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Published on: October 17, 2018
Ventilatory Responses During Submaximal Exercise in Children With Prader-Willi Syndrome
Adam M Hyde1, Robert G McMurray2, Frank A Chavoya1
11 California State University, Fullerton.
Insights
Children with Prader-Willi syndrome (PWS) show normal ventilatory responses during exercise, despite higher breathing rates. This suggests neural stimulation and metabolic cost may explain their hyperventilation.
Area of Science:
- Pediatric Pulmonology
- Neurogenetics
- Exercise Physiology
Background:
- Prader-Willi syndrome (PWS) is a genetic disorder characterized by hypothalamic dysfunction, obesity, and neurobehavioral issues.
- Individuals with PWS often exhibit hypoventilation and hypercapnia at rest.
- Understanding exercise-induced ventilatory control in PWS is crucial for managing respiratory health.
Purpose of the Study:
- To characterize and compare ventilatory responses during exercise in children with PWS versus control groups.
- To investigate potential differences in oxygen uptake, carbon dioxide output, ventilation, breathing frequency, and tidal volume during physical activity.
Main Methods:
- A study involving children aged 7-12 years with PWS (n=8) and without PWS (normal weight, n=9; obese, n=9).
- Participants completed three 5-minute exercise bouts at varying speeds (3.2, 4.0, 4.8 km/h).
- Continuous monitoring of respiratory parameters including oxygen uptake, carbon dioxide output, ventilation, breathing frequency, and tidal volume.
Main Results:
- Children with PWS demonstrated slightly higher oxygen uptake at 3.2 and 4.8 km/h compared to normal-weight controls.
- Ventilation and breathing frequency were significantly higher in the PWS group across all exercise intensities.
- Tidal volume and the ventilatory response relative to carbon dioxide output were similar between PWS and control groups.
Conclusions:
- Children with Prader-Willi syndrome do not exhibit impaired ventilatory responses during exercise.
- The observed hyperventilation in PWS during exercise may be linked to increased neural drive and metabolic demands.
- These findings contribute to a better understanding of respiratory physiology in PWS during physical activity.
Purpose:
Prader-Willi syndrome (PWS) is a genetic neurobehavioral disorder presenting hypothalamic dysfunction and adiposity. At rest, PWS exhibits hypoventilation with hypercapnia. We characterized ventilatory responses in children with PWS during exercise.
Methods:
Participants were children aged 7-12 years with PWS (n = 8) and without PWS with normal weight (NW; n = 9, body mass index ≤ 85th percentile) or obesity (n = 9, body mass index ≥ 95th percentile). Participants completed three 5-minute ambulatory bouts at 3.2, 4.0, and 4.8 km/h. Oxygen uptake, carbon dioxide output, ventilation, breathing frequency, and tidal volume were recorded.
Results:
PWS had slightly higher oxygen uptake (L/min) at 3.2 km/h [0.65 (0.46-1.01) vs 0.49 (0.34-0.83)] and at 4.8 km/h [0.89 (0.62-1.20) vs 0.63 (0.45-0.97)] than NW. PWS had higher ventilation (L/min) at 3.2 km/h [16.2 (13.0-26.5) vs 11.5 (8.4-17.5)], at 4.0 km/h [16.4 (13.9-27.9) vs 12.7 (10.3-19.5)], and at 4.8 km/h [19.7 (17.4-31.8) vs 15.2 (9.5-21.6)] than NW. PWS had greater breathing frequency (breaths/min) at 3.2 km/h [38 (29-53) vs 29 (22-35)], at 4.0 km/h [39 (29-58) vs 29 (23-39)], and at 4.8 km/h [39 (33-58) vs 32 (23-42)], but similar tidal volume and ventilation/carbon dioxide output to NW.
Conclusion:
PWS did not show impaired ventilatory responses to exercise. Hyperventilation in PWS may relate to excessive neural stimulation and metabolic cost.
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