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Record W4214598407 · doi:10.1016/j.nmd.2022.02.012

Functional outcome measures in young, steroid-naïve boys with Duchenne muscular dystrophy

2022· article· en· W4214598407 on OpenAlexafffundabout
Anna Mayhew, D. Moat, Michael McDermott, Michelle Eagle, Robert C. Griggs, Wendy King, M. James, Robert Muni‐Lofra, Alison Shillington, Sarah Gregson, Lindsey Pallant, Christy Skura, Loretta A. Staudt, Katy Eichinger, Heather McMurchie, Rosanna Rabb, Marina Di Marco, Sarah M. Brown, Riccardo Zanin, Maria Teresa Arnoldi, Melissa McIntyre, Amelia Wilson, Lindsay N. Alfano, Linda Lowes, Colleen Blomgren, Evelin Milev, Mario Iodice, Amy Pasternak, Angela W. Chiu, Ilka Lehnert, Nicole Claus, Kathy Dieruf, Enrica Rolle, Alina Nicorici, Bárbara Andres, Elke Hobbiebrunken, Gerda Roetmann, Victoria Kern, Matthew Civitello, Sibylle Emilie Vogt, Melissa J. Hayes, Cheryl Scholtes, Catherine Lacroix, Tara Gunn, Sinead Warner, Jennifer Newman, Andrea Barp, Katherine Kundrat, Staci Kovelman, Penny Powers, Michela Guglieri

Bibliographic record

VenueNeuromuscular Disorders · 2022
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicMuscle Physiology and Disorders
Canadian institutionsChildren's Hospital of Eastern OntarioUniversity of OttawaLondon Health Sciences CentreAlberta Children's Hospital
FundersNational Institutes of HealthItalfarmacoVoyager TherapeuticsAmicus TherapeuticsPTC TherapeuticsMuscular Dystrophy CanadaNational Institute of Neurological Disorders and StrokeCharcot-Marie-Tooth AssociationFSH SocietyCure SMAMuscular Dystrophy AssociationNeurocrine BiosciencesFibroGenFondazione TelethonAction DuchenneAstraZenecaParent Project Muscular DystrophyAveXisPfizerBiogenAcceleronAmerican Brain FoundationEli Lilly and CompanySarepta TherapeuticsJain FoundationFoundation for the National Institutes of Health
KeywordsDuchenne muscular dystrophyAmbulatoryMedicinePhysical therapyPopulationPhysical medicine and rehabilitationInternal medicine

Abstract

fetched live from OpenAlex

The purpose of this study was to quantitate motor performance in 196 genetically confirmed steroid-naïve boys with Duchenne muscular dystrophy (DMD), to evaluate the test-retest reliability of measures of motor performance in young DMD boys, and to assess correlations among the different functional outcomes including timed tests. Boys aged 4-7 years were recruited in the FOR-DMD study, a comparative effectiveness study of different steroid regimens in DMD. Eligible boys had to be able to rise from the floor independently and to perform pulmonary function testing consistently. The boys were evaluated with standardized assessments at the screening and baseline visits at 32 sites in 5 countries (US, UK, Canada, Italy, Germany). Assessments included timed rise from floor, timed 10 m walk/run, six-minute walk distance, North Star Ambulatory Assessment (NSAA) and forced vital capacity (FVC). Mean age at baseline was 5.9 years (range 4.1-8.1 years). Test-retest reliability was high for functional assessments, regardless of time lag between assessments (up to 90 days) and for the majority of age groups. Correlations were strong among the functional measures and timed tests, less so with FVC. Physiotherapy measures are reliable in a young, steroid-naïve population and rise from floor velocity appears to be a sensitive measure of strength in this population.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.003
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.004
Threshold uncertainty score0.007

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0010.003
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0010.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0010.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.011
GPT teacher head0.213
Teacher spread0.203 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations9
Published2022
Admission routes3
Has abstractyes

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