Introduction
   

Copper Storage Disease:

Copper storage disease is most commonly described in Bedlington Terriers but it also occurs in Dobermans, West Highland White Terrier and humans (Wilson’s Disease). In Bedlington terriers it is associated with an autosomal recessive gene that prevents normal copper excretion by hepatocytes into the biliary system. Thus hepatocytes tend to accumulate copper until a critical level is reached and then there is cell death and release of copper.

Histologically there are signs of chronic hepatic disease, ie fibrosis, fatty change, bile duct proliferation and chronic inflammatory infiltrates around the portal triads. Some pigments that maybe identified on H&E section include haemosiderin (due to haemolysis ) and lipofuscin (due to increased cell organelle turnover). Copper is difficult to identify on H&E so special stains such as rubeanic acid or rhodamine are required.

Cases
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