Wilson’s Disease

Question 1

What is Wilson’s disease?

Answer 1

Impaired binding of Copper to ceruloplasmin in the liver and impaired biliary excretion of Copper

Question 2

What is the clinical hallmark of Wilson’s disease?

Answer 2

Kayser-Fleisher ring in the eye

Question 3

What is the standard criterion for diagnosing Wilson’s disease?

Answer 3

Liver biopsy(>250mcg of Cu/1g of dry weight of liver —> diagnostic)

Question 4

How do you treat Wilson’s disease?

Answer 4

Copper chelation with oral chelating agents
Eg: D penicillamine
Treintine

Decreased copper in the diet