MovDisordV3, PascalFrancis, Corpus, bibRecord, 001417

Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease

Identifieur interne : 001417 ( PascalFrancis/Corpus ); précédent : 001416; suivant : 001418

Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease

Auteurs : Barbara Leinweber ; J. Carsten Möller ; Andre Scherag ; Ulrike Reuner ; Peter Günther ; Christoph J. G. Lang ; Hartmut H. J. Schmidt ; Christoph Schrader ; Oliver Bandmann ; Anna Czlonkowska ; Wolfgang H. Oertel ; Harald Hefter

Source :

Movement disorders [ 0885-3185 ] ; 2008.

RBID : Pascal:08-0115896

Descripteurs français

Pascal (Inist)
- Maladie de Wilson, Pathologie du système nerveux, Evaluation, Echelle d'évaluation, Allemand, Homme, Traitement, Cuivre.

English descriptors

KwdEn :
- Copper, Evaluation, Evaluation scale, German, Human, Nervous system diseases, Treatment, Wilson disease.

Abstract

Wilson's disease (WD) is an inherited autosomal-recessive disorder of copper metabolism characterized by a wide variety of neurological, hepatic, and psychiatric symptoms. The aim of the present study was the development and evaluation of a clinical rating scale, termed Unified Wilson's Disease Rating Scale (UWDRS), to assess the whole spectrum of clinical symptoms in WD. Altogether 107 patients (mean age 37.6 ± 11.9 years; 46 male, 61 female) with treated WD participated in the study. Cronbach's alpha as a measure of the internal consistency for the entire scale was 0.92, whereas the intraclass correlation coefficient (ICC) was 0.98 (confidence interval (CI_95%) 0.97-0.99), indicating an excellent interrater reliability as determined in 32 patients. Besides the total score was significantly correlated with the earning capacity of the patients as indicated by an estimated Spearman's p ≃ 0.54 (CI_95% 0.40-0.69, P < 0.001). In summary, the UWDRS appears to be a promising tool to assess the disease severity in WD. Its usefulness in clinical research and drug trials should be further addressed.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

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A08	`01`	`1`	`ENG`	`@1 Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease`
A11	`01`	`1`		`@1 LEINWEBER (Barbara)`
A11	`02`	`1`		`@1 MÖLLER (J. Carsten)`
A11	`03`	`1`		`@1 SCHERAG (Andre)`
A11	`04`	`1`		`@1 REUNER (Ulrike)`
A11	`05`	`1`		`@1 GÜNTHER (Peter)`
A11	`06`	`1`		`@1 LANG (Christoph J. G.)`
A11	`07`	`1`		`@1 SCHMIDT (Hartmut H. J.)`
A11	`08`	`1`		`@1 SCHRADER (Christoph)`
A11	`09`	`1`		`@1 BANDMANN (Oliver)`
A11	`10`	`1`		`@1 CZLONKOWSKA (Anna)`
A11	`11`	`1`		`@1 OERTEL (Wolfgang H.)`
A11	`12`	`1`		`@1 HEFTER (Harald)`
A14	`01`			`@1 Department of Neurology, Philipps-University Marburg @3 DEU @Z 1 aut. @Z 2 aut. @Z 11 aut.`
A14	`02`			`@1 Institute of Medical Biometry and Epidemiology, Philipps-University Marburg @3 DEU @Z 3 aut.`
A14	`03`			`@1 Department of Neurology, Technical University Dresden @3 DEU @Z 4 aut.`
A14	`04`			`@1 Department of Neurology, University Leipzig @3 DEU @Z 5 aut.`
A14	`05`			`@1 Department of Neurology, Friedrich-Alexander-University Erlangen-Nürnberg @3 DEU @Z 6 aut.`
A14	`06`			`@1 Department of Gastroenterology and General Surgery, Transplant Hepatology, University of Munster @3 DEU @Z 7 aut.`
A14	`07`			`@1 Department of Neurology, Hannover Medical School @3 DEU @Z 8 aut.`
A14	`08`			`@1 Academic Neurology Unit, University of Sheffield @2 Great Britain @3 GBR @Z 9 aut.`
A14	`09`			`@1 Department of Neurology, Institute of Psychiatry and Neurology and Department of Pharmacology, Medical University @2 Warsaw @3 POL @Z 10 aut.`
A14	`10`			`@1 Department of Neurology, Heinrich-Heine-University Düsseldorf @3 DEU @Z 12 aut.`
A20				`@1 54-62`
A21				`@1 2008`
A23	`01`			`@0 ENG`
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A44				`@0 0000 @1 © 2008 INIST-CNRS. All rights reserved.`
A45				`@0 30 ref.`
A47	`01`	`1`		`@0 08-0115896`
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A61				`@0 A`
A64	`01`	`1`		`@0 Movement disorders`
A66	`01`			`@0 USA`
C01	`01`		`ENG`	@0 Wilson's disease (WD) is an inherited autosomal-recessive disorder of copper metabolism characterized by a wide variety of neurological, hepatic, and psychiatric symptoms. The aim of the present study was the development and evaluation of a clinical rating scale, termed Unified Wilson's Disease Rating Scale (UWDRS), to assess the whole spectrum of clinical symptoms in WD. Altogether 107 patients (mean age 37.6 ± 11.9 years; 46 male, 61 female) with treated WD participated in the study. Cronbach's alpha as a measure of the internal consistency for the entire scale was 0.92, whereas the intraclass correlation coefficient (ICC) was 0.98 (confidence interval (CI_95%) 0.97-0.99), indicating an excellent interrater reliability as determined in 32 patients. Besides the total score was significantly correlated with the earning capacity of the patients as indicated by an estimated Spearman's p ≃ 0.54 (CI_95% 0.40-0.69, P < 0.001). In summary, the UWDRS appears to be a promising tool to assess the disease severity in WD. Its usefulness in clinical research and drug trials should be further addressed.
C02	`01`	`X`		`@0 002B17`
C02	`02`	`X`		`@0 002B17G`
C03	`01`	`X`	`FRE`	`@0 Maladie de Wilson @5 01`
C03	`01`	`X`	`ENG`	`@0 Wilson disease @5 01`
C03	`01`	`X`	`SPA`	`@0 Wilson enfermedad @5 01`
C03	`02`	`X`	`FRE`	`@0 Pathologie du système nerveux @5 02`
C03	`02`	`X`	`ENG`	`@0 Nervous system diseases @5 02`
C03	`02`	`X`	`SPA`	`@0 Sistema nervioso patología @5 02`
C03	`03`	`X`	`FRE`	`@0 Evaluation @5 09`
C03	`03`	`X`	`ENG`	`@0 Evaluation @5 09`
C03	`03`	`X`	`SPA`	`@0 Evaluación @5 09`
C03	`04`	`X`	`FRE`	`@0 Echelle d'évaluation @5 10`
C03	`04`	`X`	`ENG`	`@0 Evaluation scale @5 10`
C03	`04`	`X`	`SPA`	`@0 Escala evaluación @5 10`
C03	`05`	`X`	`FRE`	`@0 Allemand @5 11`
C03	`05`	`X`	`ENG`	`@0 German @5 11`
C03	`05`	`X`	`SPA`	`@0 Alemán @5 11`
C03	`06`	`X`	`FRE`	`@0 Homme @5 12`
C03	`06`	`X`	`ENG`	`@0 Human @5 12`
C03	`06`	`X`	`SPA`	`@0 Hombre @5 12`
C03	`07`	`X`	`FRE`	`@0 Traitement @5 13`
C03	`07`	`X`	`ENG`	`@0 Treatment @5 13`
C03	`07`	`X`	`SPA`	`@0 Tratamiento @5 13`
C03	`08`	`X`	`FRE`	`@0 Cuivre @2 NC @5 78`
C03	`08`	`X`	`ENG`	`@0 Copper @2 NC @5 78`
C03	`08`	`X`	`SPA`	`@0 Cobre @2 NC @5 78`
C07	`01`	`X`	`FRE`	`@0 Pathologie de l'appareil digestif @5 37`
C07	`01`	`X`	`ENG`	`@0 Digestive diseases @5 37`
C07	`01`	`X`	`SPA`	`@0 Aparato digestivo patología @5 37`
C07	`02`	`X`	`FRE`	`@0 Enzymopathie @5 38`
C07	`02`	`X`	`ENG`	`@0 Enzymopathy @5 38`
C07	`02`	`X`	`SPA`	`@0 Enzimopatía @5 38`
C07	`03`	`X`	`FRE`	`@0 Maladie héréditaire @5 39`
C07	`03`	`X`	`ENG`	`@0 Genetic disease @5 39`
C07	`03`	`X`	`SPA`	`@0 Enfermedad hereditaria @5 39`
C07	`04`	`X`	`FRE`	`@0 Maladie métabolique @5 40`
C07	`04`	`X`	`ENG`	`@0 Metabolic diseases @5 40`
C07	`04`	`X`	`SPA`	`@0 Metabolismo patología @5 40`
N21				`@1 063`
N44	`01`			`@1 OTO`
N82				`@1 OTO`

Format Inist (serveur)

NO :	PASCAL 08-0115896 INIST
ET :	Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease
AU :	LEINWEBER (Barbara); MÖLLER (J. Carsten); SCHERAG (Andre); REUNER (Ulrike); GÜNTHER (Peter); LANG (Christoph J. G.); SCHMIDT (Hartmut H. J.); SCHRADER (Christoph); BANDMANN (Oliver); CZLONKOWSKA (Anna); OERTEL (Wolfgang H.); HEFTER (Harald)
AF :	Department of Neurology, Philipps-University Marburg/Allemagne (1 aut., 2 aut., 11 aut.); Institute of Medical Biometry and Epidemiology, Philipps-University Marburg/Allemagne (3 aut.); Department of Neurology, Technical University Dresden/Allemagne (4 aut.); Department of Neurology, University Leipzig/Allemagne (5 aut.); Department of Neurology, Friedrich-Alexander-University Erlangen-Nürnberg/Allemagne (6 aut.); Department of Gastroenterology and General Surgery, Transplant Hepatology, University of Munster/Allemagne (7 aut.); Department of Neurology, Hannover Medical School/Allemagne (8 aut.); Academic Neurology Unit, University of Sheffield/Great Britain/Royaume-Uni (9 aut.); Department of Neurology, Institute of Psychiatry and Neurology and Department of Pharmacology, Medical University/Warsaw/Pologne (10 aut.); Department of Neurology, Heinrich-Heine-University Düsseldorf/Allemagne (12 aut.)
DT :	Publication en série; Niveau analytique
SO :	Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2008; Vol. 23; No. 1; Pp. 54-62; Bibl. 30 ref.
LA :	Anglais
EA :	Wilson's disease (WD) is an inherited autosomal-recessive disorder of copper metabolism characterized by a wide variety of neurological, hepatic, and psychiatric symptoms. The aim of the present study was the development and evaluation of a clinical rating scale, termed Unified Wilson's Disease Rating Scale (UWDRS), to assess the whole spectrum of clinical symptoms in WD. Altogether 107 patients (mean age 37.6 ± 11.9 years; 46 male, 61 female) with treated WD participated in the study. Cronbach's alpha as a measure of the internal consistency for the entire scale was 0.92, whereas the intraclass correlation coefficient (ICC) was 0.98 (confidence interval (CI_95%) 0.97-0.99), indicating an excellent interrater reliability as determined in 32 patients. Besides the total score was significantly correlated with the earning capacity of the patients as indicated by an estimated Spearman's p ≃ 0.54 (CI_95% 0.40-0.69, P < 0.001). In summary, the UWDRS appears to be a promising tool to assess the disease severity in WD. Its usefulness in clinical research and drug trials should be further addressed.
CC :	002B17; 002B17G
FD :	Maladie de Wilson; Pathologie du système nerveux; Evaluation; Echelle d'évaluation; Allemand; Homme; Traitement; Cuivre
FG :	Pathologie de l'appareil digestif; Enzymopathie; Maladie héréditaire; Maladie métabolique
ED :	Wilson disease; Nervous system diseases; Evaluation; Evaluation scale; German; Human; Treatment; Copper
EG :	Digestive diseases; Enzymopathy; Genetic disease; Metabolic diseases
SD :	Wilson enfermedad; Sistema nervioso patología; Evaluación; Escala evaluación; Alemán; Hombre; Tratamiento; Cobre
LO :	INIST-20953.354000161904810080
ID :	08-0115896

Links to Exploration step

Pascal:08-0115896

Le document en format XML

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<author><name sortKey="Hefter, Harald" sort="Hefter, Harald" uniqKey="Hefter H" first="Harald" last="Hefter">Harald Hefter</name>
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<series><title level="j" type="main">Movement disorders</title>
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<front><div type="abstract" xml:lang="en">Wilson's disease (WD) is an inherited autosomal-recessive disorder of copper metabolism characterized by a wide variety of neurological, hepatic, and psychiatric symptoms. The aim of the present study was the development and evaluation of a clinical rating scale, termed Unified Wilson's Disease Rating Scale (UWDRS), to assess the whole spectrum of clinical symptoms in WD. Altogether 107 patients (mean age 37.6 ± 11.9 years; 46 male, 61 female) with treated WD participated in the study. Cronbach's alpha as a measure of the internal consistency for the entire scale was 0.92, whereas the intraclass correlation coefficient (ICC) was 0.98 (confidence interval (CI<sub>95%</sub>
) 0.97-0.99), indicating an excellent interrater reliability as determined in 32 patients. Besides the total score was significantly correlated with the earning capacity of the patients as indicated by an estimated Spearman's p ≃ 0.54 (CI<sub>95%</sub>
 0.40-0.69, P < 0.001). In summary, the UWDRS appears to be a promising tool to assess the disease severity in WD. Its usefulness in clinical research and drug trials should be further addressed.</div>
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<fA11 i1="01" i2="1"><s1>LEINWEBER (Barbara)</s1>
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<fA11 i1="02" i2="1"><s1>MÖLLER (J. Carsten)</s1>
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<sZ>9 aut.</sZ>
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<fA14 i1="09"><s1>Department of Neurology, Institute of Psychiatry and Neurology and Department of Pharmacology, Medical University</s1>
<s2>Warsaw</s2>
<s3>POL</s3>
<sZ>10 aut.</sZ>
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<fA14 i1="10"><s1>Department of Neurology, Heinrich-Heine-University Düsseldorf</s1>
<s3>DEU</s3>
<sZ>12 aut.</sZ>
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<fA20><s1>54-62</s1>
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<fC01 i1="01" l="ENG"><s0>Wilson's disease (WD) is an inherited autosomal-recessive disorder of copper metabolism characterized by a wide variety of neurological, hepatic, and psychiatric symptoms. The aim of the present study was the development and evaluation of a clinical rating scale, termed Unified Wilson's Disease Rating Scale (UWDRS), to assess the whole spectrum of clinical symptoms in WD. Altogether 107 patients (mean age 37.6 ± 11.9 years; 46 male, 61 female) with treated WD participated in the study. Cronbach's alpha as a measure of the internal consistency for the entire scale was 0.92, whereas the intraclass correlation coefficient (ICC) was 0.98 (confidence interval (CI<sub>95%</sub>
) 0.97-0.99), indicating an excellent interrater reliability as determined in 32 patients. Besides the total score was significantly correlated with the earning capacity of the patients as indicated by an estimated Spearman's p ≃ 0.54 (CI<sub>95%</sub>
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<s5>40</s5>
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<s5>40</s5>
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<ET>Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease</ET>
<AU>LEINWEBER (Barbara); MÖLLER (J. Carsten); SCHERAG (Andre); REUNER (Ulrike); GÜNTHER (Peter); LANG (Christoph J. G.); SCHMIDT (Hartmut H. J.); SCHRADER (Christoph); BANDMANN (Oliver); CZLONKOWSKA (Anna); OERTEL (Wolfgang H.); HEFTER (Harald)</AU>
<AF>Department of Neurology, Philipps-University Marburg/Allemagne (1 aut., 2 aut., 11 aut.); Institute of Medical Biometry and Epidemiology, Philipps-University Marburg/Allemagne (3 aut.); Department of Neurology, Technical University Dresden/Allemagne (4 aut.); Department of Neurology, University Leipzig/Allemagne (5 aut.); Department of Neurology, Friedrich-Alexander-University Erlangen-Nürnberg/Allemagne (6 aut.); Department of Gastroenterology and General Surgery, Transplant Hepatology, University of Munster/Allemagne (7 aut.); Department of Neurology, Hannover Medical School/Allemagne (8 aut.); Academic Neurology Unit, University of Sheffield/Great Britain/Royaume-Uni (9 aut.); Department of Neurology, Institute of Psychiatry and Neurology and Department of Pharmacology, Medical University/Warsaw/Pologne (10 aut.); Department of Neurology, Heinrich-Heine-University Düsseldorf/Allemagne (12 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2008; Vol. 23; No. 1; Pp. 54-62; Bibl. 30 ref.</SO>
<LA>Anglais</LA>
<EA>Wilson's disease (WD) is an inherited autosomal-recessive disorder of copper metabolism characterized by a wide variety of neurological, hepatic, and psychiatric symptoms. The aim of the present study was the development and evaluation of a clinical rating scale, termed Unified Wilson's Disease Rating Scale (UWDRS), to assess the whole spectrum of clinical symptoms in WD. Altogether 107 patients (mean age 37.6 ± 11.9 years; 46 male, 61 female) with treated WD participated in the study. Cronbach's alpha as a measure of the internal consistency for the entire scale was 0.92, whereas the intraclass correlation coefficient (ICC) was 0.98 (confidence interval (CI<sub>95%</sub>
) 0.97-0.99), indicating an excellent interrater reliability as determined in 32 patients. Besides the total score was significantly correlated with the earning capacity of the patients as indicated by an estimated Spearman's p ≃ 0.54 (CI<sub>95%</sub>
 0.40-0.69, P < 0.001). In summary, the UWDRS appears to be a promising tool to assess the disease severity in WD. Its usefulness in clinical research and drug trials should be further addressed.</EA>
<CC>002B17; 002B17G</CC>
<FD>Maladie de Wilson; Pathologie du   système nerveux; Evaluation; Echelle d'évaluation; Allemand; Homme; Traitement; Cuivre</FD>
<FG>Pathologie de l'appareil digestif; Enzymopathie; Maladie héréditaire; Maladie métabolique</FG>
<ED>Wilson disease; Nervous system diseases; Evaluation; Evaluation scale; German; Human; Treatment; Copper</ED>
<EG>Digestive diseases; Enzymopathy; Genetic disease; Metabolic diseases</EG>
<SD>Wilson enfermedad; Sistema nervioso patología; Evaluación; Escala evaluación; Alemán; Hombre; Tratamiento; Cobre</SD>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease

Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease

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