A novel Global Assessment Scale for Wilson's Disease (GAS for WD)
Identifieur interne : 002478 ( Main/Exploration ); précédent : 002477; suivant : 002479A novel Global Assessment Scale for Wilson's Disease (GAS for WD)
Auteurs : Annu Aggarwal [Inde] ; Nitin Aggarwal [États-Unis] ; Aabha Nagral [Inde] ; Govindji Jankharia [Inde] ; Mohit Bhatt [Inde]Source :
- Movement Disorders [ 0885-3185 ] ; 2009-03-15.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Cuivre.
English descriptors
- KwdEn :
- Adolescent, Adult, Behavioral Symptoms (etiology), Child, Cognition Disorders (etiology), Copper, Disability, Disability Evaluation, Female, Guidelines as Topic, Hepatolenticular Degeneration (diagnosis), Hepatolenticular Degeneration (physiopathology), Hepatolenticular Degeneration (psychology), Humans, Longitudinal Studies, Male, Motor Activity (physiology), Nervous system diseases, Neurologic Examination (methods), Quality of Life, Reliability, Reproducibility of Results, Retrospective Studies, Severity of Illness Index, Validation, Wilson disease, Wilson's disease, Young Adult, disability scale, reliability, responsiveness, validation.
- MESH :
- diagnosis : Hepatolenticular Degeneration.
- etiology : Behavioral Symptoms, Cognition Disorders.
- methods : Neurologic Examination.
- physiology : Motor Activity.
- physiopathology : Hepatolenticular Degeneration.
- psychology : Hepatolenticular Degeneration.
- Adolescent, Adult, Child, Disability Evaluation, Female, Guidelines as Topic, Humans, Longitudinal Studies, Male, Quality of Life, Reproducibility of Results, Retrospective Studies, Severity of Illness Index, Young Adult.
Abstract
Wilson's disease (WD) is an inherited disorder of copper metabolism. Despite being treatable, patients with WD suffer severe disabilities due to delay in initiation and difficulty in monitoring treatment. We propose a two tier, Global Assessment Scale for Wilson's Disease (GAS for WD) that grades the multisystemic manifestations of the disease. Tier 1 scores the global disability in four domains: Liver, Cognition and behavior, Motor, and Osseomuscular. Tier 2 is multidimensional scale for a fine grained evaluation of the neurological dysfunction. We prospectively validated this scale in 30 patients with WD. Both tiers had a high inter‐rater reliability (Intraclass correlation coefficient ICC (A, 2) = 0.96–1.0). Tier 2 items were internally consistent (Cronbach's α = 0.89) and factorial analysis showed that 90.3% of the Tier 2 total score variance was determined by seven factors. Scores of both tiers were commensurate with the disease burden as assessed by standard disability scales (Child Pugh, UPDRS, SS3, and CGI) and satisfied criteria for validity. Longitudinal follow‐up over 1.5 years showed that the scale was sensitive to clinical change. This suggests that GAS for WD is a practical tool with potential applications in management of patients, and in testing and comparison of treatment regimens. © 2008 Movement Disorder Society
Url:
DOI: 10.1002/mds.22231
Affiliations:
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Le document en format XML
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Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2009-03-15">2009-03-15</date><biblScope unit="vol">24</biblScope><biblScope unit="issue">4</biblScope><biblScope unit="page" from="509">509</biblScope><biblScope unit="page" to="518">518</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">F1B6AC8B2D8E0CBDDC07230D4BEA24E86F65AC76</idno><idno type="DOI">10.1002/mds.22231</idno><idno type="ArticleID">MDS22231</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Behavioral Symptoms (etiology)</term><term>Child</term><term>Cognition Disorders (etiology)</term><term>Copper</term><term>Disability</term><term>Disability Evaluation</term><term>Female</term><term>Guidelines as Topic</term><term>Hepatolenticular Degeneration (diagnosis)</term><term>Hepatolenticular Degeneration (physiopathology)</term><term>Hepatolenticular Degeneration (psychology)</term><term>Humans</term><term>Longitudinal Studies</term><term>Male</term><term>Motor Activity (physiology)</term><term>Nervous system diseases</term><term>Neurologic Examination (methods)</term><term>Quality of Life</term><term>Reliability</term><term>Reproducibility of Results</term><term>Retrospective Studies</term><term>Severity of Illness Index</term><term>Validation</term><term>Wilson disease</term><term>Wilson's disease</term><term>Young Adult</term><term>disability scale</term><term>reliability</term><term>responsiveness</term><term>validation</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Hepatolenticular Degeneration</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Behavioral Symptoms</term><term>Cognition Disorders</term></keywords><keywords scheme="MESH" qualifier="methods" xml:lang="en"><term>Neurologic Examination</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Motor Activity</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Hepatolenticular Degeneration</term></keywords><keywords scheme="MESH" qualifier="psychology" xml:lang="en"><term>Hepatolenticular Degeneration</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Child</term><term>Disability Evaluation</term><term>Female</term><term>Guidelines as Topic</term><term>Humans</term><term>Longitudinal Studies</term><term>Male</term><term>Quality of Life</term><term>Reproducibility of Results</term><term>Retrospective Studies</term><term>Severity of Illness Index</term><term>Young Adult</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Cuivre</term><term>Fiabilité</term><term>Incapacité</term><term>Maladie de Wilson</term><term>Pathologie du système nerveux</term><term>Validation</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Cuivre</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Wilson's disease (WD) is an inherited disorder of copper metabolism. Despite being treatable, patients with WD suffer severe disabilities due to delay in initiation and difficulty in monitoring treatment. We propose a two tier, Global Assessment Scale for Wilson's Disease (GAS for WD) that grades the multisystemic manifestations of the disease. Tier 1 scores the global disability in four domains: Liver, Cognition and behavior, Motor, and Osseomuscular. Tier 2 is multidimensional scale for a fine grained evaluation of the neurological dysfunction. We prospectively validated this scale in 30 patients with WD. Both tiers had a high inter‐rater reliability (Intraclass correlation coefficient ICC (A, 2) = 0.96–1.0). Tier 2 items were internally consistent (Cronbach's α = 0.89) and factorial analysis showed that 90.3% of the Tier 2 total score variance was determined by seven factors. Scores of both tiers were commensurate with the disease burden as assessed by standard disability scales (Child Pugh, UPDRS, SS3, and CGI) and satisfied criteria for validity. Longitudinal follow‐up over 1.5 years showed that the scale was sensitive to clinical change. This suggests that GAS for WD is a practical tool with potential applications in management of patients, and in testing and comparison of treatment regimens. © 2008 Movement Disorder Society</div></front></TEI><affiliations><list><country><li>Inde</li><li>États-Unis</li></country><region><li>Illinois</li></region></list><tree><country name="Inde"><noRegion><name sortKey="Aggarwal, Annu" sort="Aggarwal, Annu" uniqKey="Aggarwal A" first="Annu" last="Aggarwal">Annu Aggarwal</name></noRegion><name sortKey="Bhatt, Mohit" sort="Bhatt, Mohit" uniqKey="Bhatt M" first="Mohit" last="Bhatt">Mohit Bhatt</name><name sortKey="Bhatt, Mohit" sort="Bhatt, Mohit" uniqKey="Bhatt M" first="Mohit" last="Bhatt">Mohit Bhatt</name><name sortKey="Jankharia, Govindji" sort="Jankharia, Govindji" uniqKey="Jankharia G" first="Govindji" last="Jankharia">Govindji Jankharia</name><name sortKey="Nagral, Aabha" sort="Nagral, Aabha" uniqKey="Nagral A" first="Aabha" last="Nagral">Aabha Nagral</name></country><country name="États-Unis"><region name="Illinois"><name sortKey="Aggarwal, Nitin" sort="Aggarwal, Nitin" uniqKey="Aggarwal N" first="Nitin" last="Aggarwal">Nitin Aggarwal</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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