Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German patients with treated Wilson's disease.
Identifieur interne : 001E70 ( Ncbi/Checkpoint ); précédent : 001E69; suivant : 001E71Evaluation of the Unified Wilson's Disease Rating Scale (UWDRS) in German patients with treated Wilson's disease.
Auteurs : Barbara Leinweber [Allemagne] ; 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 HefterSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2008.
Descripteurs français
- Wicri :
- geographic : Allemagne.
English descriptors
- KwdEn :
- Adult, Age of Onset, Antirheumatic Agents (therapeutic use), Chelating Agents (therapeutic use), Drug Therapy, Combination, Female, Germany (epidemiology), Hepatolenticular Degeneration (diagnosis), Hepatolenticular Degeneration (drug therapy), Hepatolenticular Degeneration (ethnology), Humans, Liver Diseases (diagnosis), Liver Diseases (epidemiology), Lower Extremity (physiopathology), Male, Penicillamine (therapeutic use), Posture (physiology), Prevalence, Questionnaires, Torticollis (diagnosis), Torticollis (epidemiology), Torticollis (physiopathology), Tremor (epidemiology), Tremor (physiopathology), Trientine (therapeutic use), Upper Extremity (physiopathology), Zinc (therapeutic use).
- MESH :
- chemical , therapeutic use : Antirheumatic Agents, Chelating Agents, Penicillamine, Trientine, Zinc.
- geographic , epidemiology : Germany.
- diagnosis : Hepatolenticular Degeneration, Liver Diseases, Torticollis.
- drug therapy : Hepatolenticular Degeneration.
- epidemiology : Liver Diseases, Torticollis, Tremor.
- ethnology : Hepatolenticular Degeneration.
- physiology : Posture.
- physiopathology : Lower Extremity, Torticollis, Tremor, Upper Extremity.
- Adult, Age of Onset, Drug Therapy, Combination, Female, Humans, Male, Prevalence, Questionnaires.
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 rho approximately 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.
DOI: 10.1002/mds.21761
PubMed: 17960799
Affiliations:
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Lang</name></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></author><author><name sortKey="Schrader, Christoph" sort="Schrader, Christoph" uniqKey="Schrader C" first="Christoph" last="Schrader">Christoph Schrader</name></author><author><name sortKey="Bandmann, Oliver" sort="Bandmann, Oliver" uniqKey="Bandmann O" first="Oliver" last="Bandmann">Oliver Bandmann</name></author><author><name sortKey="Czlonkowska, Anna" sort="Czlonkowska, Anna" uniqKey="Czlonkowska A" first="Anna" last="Czlonkowska">Anna Czlonkowska</name></author><author><name sortKey="Oertel, Wolfgang H" sort="Oertel, Wolfgang H" uniqKey="Oertel W" first="Wolfgang H" last="Oertel">Wolfgang H. Oertel</name></author><author><name sortKey="Hefter, Harald" sort="Hefter, Harald" uniqKey="Hefter H" first="Harald" last="Hefter">Harald Hefter</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2008">2008</date><idno type="doi">10.1002/mds.21761</idno><idno type="RBID">pubmed:17960799</idno><idno type="pmid">17960799</idno><idno type="wicri:Area/PubMed/Corpus">002489</idno><idno type="wicri:Area/PubMed/Curation">002489</idno><idno type="wicri:Area/PubMed/Checkpoint">002306</idno><idno type="wicri:Area/Ncbi/Merge">001E70</idno><idno type="wicri:Area/Ncbi/Curation">001E70</idno><idno type="wicri:Area/Ncbi/Checkpoint">001E70</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">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"><nlm:affiliation>Department of Neurology, Philipps-University Marburg, Germany. bleinweb@med.uni-marburg.de</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Philipps-University Marburg</wicri:regionArea><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>Philipps-University Marburg</wicri:noRegion><wicri:noRegion>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></author><author><name sortKey="Scherag, Andre" sort="Scherag, Andre" uniqKey="Scherag A" first="Andre" last="Scherag">Andre Scherag</name></author><author><name sortKey="Reuner, Ulrike" sort="Reuner, Ulrike" uniqKey="Reuner U" first="Ulrike" last="Reuner">Ulrike Reuner</name></author><author><name sortKey="Gunther, Peter" sort="Gunther, Peter" uniqKey="Gunther P" first="Peter" last="Günther">Peter Günther</name></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></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></author><author><name sortKey="Schrader, Christoph" sort="Schrader, Christoph" uniqKey="Schrader C" first="Christoph" last="Schrader">Christoph Schrader</name></author><author><name sortKey="Bandmann, Oliver" sort="Bandmann, Oliver" uniqKey="Bandmann O" first="Oliver" last="Bandmann">Oliver Bandmann</name></author><author><name sortKey="Czlonkowska, Anna" sort="Czlonkowska, Anna" uniqKey="Czlonkowska A" first="Anna" last="Czlonkowska">Anna Czlonkowska</name></author><author><name sortKey="Oertel, Wolfgang H" sort="Oertel, Wolfgang H" uniqKey="Oertel W" first="Wolfgang H" last="Oertel">Wolfgang H. Oertel</name></author><author><name sortKey="Hefter, Harald" sort="Hefter, Harald" uniqKey="Hefter H" first="Harald" last="Hefter">Harald Hefter</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="eISSN">1531-8257</idno><imprint><date when="2008" type="published">2008</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Age of Onset</term><term>Antirheumatic Agents (therapeutic use)</term><term>Chelating Agents (therapeutic use)</term><term>Drug Therapy, Combination</term><term>Female</term><term>Germany (epidemiology)</term><term>Hepatolenticular Degeneration (diagnosis)</term><term>Hepatolenticular Degeneration (drug therapy)</term><term>Hepatolenticular Degeneration (ethnology)</term><term>Humans</term><term>Liver Diseases (diagnosis)</term><term>Liver Diseases (epidemiology)</term><term>Lower Extremity (physiopathology)</term><term>Male</term><term>Penicillamine (therapeutic use)</term><term>Posture (physiology)</term><term>Prevalence</term><term>Questionnaires</term><term>Torticollis (diagnosis)</term><term>Torticollis (epidemiology)</term><term>Torticollis (physiopathology)</term><term>Tremor (epidemiology)</term><term>Tremor (physiopathology)</term><term>Trientine (therapeutic use)</term><term>Upper Extremity (physiopathology)</term><term>Zinc (therapeutic use)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Antirheumatic Agents</term><term>Chelating Agents</term><term>Penicillamine</term><term>Trientine</term><term>Zinc</term></keywords><keywords scheme="MESH" type="geographic" qualifier="epidemiology" xml:lang="en"><term>Germany</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Hepatolenticular Degeneration</term><term>Liver Diseases</term><term>Torticollis</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Hepatolenticular Degeneration</term></keywords><keywords scheme="MESH" qualifier="epidemiology" xml:lang="en"><term>Liver Diseases</term><term>Torticollis</term><term>Tremor</term></keywords><keywords scheme="MESH" qualifier="ethnology" xml:lang="en"><term>Hepatolenticular Degeneration</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Posture</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Lower Extremity</term><term>Torticollis</term><term>Tremor</term><term>Upper Extremity</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Age of Onset</term><term>Drug Therapy, Combination</term><term>Female</term><term>Humans</term><term>Male</term><term>Prevalence</term><term>Questionnaires</term></keywords><keywords scheme="Wicri" type="geographic" xml:lang="fr"><term>Allemagne</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(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 rho approximately 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.</div></front></TEI><affiliations><list><country><li>Allemagne</li></country></list><tree><noCountry><name sortKey="Bandmann, Oliver" sort="Bandmann, Oliver" uniqKey="Bandmann O" first="Oliver" last="Bandmann">Oliver Bandmann</name><name sortKey="Czlonkowska, Anna" sort="Czlonkowska, Anna" uniqKey="Czlonkowska A" first="Anna" last="Czlonkowska">Anna Czlonkowska</name><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></noCountry><country name="Allemagne"><noRegion><name sortKey="Leinweber, Barbara" sort="Leinweber, Barbara" uniqKey="Leinweber B" first="Barbara" last="Leinweber">Barbara Leinweber</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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