WILSON’S DISEASEFernandez, Mikee B.DMD2D

Definition•Other name of Wilson’s disease: 

“Hepatolenticular Degeneration”•is an autosomal recessive genetic disorder in

which copper accumulates in tissues; this manifests as neurological or psychiatric symptoms and liver disease. •a genetic disorder that prevents the body

from getting rid of extra copper. kidneys, and eyes.

Kayser-Fleischer rings (KF rings)

•Are dark rings that appear to encircle the iris of the eye. They are due to copper deposition as a result of particular liver diseases.

• Kayser-Fleischer rings do not cause any symptoms.

Signs and Symptoms•Swelling of arms and legs•Involuntary shaking/ Tremors•Easy bruising•Nausea•Yellowing of the skin and eyes

(Jaundice)•Fluid buildup in the legs or abdomen•Behavioral changes•Anemia

Dental Correlation•The patient cannot sit still(due to

tremors) and its very dangerous during a dental procedure.•Ask for a medical clearance before

proceeding to a dental procedure.•Cannot prescribe any medicine due to

liver disease.•Cannot prescribe copper base drugs.•For dental diagnosis.

Classification•The Wilson's disease gene (ATP7B) has been mapped

to chromosome 13 (13q14.3) and is expressed primarily in the liver, kidney, and placenta.

•Mutations can be detected in 90%. Most (60%) are homozygous for ATP7B mutations (two abnormal copies), and 30% have only one abnormal copy. Ten per cent have no detectable mutation.