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Patients with hypermobile Ehlers-Danlos syndrome have respiratory and proprioceptive disorders. The aim of this study is to explore whether there is an alteration in lung volume perception in patients with hEDS compared to healthy subjects, and whether a cognitive task can influence ventilation control differently in subjects with hEDS than in healthy subjects.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Patients | Hypermobile Ehlers-Danlos patients (n=21) | ||
| Healthy subjects | Healthy subjects recruited from the clinic's staff (n=21) Age, Gender and BMI matched with hEDS patients |
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| Measure | Description | Time Frame |
|---|---|---|
| Accuracy of lung volume perception | The perception of lung volume is assessed using a device comprising a flow meter, an electronic acquisition card and an LCD screen. Subjects will be given 3 trials to train to perceive a target lung volume and then 3 trials without feedback to reproduce that target lung volume. The mean of the absolute deviations from the target volumes in percent is used as the primary outcome. | Baseline |
| Measure | Description | Time Frame |
|---|---|---|
| Minute ventilation with or without cognitive task | The minute ventilation will be calculated over 1 minute without cognitive task and then over 1 minute with cognitive task (trail making test part B). | Baseline |
| Respiratory rate variability |
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Inclusion Criteria (common):
Inclusion Criteria (hEDS Patients):
Inclusion Criteria (healthy subjects):
Exclusion Criteria (common):
Exclusion Criteria (hEDS Patients):
Exclusion Criteria (healthy subjects):
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Patients will be recruited through practitioners working with the clinic, who will propose participation in the study during consultations with patients diagnosed with EDS.
The recruitment of subjects for the control group will be carried out by internal communication within the Clinique de la Mitterie on a voluntary basis.
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| Name | Affiliation | Role |
|---|---|---|
| Cyrille Bergoin, MD | Clinique de la Mitterie | Principal Investigator |
| Patrick Mucci, Prof. | UREPSSS | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Clinique de la Mitterie | Lomme | 59160 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 38582758 | Derived | Hakimi A, Bergoin C, De Jesus A, Hermand E, Fabre C, Mucci P. Impairment of lung volume perception and breathing control in hypermobile Ehlers-Danlos syndrome. Sci Rep. 2024 Apr 6;14(1):8119. doi: 10.1038/s41598-024-58890-2. |
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| ID | Term |
|---|---|
| C536196 | Ehlers-Danlos syndrome type 3 |
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The time interval between each inspiratory peak will be recorded and will allow the calculation of the standard deviation and the root mean square with and without the cognitive task.
| Baseline |