A novel Global Assessment Scale for Wilson's Disease (GAS for WD).
Identifieur interne : 001F81 ( PubMed/Checkpoint ); précédent : 001F80; suivant : 001F82A novel Global Assessment Scale for Wilson's Disease (GAS for WD).
Auteurs : Annu Aggarwal [Inde] ; Nitin Aggarwal ; Aabha Nagral ; Govindji Jankharia ; Mohit BhattSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2009.
English descriptors
- KwdEn :
- Adolescent, Adult, Behavioral Symptoms (etiology), Child, Cognition Disorders (etiology), Disability Evaluation, Female, Guidelines as Topic, Hepatolenticular Degeneration (diagnosis), Hepatolenticular Degeneration (physiopathology), Hepatolenticular Degeneration (psychology), Humans, Longitudinal Studies, Male, Motor Activity (physiology), Neurologic Examination (methods), Quality of Life, Reproducibility of Results, Retrospective Studies, Severity of Illness Index, Young Adult.
- 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 alpha = 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.
DOI: 10.1002/mds.22231
PubMed: 19115420
Affiliations:
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sort="Jankharia, Govindji" uniqKey="Jankharia G" first="Govindji" last="Jankharia">Govindji Jankharia</name></author><author><name sortKey="Bhatt, Mohit" sort="Bhatt, Mohit" uniqKey="Bhatt M" first="Mohit" last="Bhatt">Mohit Bhatt</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2009">2009</date><idno type="doi">10.1002/mds.22231</idno><idno type="RBID">pubmed:19115420</idno><idno type="pmid">19115420</idno><idno type="wicri:Area/PubMed/Corpus">001E95</idno><idno type="wicri:Area/PubMed/Curation">001E95</idno><idno type="wicri:Area/PubMed/Checkpoint">001F81</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">A novel Global Assessment Scale for Wilson's Disease (GAS for WD).</title><author><name sortKey="Aggarwal, Annu" sort="Aggarwal, Annu" uniqKey="Aggarwal A" first="Annu" last="Aggarwal">Annu Aggarwal</name><affiliation wicri:level="1"><nlm:affiliation>Movement Disorder Clinic, Department of Neurology, Jaslok 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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></textClass></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 alpha = 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.</div></front></TEI><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">19115420</PMID><DateCreated><Year>2009</Year><Month>05</Month><Day>11</Day></DateCreated><DateCompleted><Year>2009</Year><Month>07</Month><Day>28</Day></DateCompleted><Article PubModel="Print"><Journal><ISSN IssnType="Electronic">1531-8257</ISSN><JournalIssue CitedMedium="Internet"><Volume>24</Volume><Issue>4</Issue><PubDate><Year>2009</Year><Month>Mar</Month><Day>15</Day></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>A novel Global Assessment Scale for Wilson's Disease (GAS for WD).</ArticleTitle><Pagination><MedlinePgn>509-18</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1002/mds.22231</ELocationID><Abstract><AbstractText>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 alpha = 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. 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UI="D002648">Child</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D003072">Cognition Disorders</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000209">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="Y" UI="D004185">Disability Evaluation</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005260">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D017408">Guidelines as Topic</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006527">Hepatolenticular Degeneration</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000523">psychology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006801">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008137">Longitudinal Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008297">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009043">Motor Activity</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009460">Neurologic Examination</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000379">methods</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D011788">Quality of Life</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D015203">Reproducibility of Results</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D012189">Retrospective Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="Y" UI="D012720">Severity of Illness Index</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D055815">Young Adult</DescriptorName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="entrez"><Year>2008</Year><Month>12</Month><Day>31</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2008</Year><Month>12</Month><Day>31</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2009</Year><Month>7</Month><Day>29</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="doi">10.1002/mds.22231</ArticleId><ArticleId IdType="pubmed">19115420</ArticleId></ArticleIdList></PubmedData></pubmed><affiliations><list><country><li>Inde</li></country></list><tree><noCountry><name sortKey="Aggarwal, Nitin" sort="Aggarwal, Nitin" uniqKey="Aggarwal N" first="Nitin" last="Aggarwal">Nitin Aggarwal</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></noCountry><country name="Inde"><noRegion><name sortKey="Aggarwal, Annu" sort="Aggarwal, Annu" uniqKey="Aggarwal A" first="Annu" last="Aggarwal">Annu Aggarwal</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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