Evaluation of Dyspnea With Exercise in Hypermobile Ehlers Danlos Syndrome and Generalized Hypermobility Spectrum Disorder
Bibliographic record
Abstract
Abstract Background/Rationale: Hypermobile Ehlers-Danlos Syndromes (hEDS, most common subtype) and Generalized Hypermobility Spectrum Disorder (G-HSD) are hereditary connective tissue disorders with multisystemic involvement. Individuals with hEDS/G-HSD commonly report exertional dyspnea, which has not been well characterized. Ventilatory and cardiac limitations during exercise may contribute to respiratory limitations. The study aimed to characterize respiratory symptoms and the cardiorespiratory response with graded exercise in people with hEDS/G-HSD. Methods: Baseline cross-sectional assessment of cardiopulmonary exercise testing (CPET) was undertaken in hEDS/G-HSD participants and age and sex matched controls without any cardiopulmonary conditions, as part of an ongoing trial (NCT 04972565). Participants had baseline characterization of respiratory symptoms (MRC dyspnea, 18-Qualitative Dyspnea Descriptors), Short Form-36, and self-reported physical activity assessed with the Godin Leisure-Time Exercise Questionnaire (GLTEQ). Participants underwent spirometry, lung volumes, and respiratory muscle strength measures and symptom-limited CPET on a cycle ergometer using a ramp protocol. Tidal flow volume curves and measures of cardiac autonomic function such as chronic response index ([peak heart rate (HR) – resting HR[asterisk]100]/([220-age]-[resting HR])) and HR recovery (HR CPET end – 1 minute post CPET) were evaluated and 18-Dyspnea Descriptors re-assessed post-CPET. T-tests, Wilcoxon and Fisher's exact test were used to compare the hEDS/G-HSD group with control participants. Results: 27 hEDS/G-HSD (30±8 years, 93% female, BMI: 25.3±4.6 kg/m2) and 17 healthy controls (29 ± 8 years, 88% female, BMI: 21.2±2.9 kg/m2) were evaluated. Participants with hEDS/G-HSD had lower SF-36 Physical Component Scores (33±9 vs. 58±6), GLTEQ (32±19 vs. 51±30), and greater MRC dyspnea (2.0±0.7 vs. 1.0±0), p < 0.05 for all comparisons. Spirometry, lung volumes and maximal inspiratory pressures were normal for both groups, Table 1. hEDS/G/HSD participants had a lower aerobic capacity (86±19% vs. 103±27% predicted, p< 0.001), reduced chronotropic response index (79±15 vs. 89±8 %, p=0.045), but no difference in peak minute ventilation (58±14 vs. 62±16%, p=0.32), change in inspiratory capacity or peak workload achieved (Table 1). Borg dyspnea and leg fatigue were moderate to severe in both groups upon CPET completion. The top three dyspnea descriptors pre- and post-CPET are described in Table 1 with hEDS/G-HSD reporting a greater number of dyspnea limitations. Conclusion: hEDS/G-HSD participants exhibited a lower aerobic capacity and chronotropic response index with a trend towards greater proportion of distressing dyspnea descriptors with exercise, despite preservation of their breathing reserve. Future work aims to explore other contributors to dyspnea such as cardiac autonomic dysfunction, thoracic musculoskeletal changes, and multidimensional perception of dyspnea.
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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 | 0.001 | 0.001 |
| 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 | 0.001 |
| 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".