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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.
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