Copper and liver disease.
There has been some limited progress in the understanding of the basic defect in Wilson disease and the gene concerned has been located to the chromosome region 13q14. Treatment with zinc has emerged as a definite alternative to penicillamine administration and some shortcomings and/or hazards of both forms of therapy have emerged as their modes of action have been studied more carefully. Tetrathimolybdate may have a place in treatment, especially when rapid complexing of copper is important. Hepatic copper accumulation occurs in a number of cholestatic diseases and they play an important part in pathogenesis and can occasionally lead to neurologic toxic effects. Copper overload in the newborn period when biliary excretion of copper is inefficient may establish a vicious cycle of copper accumulation and liver damage in Indian childhood cirrhosis and less frequently in babies in other countries.
Danks
D M
DM
Murdoch Institute, Royal Children's Hospital, Parkville, Victoria, Australia.
D000328:Adult; D002614:Chelating Agents / Q000627:therapeutic use; D002648:Child; D002779:Cholestasis / Q000209:etiology / Q000503:physiopathology*; D003300:Copper / Q000502:physiology*; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008107:Liver Diseases / Q000188:drug therapy* / Q000503:physiopathology; D008982:Molybdenum / Q000627:therapeutic use; D010396:Penicillamine / Q000627:therapeutic use*; D015032:Zinc / Q000627:therapeutic use
D002614:Chelating Agents; D003300:Copper; D008982:Molybdenum; C020809:tetrathiomolybdate; D010396:Penicillamine; D015032:Zinc