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Old 07-12-2012
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Liver What are the Copper serum levels in Wilson Disease!

what are serum and urine free copper level in wilson disease

refer to goljan page number 378 in blue book . at side margin he says decreased but in text body they are increased.

i think they should be decreased because there would be no ceruloplasmin to bind to.

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Old 07-12-2012
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decreased total serum copper, decreased serum ceruloplasmin - Wilson's disease in early stages

increased serum and urine free copper - Wilson's disease in the later stages

ceruloplasmin is an enzyme synthesized in the liver. ceruloplasmin is secreted into the plasma where it represents 90-95% of the total serum copper concentration. the remaining 5-10% of copper is free copper that is bound to albumin. the copper that was bound to ceruloplasmin is excreted into the bile. the gene defect in wilson's disease affects a copper transport system that produces a dual defect-decreased incorporation of copper into ceruloplasmin in the liver and decreased excretion of copper into bile. accumulation of copper in the liver causing damage to hepatocytes. liver disease progresses from acute hepatitis to cirrhosis. in a few years , unbound copper is released from the liver into the circulation -increased in blood and urine where it damages the brain, kidneys, cornea and other tissues
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