Cause of death in Wilson disease
Identifieur interne : 002C23 ( Istex/Corpus ); précédent : 002C22; suivant : 002C24Cause of death in Wilson disease
Auteurs : John M. WalsheSource :
- Movement Disorders [ 0885-3185 ] ; 2007-11-15.
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Abstract
Before 1948, all patients with Wilson disease died shortly after diagnosis. In 1948, BAL (dimercaprol) was introduced as a possible effective treatment, to be followed by penicillamine (1955), zinc salts (1961), trientine (1969), liver transplantation (1982), and tetrathiomolybdate (1984). Despite this wide range of therapeutic options, patients still die. This article examines the cause of death in 67 patients (33 men, 34 women) out of a series of 300 seen between 1948 and 2000. Patients were classified according to their presentation as neurological, 32 patients, hepatic 11, mixed hepatic/neurological 10, hemolytic, 6, and “sibling biopsy ” 8. Diagnostic failure was the principal cause of death but there were multiple other causes of which the principal was poor compliance and the development of malignant disease after 10 years of follow‐up. The development of new symptoms should alert the physician to the possibility of a new pathology. © 2007 Movement Disorder Society
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DOI: 10.1002/mds.21693
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ISTEX:53D3E948962FB32A64EAB696CB48519EC165FC9BLe document en format XML
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