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Eur J Pediatr. 1991 Jan;150(3):142-8.

Copper and liver disease.

Author information

1
Murdoch Institute, Royal Children's Hospital, Parkville, Victoria, Australia.

Abstract

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.

PMID:
2044580
DOI:
10.1007/bf01963553
[Indexed for MEDLINE]

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