| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
Department of Human Genetics, University of Michigan, Ann Arbor, Michigan 48109–0618
Wilson's disease is a relatively rare inherited disorder of copper accumulation and toxicity, caused by a defect in an enzyme that is part of the pathway of biliary excretion of excess copper. Clinically, patients usually present as older children or young adults with hepatic, neurologic, or psychiatric manifestations, or some combination of these. Wilson's disease is unusual among genetic diseases in that it can be very effectively treated. The prevention of severe permanent damage depends upon early recognition and diagnosis by the physician, followed by appropriate anticopper treatment. Anticopper treatments have evolved considerably since the days when the only drug available was penicillamine. Zinc is now the recommended therapy for long-term management of the disease.
This article has been cited by other articles:
|
|
 |
|
  K. H. Weiss, D. Gotthardt, J. Schmidt, P. Schemmer, J. Encke, C. Riediger, W. Stremmel, P. Sauer, and U. Merle Liver transplantation for metabolic liver diseases in adults: indications and outcome Nephrol. Dial. Transplant., September 1, 2007; 22(suppl_8): viii9 - viii12. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 U Merle, M Schaefer, P Ferenci, and W Stremmel Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study Gut, January 1, 2007; 56(1): 115 - 120. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T.J. Kim, I.O. Kim, W.S. Kim, J.E. Cheon, S.G. Moon, J.W. Kwon, J.K. Seo, and K.M. Yeon MR Imaging of the Brain in Wilson Disease of Childhood: Findings Before and After Treatment with Clinical Correlation AJNR Am. J. Neuroradiol., June 1, 2006; 27(6): 1373 - 1378. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L.K. Prashanth, A.B. Taly, S. Sinha, S. Ravishankar, G.R. Arunodaya, M.K. Vasudev, and H.S. Swamy Prognostic factors in patients presenting with severe neurological forms of Wilson's disease QJM, August 1, 2005; 98(8): 557 - 563. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. G. Elner, V. M. Elner, A. Yoshida, R. D. Dick, and G. J. Brewer Effects of Tetrathiomolybdate in a Mouse Model of Retinal Neovascularization Invest. Ophthalmol. Vis. Sci., January 1, 2005; 46(1): 299 - 303. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. L. Nuttall, J. Palaty, and G. Lockitch Reference Limits for Copper and Iron in Liver Biopsies Ann. Clin. Lab. Sci., October 1, 2003; 33(4): 443 - 450. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. J. Brewer Copper Control as an Antiangiogenic Anticancer Therapy: Lessons from Treating Wilson's Disease Experimental Biology and Medicine, July 1, 2001; 226(7): 665 - 673. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. J. Brewer Crises in Academic Medicine Experimental Biology and Medicine, April 1, 2001; 226(4): 239 - 242. [Abstract] [Full Text]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
