Cause of death in Wilson disease.

Cause of death in Wilson disease. Research Abstract Details 

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Cause of death in Wilson disease. Abstract Text:

John M Walshe,John M Walshe,John M Walshe,

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.

Cause of death in Wilson disease. Publishing Authors By Initials

JM Walshe,JM Walshe,JM Walshe,

For similar nervous system diseases research abstracts see: nervous system diseases research

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Cause of death in Wilson disease. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Movement disorders : official journal of the Movem

VOLUME: 22

Page Numbers: 2216-20

Journal Abbreviation: Mov. Disord.

ISSN: 0885-3185

DAY: 15

MONTH: Nov

YEAR: 2007

Cause of death in Wilson disease. Information

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LANGUAGE: eng

NlmUniqueID: 8610688

Cause of death in Wilson disease. Keywords Mesh Terms:

KEYWORDS: Nervous System Diseases

MESH TERMS: mortality

Chemical & Substance for Abstract: Cause of death in Wilson disease. Information

Substance Name: Ceruloplasmin

Registry Number: EC 1.16.3.1

Grant and Affiliation Information for Cause of death in Wilson disease.

AFFILIATION: Formerly Department of Neurology, The Middlesex Hospital, Mortimer Street, London, UK. penicillamine@waitrose.com

Country: United States

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MEDLINETA: Mov Disord

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