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 Wilsons 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.