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Wilson's Disease

Making a Diagnosis

Wilson's disease is diagnosed through tests that measure the amount of copper in the blood, urine, and liver. An eye exam would detect the Kayser-Fleischer ring.
 

Treatment for Wilson's Disease

Treatment for Wilson's disease generally consists of anti-copper agents to remove excess copper from the body and to prevent it from reaccumulating. Most patients with Wilson's disease are treated with the following drugs:
 
  • Zinc acetate
  • Trientine
  • Penicillamine.
     
Zinc acetate blocks the absorption of copper and increases copper excretion in the stool. Because it has no serious side effects, it is often considered the treatment of choice for Wilson's disease.
 
Trientine and penicillamine increase urinary excretion of copper; however, both drugs can cause serious side effects in people taking it for Wilson's disease.
 
Tetrathiomolybdate, an experimental drug, also shows promise in treating Wilson's disease.
 
In rare cases in which there is severe liver disease, a liver transplant may be needed.
 
Patients with Wilson's disease will also need to take vitamin B6 and follow a low-copper diet, which means avoiding mushrooms, nuts, chocolate, dried fruit, liver, and shellfish.
 

Prognosis for Wilson's Disease

Wilson's disease requires lifelong treatment. If Wilson's disease is detected early and treated correctly, a person can enjoy completely normal health.
 
Without proper treatment, Wilson's disease is generally fatal, usually by the age of 30.
 
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