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Wilson's disease-cause of mortality in 164 patients during 1992-2003 observation period.
- Source :
-
Journal of neurology [J Neurol] 2005 Jun; Vol. 252 (6), pp. 698-703. Date of Electronic Publication: 2005 Mar 02. - Publication Year :
- 2005
-
Abstract
- We studied the cause of death in a consecutive series of 164 patients with Wilson's disease (WD) diagnosed over an 11 year period. A total of 20 [12% (95% CI 10.3-16.0)] died during the observation period. The relative survival rate of all patients in our group was statistically smaller than in Polish population. The main cause of death was the diagnosis in advanced stage of disease, but in six patients presenting with mild signs, we observed the progression of the disease despite treatment. There was no difference in mortality rate in patients treated with d-penicillamine or zinc sulphate as initial therapy. The prognosis for survival in the majority of WD patients is favourable, provided that therapy is introduced early.
- Subjects :
- Adult
Brain drug effects
Brain metabolism
Brain pathology
Confidence Intervals
Copper metabolism
Disease Progression
Female
Hepatolenticular Degeneration drug therapy
Hepatolenticular Degeneration epidemiology
Humans
Male
Penicillamine therapeutic use
Retrospective Studies
Survival Rate
Treatment Outcome
Zinc Sulfate therapeutic use
Hepatolenticular Degeneration mortality
Observation methods
Subjects
Details
- Language :
- English
- ISSN :
- 0340-5354
- Volume :
- 252
- Issue :
- 6
- Database :
- MEDLINE
- Journal :
- Journal of neurology
- Publication Type :
- Academic Journal
- Accession number :
- 15742108
- Full Text :
- https://doi.org/10.1007/s00415-005-0720-4