MovDisordV3, PascalFrancis, Curation, bibRecord, 001866

Cause of Death in Wilson Disease

Identifieur interne : 001866 ( PascalFrancis/Curation ); précédent : 001865; suivant : 001867

Cause of Death in Wilson Disease

Auteurs : John M. Walshe [Royaume-Uni]

Source :

Movement disorders [ 0885-3185 ] ; 2007.

RBID : Pascal:08-0071408

Descripteurs français

Pascal (Inist)
- Pathologie du système nerveux, Maladie de Wilson, Tumeur maligne, Cuivre, Diagnostic, Malignité.
Wicri :
- topic : Cuivre.

English descriptors

KwdEn :
- Copper, Diagnosis, Malignancy, Malignant tumor, Nervous system diseases, Wilson disease.

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.

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A11	`01`	`1`		`@1 WALSHE (John M.)`
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C01	`01`		`ENG`	@0 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.
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C03	`01`	`X`	`FRE`	`@0 Pathologie du système nerveux @5 01`
C03	`01`	`X`	`ENG`	`@0 Nervous system diseases @5 01`
C03	`01`	`X`	`SPA`	`@0 Sistema nervioso patología @5 01`
C03	`02`	`X`	`FRE`	`@0 Maladie de Wilson @5 02`
C03	`02`	`X`	`ENG`	`@0 Wilson disease @5 02`
C03	`02`	`X`	`SPA`	`@0 Wilson enfermedad @5 02`
C03	`03`	`X`	`FRE`	`@0 Tumeur maligne @2 NM @5 03`
C03	`03`	`X`	`ENG`	`@0 Malignant tumor @2 NM @5 03`
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C03	`04`	`X`	`FRE`	`@0 Cuivre @2 NC @5 09`
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C03	`04`	`X`	`SPA`	`@0 Cobre @2 NC @5 09`
C03	`05`	`X`	`FRE`	`@0 Diagnostic @5 10`
C03	`05`	`X`	`ENG`	`@0 Diagnosis @5 10`
C03	`05`	`X`	`SPA`	`@0 Diagnóstico @5 10`
C03	`06`	`X`	`FRE`	`@0 Malignité @5 11`
C03	`06`	`X`	`ENG`	`@0 Malignancy @5 11`
C03	`06`	`X`	`SPA`	`@0 Malignidad @5 11`
C07	`01`	`X`	`FRE`	`@0 Cancer @2 NM`
C07	`01`	`X`	`ENG`	`@0 Cancer @2 NM`
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C07	`02`	`X`	`FRE`	`@0 Pathologie de l'appareil digestif @5 37`
C07	`02`	`X`	`ENG`	`@0 Digestive diseases @5 37`
C07	`02`	`X`	`SPA`	`@0 Aparato digestivo patología @5 37`
C07	`03`	`X`	`FRE`	`@0 Enzymopathie @5 38`
C07	`03`	`X`	`ENG`	`@0 Enzymopathy @5 38`
C07	`03`	`X`	`SPA`	`@0 Enzimopatía @5 38`
C07	`04`	`X`	`FRE`	`@0 Maladie héréditaire @5 39`
C07	`04`	`X`	`ENG`	`@0 Genetic disease @5 39`
C07	`04`	`X`	`SPA`	`@0 Enfermedad hereditaria @5 39`
C07	`05`	`X`	`FRE`	`@0 Maladie métabolique @5 40`
C07	`05`	`X`	`ENG`	`@0 Metabolic diseases @5 40`
C07	`05`	`X`	`SPA`	`@0 Metabolismo patología @5 40`
N21				`@1 035`
N44	`01`			`@1 OTO`
N82				`@1 OTO`

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Pascal:08-0071408

Le document en format XML

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<front><div type="abstract" xml:lang="en">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.</div>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Cause of Death in Wilson Disease

Cause of Death in Wilson Disease

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Links to Exploration step

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri