WILSON'S DISEASE - 05/09/11
Résumé |
Concepts concerning the cause and the pathogenesis of Wilson's hepatolenticular degeneration have undergone a slow and tortuous evolution.49 Hints regarding the presence of excess copper in the tissues of a patient were obtained as early as 1913, 1 year after Wilson's publication, but Rumpel believed that silver was the principal toxin.24 A decade later, ophthalmologists noted the similarity of Kayser-Fleischer rings and the copper-induced corneal rings encountered in patients with chalcosis but failed to appreciate the significance of these isolated observations. Only toward the middle of the century did Cumings5 establish that Wilson's disease is the result of copper toxicity, concluding that removal of copper from the tissues may be therapeutic. At that time British Anti-Lewisite, 2,3-dimercaptopropanol (BAL) was the only chelating agent used to treat patients with neurologic Wilson's disease. A few years later, Scheinberg and Gitlin discovered the pathognomonic deficiency of ceruloplasmin in plasma.26 Soon thereafter, Walshe introduced two oral therapies: first penicillamine, and later trientine, which revolutionized patient management and drastically improved the prognosis of patients diagnosed before irreversible damage to liver and brain had taken place.44, 45, 47 Several years later, zinc salts were found to be effective in the management of certain patients.2, 33, 50
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Vol 4 - N° 1
P. 229-239 - février 2000 Retour au numéro
Article précédent
- HEMOCHROMATOSIS
- Lawrie W. Powell, Thomas R. Yapp
- LIVER TRANSPLANTATION AT THE MILLENNIUM : Past, Present, and Future
- Emmet B. Keeffe
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