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Background: Combined effects of leaks, mechanical property of respiratory system and upper airway (UA) patency on patient-ventilator synchrony (PVA) and the level of clinically "tolerable" leaks are not well established in home ventilators.Methods: We comparatively assessed on a bench model, the highest leak level tolerated without inducing significant asynchrony ("critical leak") in three home ventilators (Astral 150, Trilogy 100 and Vivo 60; noted as A150, T100 and V60 respectively) subjected to three simulated diseased respiratory conditions: chronic obstructive pulmonary disease (COPD), obesity hypoventilation (OHS) and neuromuscular disorders (NMD), with both open and closed UA. Also, total leak values in the device reports were compared to the bench-measured values.Results: With open UA, all ventilators were able to avoid asynchrony up to a 30 L/min leak and even to 55 L/min in some cases. UA closure and respiratory diseases especially OHS influenced PVA. With closed UA, the critical leak of A150 and T100 remained higher than 55 L/min in COPD and OHS, while for V60 decreased to 41 and 33 L/min respectively. In NMD with closed UA, only T100 reached a high critical leak of 69 L/min. Besides, inspiratory trigger sensitivity change was often necessary to avoid PVA.Conclusions: Home ventilators were able to avoid PVA in high-level leak conditions. However, asynchrony appeared in cases of abnormal mechanical properties of respiratory system or closed UA. In case of closed UA, the EPAP should be adjusted prior to the inspiratory trigger.Trial Registration: Not applicable. [ABSTRACT FROM AUTHOR]

Background: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disease due to PHOX2B mutations. CCHS patients suffer from many autonomic disorders, dominated clinically by defective ventilatory automatisms. From birth, the life of CCHS patients depends on ventilatory support during sleep, involving a high burden of care. Whether or not this impairs the quality of life of these patients during adulthood remains unknown. Methods: We applied the medical outcome study short form-36 (SF-36) to 12 CCHS patients aged 15-33 (9 women) at the time of their passage from pediatric to adult care. Scores for the SF-36 dimensions were compared to the age- and gender-matched French reference population after transformation into standardized Z-scores. The SF-36 physical component summary score (PCS) and mental component summary score (MCS) were compared to American reference values. Results: Median Z-scores were significantly different from zerofor PF (physical functioning, p = 0.020) and GH (general health perception, p = 0.0342) and for PCS (p = 0.020). The other physical dimensions (RP, role limitation due to physical function; BP, bodily pain) and the mental dimensions (VT, vitality; SF, social functioning; RE, role limitation due to emotional function; MH, mental health) and MCS were not altered. Conclusions: We conclude that, despite the physical constraints imposed by CCHS and its anxiogenic nature, this disease is associated with an impairment of health-related quality of life in young adults that remains moderate. Whatever the underlying explanations, these results convey hope to parents with a child diagnosed with CCHS and for patients themselves. [ABSTRACT FROM AUTHOR]

Background: Combined effects of leaks, mechanical property of respiratory system and upper airway (UA) patency on patient-ventilator synchrony (PVA) and the level of clinically "tolerable" leaks are not well established in home ventilators., Methods: We comparatively assessed on a bench model, the highest leak level tolerated without inducing significant asynchrony ("critical leak") in three home ventilators (Astral 150, Trilogy 100 and Vivo 60; noted as A150, T100 and V60 respectively) subjected to three simulated diseased respiratory conditions: chronic obstructive pulmonary disease (COPD), obesity hypoventilation (OHS) and neuromuscular disorders (NMD), with both open and closed UA. Also, total leak values in the device reports were compared to the bench-measured values., Results: With open UA, all ventilators were able to avoid asynchrony up to a 30 L/min leak and even to 55 L/min in some cases. UA closure and respiratory diseases especially OHS influenced PVA. With closed UA, the critical leak of A150 and T100 remained higher than 55 L/min in COPD and OHS, while for V60 decreased to 41 and 33 L/min respectively. In NMD with closed UA, only T100 reached a high critical leak of 69 L/min. Besides, inspiratory trigger sensitivity change was often necessary to avoid PVA., Conclusions: Home ventilators were able to avoid PVA in high-level leak conditions. However, asynchrony appeared in cases of abnormal mechanical properties of respiratory system or closed UA. In case of closed UA, the EPAP should be adjusted prior to the inspiratory trigger., Trial Registration: Not applicable.