper pipes.
– Avoid using copper cooking utensils.
– Symptoms may be treated with exercise or physical therapy.
– People who are confused or unable to care for themselves may need special protective measures.
– A liver transplant may be considered in cases.
– This is done where the liver is severely damaged by the disease.

Possible Complications:
– Anemia (hemolytic anemia is rare)
– Central nervous system complications
– Cirrhosis
– Death of liver tissues
– Fatty liver
– Hepatitis
– Increased number of bone fractures
– Increased number of infections
– Injury caused by falls
– Jaundice
– Joint contractures or other deformity
– Loss of ability to care for self
– Loss of ability to function at work and home
– Loss of ability to interact with other people
– Loss of muscle mass (muscle atrophy)
– Psychological complications
– Side effects of penicillamine and other medications used to treat the disorder.
– Spleen problems

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