Wilsons Disease

...less medical jargon in a 'Quick Glance' format!

Wilsons Disease causes the body to retain copper. The liver of a person who has Wilson disease does not release copper into bile as it should. Bile is a liquid produced by the liver that helps with digestion. As the intestines absorb copper from food, the copper builds up in the liver and injures liver tissue. Eventually, the damage causes the liver to release the copper directly into the bloodstream, which carries the copper throughout the body. The copper buildup leads to damage in the kidneys, brain, and eyes. If not treated, Wilson disease can cause severe brain damage, liver failure, and death.

Symptoms:

• Enlargement of the abdomen
• Splenomegaly
• Yellow skin (jaundice) or yellow color of the white of the eye
• Vomiting blood
• Weakness
• Tremors of the arms or hands
• Difficulty moving arms and legs, stiffness
• Abnormal arms and legs posture
• Slow movements
• Difficulty walking
• Unpredictable and jerky movement
• Uncontrollable movement
• Weakness of the head, neck, face, or arms
• Speech impairment
• Slow or decreased facial movement and expressions
• Emotional or behavioral changes
• Confusion or delirium
• Dementia

Causes:
Wilson's disease is a rare inherited disorder. If both parents carry an abnormal gene for Wilson's disease, there is a 25% chance in each pregnancy that the child will have the disorder.

Wilsons disease causes the body to take in and keep too much copper. The copper deposits in the liver, brain, kidneys, and the eyes. The deposits of copper cause tissue damage, death of the tissues, and scarring, which causes the affected organs to stop working correctly. Liver failure and damage to the central nervous system are the most predominant, and the most dangerous, effects of the disorder. If not caught and treated early, Wilson's disease is fatal.

It is most common in eastern Europeans, Sicilians, and southern Italians, but may occur in any group. The disorder most commonly appears in people under 40 years old. In children, the symptoms begin to show by around 4 years of age.

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Treatment:
Wilsons disease requires lifelong treatment, generally using drugs to remove excess copper from the body and to prevent it from re-accumulating. Zinc salt, which blocks the absorption of copper in the stomach and causes no serious side effects, is often considered the treatment of choice. Penicillamine and trientine increase urinary excretion of copper; however, both drugs can cause serious side effects. Tetrathiomolybdate is an investigational drug with a lower toxic profile, but it has not been approved by the Food and Drug Administration for the treatment of WD and its long-term safety and effectiveness aren’t known. A low-copper diet may also be recommended, which involves avoiding mushrooms, nuts, chocolate, dried fruit, liver, and shellfish. In rare cases where there is severe liver disease, a liver transplant may be needed. Symptomatic treatment for symptoms of muscle spasm, stiffness, and tremor may include anticholinergics, tizanidine, baclofen, levodopa, or clonazepam.

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...less medical jargon in a 'Quick Glance' format!