INFORMATION ON WILSON DISEASE

Wilson's disease is a genetic disorder that is fatal unless detected and treated before serious illness from copper poisoning develops. Wilson's disease affects approximately one in 30,000 people worldwide. The genetic defect causes excessive copper accumulation in the liver or brain. Wilson's disease causes the body to retain copper. The liver of a person who has Wilson's disease does not release copper into bile as it should. Bile is a liquid produced by the liver that helps with digestion.

SYNONYMS OF WILSON DISEASE

Synonyms and related keywords:
1. Hepatolenticular degeneration.
2. Copper metabolism.
3. Cirrhosis.
4. Fulminant hepatic failure.
5. Chronic liver disease.
6. Hepatic dysfunction.
7. Chelation therapy.

CAUSES OF WILSON DISEASE

Wilson disease is a rare inherited disorder, passed in families as a recessive trait. Wilson's disease can cause severe brain damage, liver failure, and death. A number of foods, especially liver, shellfish, nuts, avocados and mushrooms, contain abundant amounts of copper. When you eat these foods, the copper is absorbed by your small intestine, bound to circulating proteins in your blood and delivered to your liver. Any copper your body doesn't use is carried away by bile, a substance produced in your liver that helps digest fats. This manipulates copper causes the Wilson diseases. It is mainly the liver that controls levels of copper in the body by excreting excess copper mostly in the bile. This process fails in Wilson’s disease, causing copper to build up in the body. This mainly damages the liver but can also affect other organs, especially the brain.

SYMPTOMS OF WILSON DISEASE

Symptoms of Wilson Disease are:
1. Liver symptoms.
2. Hepatitis.
3. Cirrhosis of the liver or scarring.
4. Tiredness and loss of appetite.
5. Jaundice.
6. Abdominal pain.
7. Swelling of the ankles or abdomen.
8. Enlargement of the liver or spleen.
9. Bleeding from the gut or coma in severe cases.
10. Tremors or shaking.
11. Poor co-ordination.
12. Difficulty with speech, eating or walking.
13. Spontaneous movement.
14. Psychiatric symptoms.
15. Anemia.
16. Kidney damage.

DIAGNOSIS OF WILSON DISEASE

Diagnosis of Wilson Disease Includes:

Eye Examination for:

1. Kayser-Fleischer rings.
2. Eye movement may be restricted.

Physical examination for

1. Liver or spleen disorders.
2. Damage to the central nervous system.

Lab Test For.

1. Finding percentage of Serum ceruloplasmin.
2. Finding percentage of Urine copper.
3. Show hemolytic anemia or decreased white blood cell count.

Other tests:

1. Head MRI.
2. Head CT scan.
3. X-ray.

TREATMENT OF WILSON DISEASE

The main aim behind the treatment is to reduce the amount of copper in the tissues and to manage the symptoms of the disorder. A low-copper diet should be used, including avoiding mushrooms, nuts, chocolate, dried fruit, liver, and shellfish. Distilled water may be suggested because most tap water flows through copper pipes. Avoid using copper cooking utensils. Symptoms are treated as appropriate, including exercises or physical therapy, and protective measures for people who are confused or unable to care for themselves.

Some of these medicines can be used if needed.
1. Zinc acetate.
2. Trientine.
3. Penicillamine.