Factors associated with lower forced vital capacity in children and adults with Duchenne muscular dystrophy using non-invasive ventilation: a multicenter analysis
Bibliographic record
Abstract
BACKGROUND: Reduced forced vital capacity (FVC) is associated with morbidity and mortality in individuals with Duchenne muscular dystrophy (DMD). Non-invasive ventilation (NIV) is often prescribed for the treatment of sleep-disordered breathing (SDB), and chronic respiratory insufficiency. Despite the common practice of initiating NIV later in the progression of DMD, the factors influencing FVC subsequent to the commencement of NIV remain unclear. OBJECTIVE: To evaluate the demographic, clinical and socioeconomic determinants of FVC% predicted across several cohorts of DMD children and adults prescribed NIV. METHODS: A multicenter retrospective review of individuals with DMD prescribed NIV was performed between February 2016 to October 2020. Patients were identified from three sites: The Hospital for Sick Children, Canada; Rady Children's Hospital San Diego, USA; and University of California San Diego Health, USA. Multivariate regression analysis was performed to determine factors that influence FVC. RESULTS: ) were included. Following multivariate analysis, a lower FVC% predicted was associated with older age (β = -1.44, p = 0.001), presence of scoliosis (β = -16.94, p = 0.002), absent deflazacort prescription (β = 14.43, p = 0.009), and use of in-ex sufflator (β = -39.73, p < 0.001), respectively. CONCLUSION: In our study, several factors were associated with reduced FVC% predicted in a DMD population using NIV. Future, prospective, longitudinal studies are imperative to comprehend the trajectory of FVC% predicted over time in individuals with DMD using NIV.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
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.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
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".