Categories

A sample text widget

Etiam pulvinar consectetur dolor sed malesuada. Ut convallis euismod dolor nec pretium. Nunc ut tristique massa.

Nam sodales mi vitae dolor ullamcorper et vulputate enim accumsan. Morbi orci magna, tincidunt vitae molestie nec, molestie at mi. Nulla nulla lorem, suscipit in posuere in, interdum non magna.

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.

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

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.

Risks
– 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

Leave a Reply

You can use these HTML tags

<a href="" title=""> <abbr title=""> <acronym title=""> <b> <blockquote cite=""> <cite> <code> <del datetime=""> <em> <i> <q cite=""> <s> <strike> <strong>