Drugs - Dyskenesia - Wilsons Disease Flashcards
What is Wilson’s disease?
An inherited disorder of copper accumulation
What causes Wilson’s disease?
A genetic defect in the ATPase that allows copper to be excreted into the bile
What are the manifestations of Wilson’s disease?
- Dysarthria (speech disorder) and movement disorders due to copper affecting the basal ganglia
- chronic active hepatitis leading to cirrhosis
- psychiatric disturbances
What drugs are used to treat Wilson’s disease?
- Penicillamine
- Triantene
What does Penicillamine do?
A chelating agent that forms water-soluble complexes with copper and promotes its excretion in urine
What does trientine do?
It is another chelating agent that has fewer adverse effects than penicillamine
What does zinc acetate do in Wilson’s disease?
PO increases the fecal excretion of copper
What does zinc sulfate do in Wilson’s disease?
Decreases copper absorption
If schizophrenia shows up all of a sudden, what should be suspected?
Wilson’s disease
Which is a biological marker of Wilson’s disease?
A deficiency of serum ceruloplasmin
Is absorption of copper impaired in Wilson’s disease?
No, only excretion
What type of genetic disease is Wilson’s disease?
Autosomal recessive
What is Wilson’s disease sometimes mistaken for because of the choreiform movements, abnormal facial expressions, tremor, and drooling?
Schizophrenia
Why don’t we use penicillamine anymore for Wilson’s disease?
It has a bad side effect profile (treatment should be monitored with frequent urinalysis and complete blood counts)
