Wilson’s disease (Hepato-lenticular degeneration) is a rare hereditary disorder of copper metabolism. In Wilson’s disease, about 50 per cent of the ingested copper is absorbed as compared with 2 to 5 per cent in normal persons. There is also defect in ceruloplasmin formation. Hence, the copper absorbed is deposited in various tissues such as brain, liver, kidney and membrane in the eye. These organs may be damaged by poisoning of certain enzymes by copper or by cellular necrosis followed by fibrosis (or gliosis in the brain).
Last modified: Tuesday, 14 February 2012, 11:42 AM