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Wilson’s Disease — Hepatolenticular Degeneration the Importance of Early Diagnosis

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Pediatric Liver Disease

Part of the book series: Hepatology ((H,volume 5))

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Abstract

Wilson’s disease is a genetically determined disease, due to a defect in a single gene (1). Both parents are heterozygous carriers of this abnormal gene and never develop any signs or symptoms of Wilson’s disease. However, their children have a 1:4 chance of being homozygous and thus having Wilson’s disease. They also have a 1:2 chance of being heterozygous carriers like their parents and a 1:4 chance of being homozygous normal (2). All this is characteristic of an autosomal recessive form of inheritance.

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References

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© 1983 Plenum Press, New York

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Sass-Kortsak, A. (1983). Wilson’s Disease — Hepatolenticular Degeneration the Importance of Early Diagnosis. In: Fisher, M.M., Roy, C.C. (eds) Pediatric Liver Disease. Hepatology, vol 5. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-4385-1_24

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  • DOI: https://doi.org/10.1007/978-1-4684-4385-1_24

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-4387-5

  • Online ISBN: 978-1-4684-4385-1

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