Abstract
Remarkable improvements in the accuracy of the diagnosis, and an increased range of therapeutic measures available for the treatment of Wilson’s disease have dramatically changed the clinical picture and the prognosis of patients with this previously progressive and fatal disorder1. This inherited disturbance of copper metabolism is distributed worldwide2. It is characterized by copper toxicosis first affecting the liver and later the central nervous system, eyes and kidneys. Despite the presence of the genetic disorder from birth, clinically overt disease hardly ever occurs before 5 years of age.
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References
Wilson, S. A. K. (1912). Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain, 34, 295–509
Scheinberg, I. H. and Sternlieb, I. (1984). Wilson’s Disease. 171 pp. ( Philadelphia: W. B. Saunders )
O’Reilly, S., Weber, P. M., Oswald, M. and Shipley, L. (1971). Abnormalities of the physiology of copper in Wilson’s disease. III. The excretion of copper. Arch. Neurol., 25, 28–32
Frommer, D. J. (1974). Defective biliary excretion of copper in Wilson’s disease. Gut, 15, 125–9
Sternlieb, I., van den Hamer, C. J. A., Morell, A. G., Alpert, S., Gregoriadis, G. and Scheinberg, I. H. (1973). Lysosomal defect of hepatic copper excretion in Wilson’s disease (hepatolenticular degeneration). Gastroenterology, 64, 99–105
Goldfischer, S. and Sternlieb, I. (1968). Changes in the distribution of hepatic copper in relation to the progression of Wilson’s disease (hepatolenticular degeneration). Am. J. Pathol., 53, 883–901
Sternlieb, I. and Scheinberg, I. H. (1972). Chronic hepatitis as a first manifestation of Wilson’s disease. Ann. Intern. Med., 76, 59–64
McCullough, A. J., Fleming, C. R., Thistle, J. L. et al. (1983). Diagnosis of Wilson’s disease presenting as fulminant hepatic failure. Gastroenterology, 84, 161–7
Walshe, J. M . (1973). Copper chelation in patients with Wilson’s disease. Q. J. Med., 46, 73–83
Walshe, J. M . (1982). Treatment of Wilson’s disease with Trientine (Triethylene tetramine) dihydrochloride. Lancet, i, 643–7
Hoogenraad, T. U., van den Hamer, C. J. A. and van Hattum, J. (1984). Effective treatment of Wilson’s disease with oral zinc. Br. Med. J., 288, 273–6
Sternlieb, I. (1984). Wilson’s disease: Indications for liver transplants. Hepatology, 4, 15S–17S
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© 1985 MTP Press Limited
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Sternlieb, I. (1985). Wilson’s disease. In: Bianchi, L., Gerok, W., Popper, H. (eds) Trends in Hepatology. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-4904-1_18
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DOI: https://doi.org/10.1007/978-94-009-4904-1_18
Publisher Name: Springer, Dordrecht
Print ISBN: 978-94-010-8672-1
Online ISBN: 978-94-009-4904-1
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