Title |
Reliability and validity of the Wolfram Unified Rating Scale (WURS)
|
---|---|
Published in |
Orphanet Journal of Rare Diseases, November 2012
|
DOI | 10.1186/1750-1172-7-89 |
Pubmed ID | |
Authors |
Chau Nguyen, Erin R Foster, Alexander R Paciorkowski, Amy Viehoever, Colleen Considine, Aidena Bondurant, Bess A Marshall, Tamara Hershey, Washington University Wolfram Study Group |
Abstract |
Wolfram syndrome (WFS) is a rare, neurodegenerative disease that typically presents with childhood onset insulin dependent diabetes mellitus, followed by optic atrophy, diabetes insipidus, deafness, and neurological and psychiatric dysfunction. There is no cure for the disease, but recent advances in research have improved understanding of the disease course. Measuring disease severity and progression with reliable and validated tools is a prerequisite for clinical trials of any new intervention for neurodegenerative conditions. To this end, we developed the Wolfram Unified Rating Scale (WURS) to measure the severity and individual variability of WFS symptoms. The aim of this study is to develop and test the reliability and validity of the Wolfram Unified Rating Scale (WURS). |
X Demographics
Geographical breakdown
Country | Count | As % |
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Mexico | 1 | 20% |
Switzerland | 1 | 20% |
Unknown | 3 | 60% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 4 | 80% |
Scientists | 1 | 20% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Spain | 1 | 2% |
Unknown | 49 | 98% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Ph. D. Student | 11 | 22% |
Student > Bachelor | 5 | 10% |
Researcher | 4 | 8% |
Other | 3 | 6% |
Student > Master | 3 | 6% |
Other | 6 | 12% |
Unknown | 18 | 36% |
Readers by discipline | Count | As % |
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Medicine and Dentistry | 10 | 20% |
Nursing and Health Professions | 5 | 10% |
Psychology | 3 | 6% |
Biochemistry, Genetics and Molecular Biology | 3 | 6% |
Sports and Recreations | 2 | 4% |
Other | 6 | 12% |
Unknown | 21 | 42% |