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Pathology

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

The fundamental defect is a failure of hepatic excretion of copper into bile - copper accumulates in the liver and secondarily suppresses the synthesis of caeruloplasmin. Eventually, copper spills over into the circulation and deposits also in the:

  • basal ganglia - with neurological sequalae
  • kidneys - causing tubuar degeneration and thus a Fanconi syndrome
  • cornea - with Kayser-Fleischer rings
  • bones and joints, with erosions
  • parathyroid glands

Histological appearances of the liver are not diagnostic and vary from that of chronic active hepatitis to a macronodular cirrhosis. Copper staining may reveal a periportal distribution.

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