Description

Once a person is started on therapy for Wilson's Disease it should be continued for the rest of the patient's life.


Patient selection: Wilson's disease

 

Causes of interruption:

(1) noncompliance

(2) pregnancy (may be related to concern about teratogenicity)

(3) running out of medication

(4) loss of medication

(5) concern about adverse effects

 

If therapy is interrupted, then the person is at risk for acute liver failure and/or a hemolytic crisis.

 

The risk is increased if the person also fails to restrict dietary copper intake.

 

Exception: liver transplantation

 

Clue to noncompliance: appearance or reappearance of Kayser-Fleischer rings


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