Copper toxicosis is an hereditary disease in which failure of the liver to expel dietary copper leads to a build-up of this toxic metal causing illness and death. It is inherited as an autosomal recessive trait. As such, two copies of the defective gene, one inherited from each parent, need to be present before an individual displays clinical signs. Dogs with one copy of the defective gene and ...

BACKGROUND Wilson's disease (WD) is an inherited disorder of copper metabolism leading to liver failure and/or neurological impairment. Its diagnosis often remains difficult even with genetic testing. Relative exchangeable copper (REC) has recently been described as a reliable serum diagnostic marker for WD. METHODOLOGY/PRINCIPAL FINDINGS The aim of this study was to validate the use of REC i...

BACKGROUND AND PURPOSE MR imaging sheds new light on CNS involvement in the course of acquired chronic liver disease; however, the exact pathogenetic mechanisms of hepatic encephalopathy and associated MR abnormalities remain unclear. Our purpose was to relate MR signal intensity abnormalities of the CNS to clinical, biochemical, and pathologic features of childhood-onset chronic liver disease....