Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease
Identifieur interne : 003870 ( Main/Merge ); précédent : 003869; suivant : 003871Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease
Auteurs : Barbara Leinweber [Allemagne] ; J. Carsten Möller [Allemagne] ; Andre Scherag [Allemagne] ; Ulrike Reuner [Allemagne] ; Peter Günther [Allemagne] ; Christoph J. G. Lang [Allemagne] ; Hartmut H. J. Schmidt [Allemagne] ; Christoph Schrader [Allemagne] ; Oliver Bandmann [Royaume-Uni] ; Anna Czlonkowska [Pologne] ; Wolfgang H. Oertel [Allemagne] ; Harald Hefter [Allemagne]Source :
- Movement disorders [ 0885-3185 ] ; 2008.
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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 (CI95%) 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 (CI95% 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.
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease</title><author><name sortKey="Leinweber, Barbara" sort="Leinweber, Barbara" uniqKey="Leinweber B" first="Barbara" last="Leinweber">Barbara Leinweber</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Philipps-University Marburg</s1><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>11 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>Department of Neurology, Philipps-University Marburg</wicri:noRegion></affiliation></author><author><name sortKey="Moller, J Carsten" sort="Moller, J Carsten" uniqKey="Moller J" first="J. Carsten" last="Möller">J. Carsten Möller</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Philipps-University Marburg</s1><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>11 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>Department of Neurology, Philipps-University Marburg</wicri:noRegion></affiliation></author><author><name sortKey="Scherag, Andre" sort="Scherag, Andre" uniqKey="Scherag A" first="Andre" last="Scherag">Andre Scherag</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Institute of Medical Biometry and Epidemiology, Philipps-University Marburg</s1><s3>DEU</s3><sZ>3 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>Institute of Medical Biometry and Epidemiology, Philipps-University Marburg</wicri:noRegion></affiliation></author><author><name sortKey="Reuner, Ulrike" sort="Reuner, Ulrike" uniqKey="Reuner U" first="Ulrike" last="Reuner">Ulrike Reuner</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Neurology, Technical University Dresden</s1><s3>DEU</s3><sZ>4 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Technical University Dresden</wicri:noRegion><wicri:noRegion>Department of Neurology, Technical University Dresden</wicri:noRegion></affiliation></author><author><name sortKey="Gunther, Peter" sort="Gunther, Peter" uniqKey="Gunther P" first="Peter" last="Günther">Peter Günther</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Neurology, University Leipzig</s1><s3>DEU</s3><sZ>5 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>University Leipzig</wicri:noRegion><wicri:noRegion>Department of Neurology, University Leipzig</wicri:noRegion></affiliation></author><author><name sortKey="Lang, Christoph J G" sort="Lang, Christoph J G" uniqKey="Lang C" first="Christoph J. G." last="Lang">Christoph J. G. Lang</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Department of Neurology, Friedrich-Alexander-University Erlangen-Nürnberg</s1><s3>DEU</s3><sZ>6 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Friedrich-Alexander-University Erlangen-Nürnberg</wicri:noRegion><wicri:noRegion>Department of Neurology, Friedrich-Alexander-University Erlangen-Nürnberg</wicri:noRegion></affiliation></author><author><name sortKey="Schmidt, Hartmut H J" sort="Schmidt, Hartmut H J" uniqKey="Schmidt H" first="Hartmut H. J." last="Schmidt">Hartmut H. J. Schmidt</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Gastroenterology and General Surgery, Transplant Hepatology, University of Munster</s1><s3>DEU</s3><sZ>7 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>University of Munster</wicri:noRegion><wicri:noRegion>Department of Gastroenterology and General Surgery, Transplant Hepatology, University of Munster</wicri:noRegion></affiliation></author><author><name sortKey="Schrader, Christoph" sort="Schrader, Christoph" uniqKey="Schrader C" first="Christoph" last="Schrader">Christoph Schrader</name><affiliation wicri:level="1"><inist:fA14 i1="07"><s1>Department of Neurology, Hannover Medical School</s1><s3>DEU</s3><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Hannover Medical School</wicri:noRegion><wicri:noRegion>Department of Neurology, Hannover Medical School</wicri:noRegion></affiliation></author><author><name sortKey="Bandmann, Oliver" sort="Bandmann, Oliver" uniqKey="Bandmann O" first="Oliver" last="Bandmann">Oliver Bandmann</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Academic Neurology Unit, University of Sheffield</s1><s2>Great Britain</s2><s3>GBR</s3><sZ>9 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Great Britain</wicri:noRegion></affiliation></author><author><name sortKey="Czlonkowska, Anna" sort="Czlonkowska, Anna" uniqKey="Czlonkowska A" first="Anna" last="Czlonkowska">Anna Czlonkowska</name><affiliation wicri:level="1"><inist: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></inist:fA14><country>Pologne</country><wicri:noRegion>Warsaw</wicri:noRegion></affiliation></author><author><name sortKey="Oertel, Wolfgang H" sort="Oertel, Wolfgang H" uniqKey="Oertel W" first="Wolfgang H." last="Oertel">Wolfgang H. Oertel</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Philipps-University Marburg</s1><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>11 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>Department of Neurology, Philipps-University Marburg</wicri:noRegion></affiliation></author><author><name sortKey="Hefter, Harald" sort="Hefter, Harald" uniqKey="Hefter H" first="Harald" last="Hefter">Harald Hefter</name><affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Department of Neurology, Heinrich-Heine-University Düsseldorf</s1><s3>DEU</s3><sZ>12 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Heinrich-Heine-University Düsseldorf</wicri:noRegion><wicri:noRegion>Department of Neurology, Heinrich-Heine-University Düsseldorf</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">08-0115896</idno><date when="2008">2008</date><idno type="stanalyst">PASCAL 08-0115896 INIST</idno><idno type="RBID">Pascal:08-0115896</idno><idno type="wicri:Area/PascalFrancis/Corpus">001417</idno><idno type="wicri:Area/PascalFrancis/Curation">001902</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">001262</idno><idno type="wicri:doubleKey">0885-3185:2008:Leinweber B:evaluation:of:the</idno><idno type="wicri:Area/Main/Merge">003870</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German Patients with Treated Wilson's Disease</title><author><name sortKey="Leinweber, Barbara" sort="Leinweber, Barbara" uniqKey="Leinweber B" first="Barbara" last="Leinweber">Barbara Leinweber</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Philipps-University Marburg</s1><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>11 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>Department of Neurology, Philipps-University Marburg</wicri:noRegion></affiliation></author><author><name sortKey="Moller, J Carsten" sort="Moller, J Carsten" uniqKey="Moller J" first="J. Carsten" last="Möller">J. Carsten Möller</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Philipps-University Marburg</s1><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>11 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>Department of Neurology, Philipps-University Marburg</wicri:noRegion></affiliation></author><author><name sortKey="Scherag, Andre" sort="Scherag, Andre" uniqKey="Scherag A" first="Andre" last="Scherag">Andre Scherag</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Institute of Medical Biometry and Epidemiology, Philipps-University Marburg</s1><s3>DEU</s3><sZ>3 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>Institute of Medical Biometry and Epidemiology, Philipps-University Marburg</wicri:noRegion></affiliation></author><author><name sortKey="Reuner, Ulrike" sort="Reuner, Ulrike" uniqKey="Reuner U" first="Ulrike" last="Reuner">Ulrike Reuner</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Neurology, Technical University Dresden</s1><s3>DEU</s3><sZ>4 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Technical University Dresden</wicri:noRegion><wicri:noRegion>Department of Neurology, Technical University Dresden</wicri:noRegion></affiliation></author><author><name sortKey="Gunther, Peter" sort="Gunther, Peter" uniqKey="Gunther P" first="Peter" last="Günther">Peter Günther</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Neurology, University Leipzig</s1><s3>DEU</s3><sZ>5 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>University Leipzig</wicri:noRegion><wicri:noRegion>Department of Neurology, University Leipzig</wicri:noRegion></affiliation></author><author><name sortKey="Lang, Christoph J G" sort="Lang, Christoph J G" uniqKey="Lang C" first="Christoph J. G." last="Lang">Christoph J. G. Lang</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Department of Neurology, Friedrich-Alexander-University Erlangen-Nürnberg</s1><s3>DEU</s3><sZ>6 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Friedrich-Alexander-University Erlangen-Nürnberg</wicri:noRegion><wicri:noRegion>Department of Neurology, Friedrich-Alexander-University Erlangen-Nürnberg</wicri:noRegion></affiliation></author><author><name sortKey="Schmidt, Hartmut H J" sort="Schmidt, Hartmut H J" uniqKey="Schmidt H" first="Hartmut H. J." last="Schmidt">Hartmut H. J. Schmidt</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Gastroenterology and General Surgery, Transplant Hepatology, University of Munster</s1><s3>DEU</s3><sZ>7 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>University of Munster</wicri:noRegion><wicri:noRegion>Department of Gastroenterology and General Surgery, Transplant Hepatology, University of Munster</wicri:noRegion></affiliation></author><author><name sortKey="Schrader, Christoph" sort="Schrader, Christoph" uniqKey="Schrader C" first="Christoph" last="Schrader">Christoph Schrader</name><affiliation wicri:level="1"><inist:fA14 i1="07"><s1>Department of Neurology, Hannover Medical School</s1><s3>DEU</s3><sZ>8 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Hannover Medical School</wicri:noRegion><wicri:noRegion>Department of Neurology, Hannover Medical School</wicri:noRegion></affiliation></author><author><name sortKey="Bandmann, Oliver" sort="Bandmann, Oliver" uniqKey="Bandmann O" first="Oliver" last="Bandmann">Oliver Bandmann</name><affiliation wicri:level="1"><inist:fA14 i1="08"><s1>Academic Neurology Unit, University of Sheffield</s1><s2>Great Britain</s2><s3>GBR</s3><sZ>9 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Great Britain</wicri:noRegion></affiliation></author><author><name sortKey="Czlonkowska, Anna" sort="Czlonkowska, Anna" uniqKey="Czlonkowska A" first="Anna" last="Czlonkowska">Anna Czlonkowska</name><affiliation wicri:level="1"><inist: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></inist:fA14><country>Pologne</country><wicri:noRegion>Warsaw</wicri:noRegion></affiliation></author><author><name sortKey="Oertel, Wolfgang H" sort="Oertel, Wolfgang H" uniqKey="Oertel W" first="Wolfgang H." last="Oertel">Wolfgang H. Oertel</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Neurology, Philipps-University Marburg</s1><s3>DEU</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>11 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>Department of Neurology, Philipps-University Marburg</wicri:noRegion></affiliation></author><author><name sortKey="Hefter, Harald" sort="Hefter, Harald" uniqKey="Hefter H" first="Harald" last="Hefter">Harald Hefter</name><affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Department of Neurology, Heinrich-Heine-University Düsseldorf</s1><s3>DEU</s3><sZ>12 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Heinrich-Heine-University Düsseldorf</wicri:noRegion><wicri:noRegion>Department of Neurology, Heinrich-Heine-University Düsseldorf</wicri:noRegion></affiliation></author></analytic><series><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno><imprint><date when="2008">2008</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Copper</term><term>Evaluation</term><term>Evaluation scale</term><term>German</term><term>Human</term><term>Nervous system diseases</term><term>Treatment</term><term>Wilson disease</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Maladie de Wilson</term><term>Pathologie du système nerveux</term><term>Evaluation</term><term>Echelle d'évaluation</term><term>Allemand</term><term>Homme</term><term>Traitement</term><term>Cuivre</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</term><term>Cuivre</term></keywords></textClass></profileDesc></teiHeader><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></front></TEI><affiliations><list><country><li>Allemagne</li><li>Pologne</li><li>Royaume-Uni</li></country></list><tree><country name="Allemagne"><noRegion><name sortKey="Leinweber, Barbara" sort="Leinweber, Barbara" uniqKey="Leinweber B" first="Barbara" last="Leinweber">Barbara Leinweber</name></noRegion><name sortKey="Gunther, Peter" sort="Gunther, Peter" uniqKey="Gunther P" first="Peter" last="Günther">Peter Günther</name><name sortKey="Hefter, Harald" sort="Hefter, Harald" uniqKey="Hefter H" first="Harald" last="Hefter">Harald Hefter</name><name sortKey="Lang, Christoph J G" sort="Lang, Christoph J G" uniqKey="Lang C" first="Christoph J. G." last="Lang">Christoph J. G. Lang</name><name sortKey="Moller, J Carsten" sort="Moller, J Carsten" uniqKey="Moller J" first="J. Carsten" last="Möller">J. Carsten Möller</name><name sortKey="Oertel, Wolfgang H" sort="Oertel, Wolfgang H" uniqKey="Oertel W" first="Wolfgang H." last="Oertel">Wolfgang H. Oertel</name><name sortKey="Reuner, Ulrike" sort="Reuner, Ulrike" uniqKey="Reuner U" first="Ulrike" last="Reuner">Ulrike Reuner</name><name sortKey="Scherag, Andre" sort="Scherag, Andre" uniqKey="Scherag A" first="Andre" last="Scherag">Andre Scherag</name><name sortKey="Schmidt, Hartmut H J" sort="Schmidt, Hartmut H J" uniqKey="Schmidt H" first="Hartmut H. J." last="Schmidt">Hartmut H. J. Schmidt</name><name sortKey="Schrader, Christoph" sort="Schrader, Christoph" uniqKey="Schrader C" first="Christoph" last="Schrader">Christoph Schrader</name></country><country name="Royaume-Uni"><noRegion><name sortKey="Bandmann, Oliver" sort="Bandmann, Oliver" uniqKey="Bandmann O" first="Oliver" last="Bandmann">Oliver Bandmann</name></noRegion></country><country name="Pologne"><noRegion><name sortKey="Czlonkowska, Anna" sort="Czlonkowska, Anna" uniqKey="Czlonkowska A" first="Anna" last="Czlonkowska">Anna Czlonkowska</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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