Abstract
Wilson’s disease (hepatolenticular degeneration or hepatocerebral degeneration) is the consequence of an inherited, progressive and ultimately fatal defect in copper metabolism. This defect which is inherited in autosomal recessive fashion, results in the accumulation of excessive amounts of copper, first in the liver and subsequently in the brain, kidneys, eyes, erythrocytes and other tissues where toxic concentrations of copper cause pathologic changes. The liver is not only the organ which is most frequently and severely affected by the toxicity of copper, but it is also the site of the etiologic biochemical abnormality which causes the retention of the excessive oncentrations of copper (1).
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© 1982 The Humana Press Inc.
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Sternlieb, I. (1982). Wilson’s Disease. In: Sorenson, J.R.J. (eds) Inflammatory Diseases and Copper. Experimental Biology and Medicine, vol 2. Humana Press. https://doi.org/10.1007/978-1-4612-5829-2_8
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DOI: https://doi.org/10.1007/978-1-4612-5829-2_8
Publisher Name: Humana Press
Print ISBN: 978-1-4612-5831-5
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