Treatment of Wilson's disease with zinc: XV long-term follow-up studies

George J. Brewer, Robert D. Dick, Virginia D. Johnson, James A Brunberg, Karen J. Kluin, John K. Fink

Research output: Contribution to journalArticle

193 Citations (Scopus)

Abstract

Wilson's disease is an inherited disease of copper accumulation caused by a failure of biliary excretion of excess copper. Accumulated copper causes liver disease in these patients, and in perhaps two thirds of patients, it causes brain damage leading to clinical neurologic or psychiatric dysfunction. Maintenance treatment involves reversing the positive copper balance. The earliest approaches have used chelators, such as penicillamine or trientine, which increase the urinary excretion of copper. A more recent approach has used zinc, which blocks the absorption of copper and increases copper excretion in the stool. Because of the high level of endogenously secreted copper in alimentary secretions, the reabsorption of which is partially blocked by zinc therapy, zinc acts to remove accumulated copper from the body as well as prevent its reaccumulation. In the present article we present data on the long-term follow-up (up to 10 years) of maintenance zinc treatment of 141 patients with Wilson's disease. The data presented document that zinc is effective as a sole therapy in the long-term maintenance treatment of Wilson's disease and that it has a low toxicity. The results demonstrate the efficacy of zinc therapy in treating the presymptomatic patient from the beginning of therapy. We also present limited data on the use of zinc in the treatment of pregnant patients and children who have Wilson's disease; these data also indicate efficacy and low toxicity. The median follow-up period for the group as a whole is 4.8 years; for the presymptomatic patients it is 6.5 years; for the children it is 3.6 years.

Original languageEnglish (US)
Pages (from-to)264-278
Number of pages15
JournalJournal of Laboratory and Clinical Medicine
Volume132
Issue number4
StatePublished - Oct 1998
Externally publishedYes

Fingerprint

Hepatolenticular Degeneration
Zinc
Copper
Therapeutics
Toxicity
Trientine
Penicillamine
Chelating Agents
Liver
Nervous System
Psychiatry
Liver Diseases
Brain
Maintenance

ASJC Scopus subject areas

  • Medicine(all)
  • Pathology and Forensic Medicine

Cite this

Brewer, G. J., Dick, R. D., Johnson, V. D., Brunberg, J. A., Kluin, K. J., & Fink, J. K. (1998). Treatment of Wilson's disease with zinc: XV long-term follow-up studies. Journal of Laboratory and Clinical Medicine, 132(4), 264-278.

Treatment of Wilson's disease with zinc : XV long-term follow-up studies. / Brewer, George J.; Dick, Robert D.; Johnson, Virginia D.; Brunberg, James A; Kluin, Karen J.; Fink, John K.

In: Journal of Laboratory and Clinical Medicine, Vol. 132, No. 4, 10.1998, p. 264-278.

Research output: Contribution to journalArticle

Brewer, GJ, Dick, RD, Johnson, VD, Brunberg, JA, Kluin, KJ & Fink, JK 1998, 'Treatment of Wilson's disease with zinc: XV long-term follow-up studies', Journal of Laboratory and Clinical Medicine, vol. 132, no. 4, pp. 264-278.
Brewer, George J. ; Dick, Robert D. ; Johnson, Virginia D. ; Brunberg, James A ; Kluin, Karen J. ; Fink, John K. / Treatment of Wilson's disease with zinc : XV long-term follow-up studies. In: Journal of Laboratory and Clinical Medicine. 1998 ; Vol. 132, No. 4. pp. 264-278.
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