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WILSON’S DISEASE
Fernandez, 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.
6. fernandez   wilson's disease

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6. fernandez wilson's disease

  • 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.