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Purpose: To identify specific determinants of non-adherence or cessation of continuous positive airway pressure (CPAP) therapy in a population of patients with spinal cord injuries (SCI)., Methods: Retrospective analysis of data from patients with SCI who underwent a full night supervised polysomnography between 2015 and 2021 and presented with moderate to severe obstructive sleep apnea (OSA) and for whom CPAP was indicated. Adherence was studied at 1, 6, and 12 months. Univariate and multivariate analyses were performed to identify factors associated with non-adherence (< 4 h per night or CPAP cessation). Factors studied were demographic and disease-related data and both subjective and objective sleep parameters., Results: A total of 60 patients were included (40% cervical SCI). In univariate analysis, the only predictive parameters of non-adherence observed at 1, 6, and 12 months were the average use of CPAP on the 1st night (p = 0.02) and over the 1st week (p ≤ 0.001). A complete lesion (AIS-A) was predictive of non-adherence at 1 and 6 months (p = 0.02 at 6 months), while mask leakage was associated with non-adherence at 12 months (p = 0.02). Upper limb autonomy and the presence of family caregivers did not appear to be protective. In multivariate analysis, only the average use in the first week remained predictive of adherence (> 4 h) in the short, medium and long term., Conclusion: In patients with SCI and OSA, the 1st week of CPAP treatment seems to be determinant of short-, medium-, and long-term CPAP adherence. Support for SCI patients from the start of treatment is essential and may help avoid treatment failures., (© 2023. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Background and Objective: Around 25% of patients with neuro-muscular diseases (NMD) are treated by home noninvasive ventilation (NIV) through an oronasal mask. However, there is growing evidence that nasal masks require lower NIV pressures and result in fewer residual obstructive events. We hypothesized that nasal masks would improve efficacy and reduce side effects compared to oronasal masks in this population., Methods: open label, cross-over, randomized, study in 2 tertiary care hospitals. Patients with NMD treated by home NIV were randomized for one-week periods to nasal and oronasal interfaces respectively (cross-over). At the end of each period, nocturnal polygraphy (monitoring mouth opening) under NIV, synchronized with transcutaneous partial pressure in CO 2 (tcCO 2 ) was performed. Data were collected from the NIV built-in software and NIV side-effects were collected. Intention-to-treat and per protocol analyses were performed. The primary outcome was mean nocturnal SpO 2 . The secondary outcomes were: percentage of sleep with SpO 2 <90%, oxygen desaturation index (ODI), mean tcCO 2 , mean duration of mouth opening during sleep, level of non-intentional leaks and side-effects., Results: Thirty patients with NMD were included. There were no between-group differences for either the primary or secondary outcomes. Post hoc comparisons showed that changing between interfaces reduced NIV efficacy: mean nocturnal SpO 2 (p=0.04), ODI (p=0.01), mean tcCO2 (p=0.048), side-effects (p=0.008)., Conclusion: Nasal masks did not improve NIV efficacy or reduce side effects compared to oronasal masks in patients with NMD treated by home NIV. The efficacy of NIV is reduced during the transition to another interface, requiring close monitoring. Registration number: NCT03458507., (Copyright © 2020 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Polysomnography (PSG) is the gold standard for the analysis of sleep architecture but is not always available in routine practice, as it is time consuming and cumbersome for patients. Bispectral index (BIS), developed to quantify the deepness of general anesthesia, may be used as a simplified tool to evaluate natural sleep depth. We objectively recorded sleep architecture in young patients using the latest BIS Vista monitor and correlated BIS values with PSG sleep stages in order to determine BIS thresholds. Patients, referred for the screening of sleep apnea/hypopnea syndrome or differential diagnosis of hypersomnia were recruited. Overnight PSG and BIS were performed simultaneously. BIS values were averaged for each sleep stage. Pre-sleep wakefulness (W) and wake after sleep onset (WASO) were also differentiated. BIS values were discarded for a signal quality index <90 %. ROC curves were plotted to discriminate sleep stages from each other. Twelve patients (5.7-29.3 years old) were included. Mean BIS values were 83 ± 8, 76 ± 12, 77 ± 11, 70 ± 10, 43 ± 10, and 75 ± 10 for W, WASO, N1, N2, N3 and R (REM) stages, respectively. BIS failed to distinguish W, WASO, N1 and R stages. BIS threshold that identified stage N2 was <73 (AUC = 0.784, p < 0.001) with low sensitivity (75 %) and poor specificity (64 %). BIS threshold that identified stage N3 was <55 (AUC = 0.964, p < 0.001) with an 87 %-sensitivity and a 93 %-specificity. BIS identified stage N3 with satisfactory sensitivity and specificity but is limited by its inability to distinguish REM sleep from wake. Further studies combining BIS with chin electromyogram and/or electrooculogram could be of interest.

Background and Aims: Even though numerous ventilators are licensed for a use in children, very few have been specifically developed for this age range. Therefore, home ventilators may not be able to adequately synchronize with the child's respiratory effort, and the inspiratory triggers (ITs) of assist modes are not always appropriate for children. The aim of the study was to test the improvement of the IT of a ventilator on a pediatric bench and in pediatric patients., Methods: A classical IT (ITc) and an improved IT [non-invasive ventilation (NIV) + IT] were tested on a bench with six pediatric profiles and in six young patients (mean age 14.1 ± 2.7 years old) requiring long-term NIV., Results: On the bench, trigger time delays (ΔT) and trigger pressures (ΔP) were reduced with the NIV + IT as compared with the ITc (ΔT: 0.481 ± 0.332 vs 0.079 ± 0.022 s for ITc and NIV + IT, respectively, P = 0.027; ΔP: -1.40 ± 0.70 vs -0.42 ± 0.28 cmH2 O for ITc and NIV + IT, respectively, P = 0.046). The clinical study confirmed the decrease in ΔT (0.267 ± 0.061 vs 0.178 ± 0.074 s for ITc and NIV + IT, respectively, P = 0.024) and ΔP (-0.68 ± 0.26 vs -0.39 ± 0.11 cmH2 O for ITc and NIV + IT, respectively, P = 0.030)., Conclusions: The sensitivity of the IT of a ventilator can be improved for pediatric use. The improvements observed on the bench study were confirmed in pediatric patients., (© 2014 John Wiley & Sons Ltd.)

Introduction: Long term noninvasive continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV) are increasingly used in children but limited information is available on the criteria and conditions leading to the initiation of these treatments. The aim of the study is to describe the objective overnight respiratory parameters and clinical situations that led to the initiation of CPAP/NIV in a pediatric NIV unit., Material and Methods: Retrospective analysis of the data of all the children discharged on home CPAP/NIV over a 1 year period., Results: Seventy-six patients were started on CPAP (n = 64) or NIV (n = 12). CPAP/NIV was initiated because of CPAP/NIV weaning failure (Acute group) in 15 patients. None of these patients had an overnight gas exchange or sleep study before CPAP/NIV initiation. In 18 patients, CPAP/NIV was initiated on abnormal nocturnal gas exchange alone (Subacute group). These patients had a median of three of the following five overnight gas exchange abnormalities: minimal pulse oximetry (SpO2 ) <90%, maximal transcutaneous carbon dioxide (PtcCO2 ) >50 mmHg, time spent with SpO2 <90% or PtcCO2 >50 mmHg ≥2% of recording time, oxygen desaturation index >1.4/hr. In the last 43 patients, CPAP/NIV was initiated after an abnormal sleep study (Chronic group) on a mean of four of the aforementioned criteria and an apnea-hypopnea index >10/hr., Conclusion: In clinical practice, CPAP/NIV was initiated in an acute, subacute and chronic setting with most patients having an association of several abnormal gas exchange or sleep study parameters. Future studies should evaluate the effectiveness and benefits of CPAP/NIV according to the clinical situation and initiation criteria. Pediatr Pulmonol. 2016; 51:968-974. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Rationale: Expiratory muscle strength is a determinant of cough function. Maximal static expiratory pressure (PEmax) manoeuvres are widely used but are limited by patient motivation and technique. The study hypothesized that whistle mouth (PmW) and cough gastric (PgasCough) pressures might provide additional tests of expiratory muscle strength in children and young adults with neuromuscular disease (NMD)., Methods: We retrospectively reviewed the data of lung function and respiratory muscle tests of all the patients with NMD followed in our centre between November 2001 and December 2013. PmW and PgasCough were compared to other common tests., Results: Three hundred and four respiratory evaluations were performed in 143 patients, aged 3-29 years old. Seventy-two patients had 2 to 8 evaluations. Median [interquartiles] PEmax (38 [28-54] cmH2O) did not differ significantly from PgasCough (45 [30-60] cmH2O) and both were significantly greater than PmW (30 [19-44] cmH2O). Significant good correlations were observed between all the expiratory muscle parameters. The best correlation was observed between PEmax and PmW (r = 0.812, p < 0.001). Moreover, good correlations were found between the percentage of predicted forced vital capacity and PmW (r = 0.619, p < 0.001) and PgasCough (r = 0.568, p < 0.001). Concerning the whistle test, the non-invasive measurement highly correlated with invasive measurements., Conclusions: PmW and PgasCough are simple and valuable tests to assess expiratory muscle strength in children and young adults with NMD. These tests are particularly useful in children having difficulties to perform PEmax manoeuvre. They have the great advantage of their simplicity, but PgasCough is limited by its invasiveness., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Objectives: Sleep disordered breathing (SDB) is common in patients with Prader-Willi syndrome (PWS) and systematic screening is recommended, especially before growth hormone treatment. The aim of the study was to describe the baseline SDB and therapeutic interventions in a large cohort of patients., Study Design: Retrospective study., Subject Selection: Eighty-eight patients with PWS, median [interquartile range] age of 5.1 [1.0-14.5] years old (range 0.3-44.3), who were followed in three centers (France, Italy)., Methodology: Anthropometrics, polygraphy (PG), and gas exchange data were analyzed., Results: Median body mass index (BMI) was 20 [16-34] kg/m(2), BMI z-score for patients aged 2-20 years old was 2.1 [1.2-2.8] SD, mixed-obstructive apnea-hypopnea index (MOAHI) 1.8 [0.6-5.0] events/hr, and central apnea index (CAI) 0.1 [0.0-0.6] events/hr. Minimum pulse oximetry (SpO2) was 88 [84-91]%, percentage of time with SpO2 <90% 0.1 [0.0-1.0]%, and oxygen desaturation index 2 [1-4]/hr. An apnea-hypopnea index (AHI) ≥ 1.5 and ≥ 5 events/hr was observed in 53% of children and 41% of adults, respectively. No correlations were observed between MOAHI and anthropometrics data (age, BMI, BMI z-score), while MOAHI significantly correlated with SpO2 indexes. Age and BMI only weakly correlated with SpO2 indexes. Growth hormone could be initiated in 48 patients. Regarding post-PG therapy, 9 patients had upper airway surgery, and noninvasive CPAP/bilevel ventilation was started in 16 patients., Conclusions: Patients with PWS exhibit a high prevalence of SDB. The lack of association between obesity and SDB leads to hypothesize that hypotonia and/or facial dysmorphic features may play a major role in the occurrence of SDB., (© 2015 Wiley Periodicals, Inc.)

Respiratory mechanics have been poorly studied in children with chronic diffuse parenchymal lung disease (DPLD). The aim of the study was to assess the usefulness of respiratory mechanics to monitor lung function alteration in children with DPLD. Respiratory mechanics, total (WOBt), elastic (WOBe) and resistive (WOBr) work of breathing, gas exchange, lung function and respiratory muscle strength were measured in 10 children, aged 1.8-18.4 years old, who were followed in our national reference centre. Mean tidal volume (Vt) was normal (11±4mL/kg) but respiratory rate (fr, 32±19breaths/min), fr/Vt (118±75breaths/min/L) and total lung resistance (10.2±4.8cmH2OL(-1)s) were increased. Mean WOBt was increased mainly due to WOBe. Dynamic lung compliance (Cldyn) was severely reduced (26±24mL/cmH2O). Cldyn and the oesophageal pressure-time product strongly correlated with vital capacity and functional residual capacity. Respiratory muscle strength was within the normal range. In conclusion, lung mechanics may be considered as useful complementary or alternative markers of functional abnormalities in children with DPLD., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Objective: Data are scarce on respiratory events during sleep for children treated at home with continuous positive airway pressure (CPAP). The present study aimed to characterize the respiratory events with CPAP during sleep and to analyze their clinical consequences., Patients/methods: Consecutive polygraphies (PG) performed on stable children treated with CPAP were analyzed and scored using SomnoNIV Group definitions. For every respiratory event, the presence of a 3% oxygen desaturation and/or an autonomic arousal was systematically searched. Nocturnal gas exchange was assessed using summary data of oximetry and transcutaneous carbon dioxide pressure recordings., Results: Twenty-nine consecutive polygraphies, performed on 26 children (mean age 7.8 ± 6.2 years, mean CPAP use 10.6 ± 14.4 months), were analyzed. The index of total respiratory events was low (median value 1.4/h, range 0-34). The mean number of different types of respiratory events per PG was 2 ± 1 (range 0-4), with always a predominant event. Partial or total upper airway obstruction without a decrease in ventilatory drive was the most frequent event and was the most frequently associated with an oxygen desaturation (in 30% of the events) and an autonomic arousal (in 55% of the events). Weak correlations were observed between nocturnal oximetry and PG results., Conclusions: The index of respiratory events during CPAP treatment for stable children is low. As these events may be associated with an oxygen desaturation or an autonomic arousal, and as nocturnal gas exchange cannot predict PG results, a systematic sleep study seems justified for the routine follow-up of children treated with CPAP., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Collagen VI-related myopathies are hereditary disorders causing progressive restrictive respiratory insufficiency. Specific diaphragm involvement has been suggested by a drop in supine volumes. This pilot study aimed at characterizing the respiratory muscle phenotype in patients with COL6A1-3 genes mutations. Lung function, blood gases, muscle strength and respiratory mechanics were measured in 7 patients between 2002 and 2012. Patients were classified as Early-Severe (n = 3), Moderate-Progressive (n = 2) and Mild (n = 2) according to clinical disease presentation. Seven patients (aged 6-28) were evaluated. Forced vital capacity distinguished the Mild group (>60% predicted) from the two other groups (<50% predicted). This distinction was also possible using the motor function measure scale. Diaphragmatic dysfunction at rest was observed in all the Early-Severe and Moderate-Progressive patients. During a voluntary sniff maneuver diaphragmatic dysfunction was observed in all patients, as assessed by a negative gastric pressure. All patients had diaphragmatic fatigue assessed by a tension-time index over the threshold of 0.15. Diaphragmatic dysfunction during a maximal voluntary maneuver and diaphragmatic fatigue are constant features in Collagen VI myopathies. These observations can assist the diagnosis and should be taken in account for the clinical management, with the early detection of sleep-disordered breathing., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Background: Adherence to continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV) is crucial for the successful treatment of sleep-disordered breathing. The aim of our study was to analyze the adherence of children to long-term home CPAP/NIV treatment., Methods: We analyzed data from all consecutive patients older than the age of 2years, in whom CPAP/NIV treatment was initiated in a specialized pediatric NIV and in those who were receiving CPAP/NIV treatment at home for at least 1month. Data of the memory cards of the ventilators and nocturnal gas exchange were analyzed during a routine CPAP/NIV overnight control in the hospital. CPAP/NIV adherence during the previous month was analyzed according to patient's age, ventilatory mode, type of interface, nocturnal gas exchange, and duration of treatment., Results: The data of 62 children (mean age, 10±5years) with obstructive sleep apnea (n=51) treated with CPAP and neuromuscular disease (n=6) or lung diseases (n=5) treated with NIV were analyzed. Mean adherence was 8:17±2:30h:min per night, and the results did not significantly differ between CPAP and NIV adherence. Seventy-two percent of the patients used their device >8h per night. The mean number of nights of CPAP/NIV use during the last month was 26±5 nights per month. Treatment adherence was not correlated to age, the type of underlying disease, the type of interface (nasal, facial mask, or nasal cannula), nocturnal gas exchange, and duration of CPAP/NIV treatment., Conclusion: Long-term CPAP/NIV adherence at home was extremely high in this group of children followed in a pediatric NIV unit. This finding may explain the lack of effect of the interface, nocturnal gas exchange, and duration of CPAP/NIV treatment., (Copyright © 2013 Elsevier B.V. All rights reserved.)

Background: Patient cooperation is crucial for the success of noninvasive positive pressure ventilation (NPPV). This study evaluated the efficacy of medical hypnosis to reduce anticipatory anxiety and acclimatization time in children who are candidates for long-term NPPV., Methods: Medical hypnosis was performed by a trained nurse. The acclimatization time and long-term compliance with NPPV were evaluated., Results: Hypnosis was performed in nine children aged 2 to 15 years. Seven children had a high level of anticipatory anxiety because of a tracheotomy since birth (n=2), a history of maxillofacial surgery (n=2), severe dyspnea because of lung disease (n=2), and morbid obesity and depression (n=1), and two children with obstructive sleep apnea failed standard NPPV initiation. The hypnosis techniques were based on distraction in the youngest patient and indirect or direct hypnotic suggestions in the older children to obtain a progressive psychocorporal relaxation. All patients accepted the interface and the NPPV after the first hypnosis session. A median of three sessions was needed for overnight (>6 h) NPPV acceptance. The 6-month compliance with NPPV was excellent, with a median use of 7.5 h per night., Conclusion: Medical hypnosis is an effective, safe, noninvasive, and inexpensive tool for reducing the anticipatory distress and acclimatization time for NPPV. This therapy is particularly useful in children with traumatic experiences, such as a tracheotomy or facial surgical procedures.

Purpose: The aims of this study were to assess the interest of pulse wave amplitude (PWA) and actigraphy for characterizing sleep in children with sleep-disordered breathing and to evaluate PWA and actigraphy to assess the efficacy of non-invasive positive pressure ventilation (NPPV)., Methods: We performed a retrospective analysis of children with sleep-disordered breathing. Patients were classified to upper airway obstructive disease (UAO) group or non-obstructive disease (non-UAO) group. Pulse oximetry (SpO2) and PWA were measured by photoplethysmography. Autonomic micro-arousals (AA) and AA related to SpO2 desaturations above 4 % (AA + DS4%) were quantified. The fragmentation index, sleep efficiency, sleep duration, and sleep latency were measured with actigraphy. Transcutaneous carbon dioxide (PtcCO2) was monitored. NPPV was started in case of severe OSA., Results: AA + DS4% were more common in the UAO (n = 15) than the non-UAO group (n = 13) (p < 0.001). All nocturnal gas exchange parameters were worse in the UAO group. Eight children required NPPV. AA + DS4%, maximal PtcCO2, percent of time with PtcCO2 > 50 mmHg, and percent of time with SpO2 < 90 % decreased significantly after 1 month of NPPV., Conclusions: The analysis of AA + DS4% is very informative for the grading of the severity of OSA and for the efficacy of NPPV in children with sleep-disordered breathing.