2. 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.
3. 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.
4. 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
5. 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.
6. 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.
