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Objective: Dyspnoea and sleep-disordered breathing (SDB) are common in children with life-limiting conditions but studies on treatment with non-invasive ventilation (NIV) or continuous positive airway pressure (CPAP) are scarce. The aim of the study was to describe children treated with long-term NIV/CPAP within a paediatric palliative care programme in France., Methods: Cross-sectional survey on children and young adults with complex medical conditions treated within the French paediatric NIV network with long-term NIV/CPAP. Characteristics of the patients were analysed and patient-related outcome measures of NIV/CPAP benefit were reported., Results: The data of 50 patients (68% boys), median age 12 (0.4-21) years were analysed. Twenty-three (46%) patients had a disorder of the central nervous system and 5 (10%) a chromosomal anomaly. Thirty-two (64%) patients were treated with NIV and 18 (36%) with CPAP. NIV/CPAP was initiated on an abnormal Apnoea-Hypopnoea Index in 18 (36%) of the patients, an abnormal nocturnal gas exchange alone in 28 (56%), and after an acute respiratory failure in 11 (22%) of the patients. Mean objective NIV/CPAP adherence was 9.3±3.7 hours/night. NIV/CPAP was associated with a decrease in dyspnoea in 60% of patients, an increase in sleep duration in 60% and in sleep quality in 74%, and an improvement in parents' sleep in 40%., Conclusions: In children with life-limiting conditions, long-term NIV/CPAP may be associated with relief of dyspnoea, an improvement of SDB and an improvement in parents' sleep., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To describe the characteristics of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in France., Design: Cross-sectional national survey., Setting: Paediatric CPAP/NIV teams of 28 tertiary university hospitals in France., Patients: Children aged <20 years treated with CPAP/NIV since at least 3 months on June 1st, 2019., Intervention: An anonymous questionnaire was filled in for every patient., Results: The data of 1447 patients (60% boys), mean age 9.8 ± 5.8 years were analysed. The most frequent underlying disorders were: upper airway obstruction (46%), neuromuscular disease (28%), disorder of the central nervous system (13%), cardiorespiratory disorder (7%), and congenital bone disease (4%). Forty-five percent of the patients were treated with CPAP and 55% with NIV. Treatment was initiated electively for 92% of children, while 8% started during an acute illness. A poly(somno)graphy (P(S)G) was performed prior to treatment initiation in 26%, 36% had a P(S)G with transcutaneous carbon dioxide monitoring (PtcCO 2 ), while 23% had only a pulse oximetry (SpO 2 ) with PtcCO 2 recording. The decision of CPAP/NIV initiation during an elective setting was based on the apnea-hypopnea index (AHI) in 41% of patients, SpO 2 and PtcCO 2 in 25% of patients, and AHI with PtcCO 2 in 25% of patients. Objective adherence was excellent with a mean use of 7.6 ± 3.2 h/night. Duration of CPAP/NIV was 2.7 ± 2.9 years at the time of the survey., Conclusion: This survey shows the large number of children treated with long term CPAP/NIV in France with numerous children having disorders other than neuromuscular diseases., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Objectives: General emergency medicine physicians sometimes have to deal with acute management of pediatric emergencies. The objectives of this study were to assess the pediatric medical education background of emergency physicians, how often they encounter pediatric emergencies, as well as the knowledge and organizational gaps regarding the management of children in general emergency departments., Methods: A survey was conducted from March 25 to June 25, 2017, by emailing an anonymized questionnaire to all senior emergency physicians of the 22 general emergency departments of western Normandy public hospitals., Results: A total of 81 responses were analyzed. In all, 83% percent of respondents had previously worked in a pediatric department. In total, 90% of the respondents reported caring for children within their emergency department and 93% out-of-hospital (100% of them during primary interventions and 39% during secondary interventions such as inter-hospital transfers). Fourteen percent of the respondents considered that the pediatric medical education they received was adapted to their current practice, while 73% reported experiencing difficulties during management of pediatric emergencies (technical difficulties, unsuitable material and therapeutics, relational problems, personal apprehension, disease- or age-specific difficulties, especially with children under the age of 2-3 years)., Conclusion: Most general emergency physicians report caring for children despite a lack of medical education in pediatrics. Pediatric medical education as well as collaborations between general practitioners and specialized pediatric teams should be enhanced to better match the needs of general emergency departments and improve the quality of primary and acute care for children., (Copyright © 2020 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.)

Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a rare autosomal recessive neuromuscular disorder characterized by progressive motor and respiratory decline during the first year of life. Early and late-onset cases have recently been reported, although not meeting the established diagnostic criteria, these cases have been genotyped. We thus conducted a national multicenter observational retrospective study to determine the prognosis of children with SMARD1 according to their phenotype. We recorded all known French pediatric cases with mutations identified on the immunoglobulin μ-binding protein 2 gene and the presence of respiratory symptoms. Thirty centers provided 22 observations. A diaphragmatic palsy was diagnosed 1.5 months (p = 0.02) after first respiratory symptoms, and hypotonia preceded areflexia by 4 months (p = 0.02). Early onset of symptoms leading to specialist consultation before the age of 3 months was associated with a significantly worse prognosis (p < 0.01). Among the 6 patients who were still alive, all were tracheostomized. Only one case survived beyond 2 years without artificial ventilation. The remaining patients died at a median age of 7 months. Our results may help pediatricians to provide medical information to parents and improve the decision-making process of setting up life support., (Copyright © 2018 Elsevier B.V. All rights reserved.)