INFORMATION ON WILSON DISEASE

Wilson's disease is a condition where too much copper builds up in the body. It is a rare inherited disorder that affects about 1 in 30,000 people. It is four times more common in females than in males. Copper is normally metabolized by being incorporated into copper-containing enzymes called ceruloplasmin and being excreted into bile. People with Wilson's disease cannot get rid of excess copper and so it builds up in the body, mainly in the liver, the brain, the cornea and kidneys. The copper deposits cause tissue damage, tissue death, and scarring, which cause the affected organs to stop functioning properly.

SYNONYMS OF WILSON DISEASE

Synonyms and related keywords
1. Wilson disease
2. Hepatolenticular degeneration
3. Ceruloplasmin, copper accumulation
4. Disorder of copper metabolism
5. Hepatic disease
6. Kayser-Fleischer rings
7. Neurologic disorder, cirrhosis

CAUSES OF WILSON DISEASE

Wilson's disease is caused by having too much copper in the bodyand it is a inheritance diseases where each parent carries a gene for the disease on one of his or her chromosome pairs. In Wilson's disease this process fails, causing copper to build up in the body. This mainly damages the liver but can also affect other organs, especially the brain.
It is mainly the liver that controls levels of copper in the body by excreting excess copper mostly in the bile. This copper can cause damage in the kidneys, brain and eyes. If not treated, Wilson's disease can cause liver failure, brain damage and even death
The liver is most commonly affected by the disease followed by the brain.

SYMPTOMS OF WILSON DISEASE
Neurological symptoms:

Symptoms include tremors of the hands, uncontrollable movements of the limbs, stiffness, drooling, difficulty swallowing, difficulty talking, and headache.

Psychiatric symptoms:

Symptoms include personality changes, mood disturbances, depression, anxiety or phobias, or just poor memory.

Rare symptoms include:

Anemia, if the blood is affected.
Kidney damage, shown by blood and protein in the urine.

DIAGNOSIS OF WILSON DISEASE

The diagnosis of Wilson's disease can usually be made by a combination of blood tests, urine tests, eye test and liver biopsy. A decreased copper binding protein in blood, called serum ceruloplasmin, is found in 95% of patients with Wilson’s disease.

TREATMENT OF WILSON DISEASE

Treatment of Wilson's disease generally consists of anti-copper agents to remove excess copper from the body and to prevent it from reaccumulating.
It is important to screen all members of a family once a case is diagnosed in the family. Children of a person with Wilson's disease have a 1 in 200 chance of having the disease.
Most cases are treated with the drugs zinc acetate, trientine, or penicillamine. Penicillamine and trientine increase urinary excretion of copper.
In rare cases in which there is severe liver disease, a liver transplant may be needed.
The liver is usually the first part of the body affected, with initial physical changes only visible through a microscope. These cases occur predominantly in children and may result in hepatitis.