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Background: Children with chronic respiratory failure and/or sleep disordered breathing due to a broad range of diseases may require long-term ventilation to be managed at home. Advances in the use of long-term non-invasive ventilation has progressively leaded to a reduction of the need for invasive mechanical ventilation through tracheostomy. In this study, we sought to characterize a cohort of children using long-term NIV and IMV and to perform an analysis of those children who showed significant changes in ventilatory support management., Methods: We performed a retrospective cohort study of pediatric (within 18 years old) patients using long-term, NIV and IMV, hospitalized in our center between January 1, 2000 and December 31, 2017. A total of 432 children were included in the study. Long Term Ventilation (LTV) was defined as IMV or NIV, performed on a daily basis, at least 6 h/day, for a period of at least 3 months., Results: 315 (72.9%) received non-invasive ventilation (NIV); 117 (27.1%) received invasive mechanical ventilation (IMV). Children suffered mainly from neuromuscular (30.6%), upper airway (24.8%) and central nervous system diseases (22.7%). Children on IMV were significantly younger when they start LTV [NIV: 6.4 (1.2-12.8) years vs IMV 2.1 (0.8-7.8) years] (p < 0.001)]. IMV was likely associated with younger age at starting ventilatory support (aOR 0.9428; p = 0.0220), and being a child with home health care (aOR 11.4; p < 0.0001). Overtime 39 children improved (9%), 11 children on NIV (3.5%) received tracheostomy; 62 children died (14.3%); and 74 children (17.1%) were lost to follow-up (17.8% on NIV, 15.4% on IMV)., Conclusions: Children on LTV suffered mainly from neuromuscular, upper airways, and central nervous system diseases. Children invasively ventilated usually started support younger and were more severely ills.

Background: Long-term mechanical ventilation (LTV) with non-invasive ventilation (NIV) prolongs survival in patients with Neuromuscular Diseases (NMDs). Transition from paediatric to adult healthcare system is an undervalued and challenging issue for children with chronic conditions on mechanical ventilation., Methods: this retrospective study aims to compare issues of young adults in age to transition to adult care (≥ 15 years old) affected by NMDs on NIV in two different Paediatric Respiratory Units in two different countries: Bambino Gesù Children's Hospital, Research Institute, (Rome, Italy) (BGCH) and the Paediatric Respiratory Unit of the Royal Brompton Hospital (London, UK) (RBHT)., Results: The median (min-max) age at starting ventilation was significantly different in the two groups (16 years old vs 12, p = 0.0006). We found significant difference in terms of median age at the time of observation (18 (15-22) vs 17 (15-19) years, p = 0.0294) and of type of referral (all the patients from the BGCH group were referred to paediatric services (n = 15, 100%), median age 18 (15-22); only 6 patients, in the RBHT group, with a median age 15.50 (15-17) years, were entirely referred to paediatric service). We found different sleep-disordered breathing assessments 6 full Polysomnographies, 7 Cardio-Respiratory Polygraphies and 2 oximetry with capnography (SpO 2 -tcCO 2 ) studies in the BCGH group, while all patients of RBHT group were assessed with an SpO 2 -tcCO 2 study. All patients from both groups underwent multidisciplinary assessment., Conclusions: In conclusion, patients with NMDs on NIV in age to transition to adult require complex multidisciplinary management: significant efforts are needed to achieve the proper transition to adult care.

Background: Seasonal variability on obstructive sleep apnea has already been studied by polysomnography in children. Winter and spring season emerged as critical periods. No data are currently available for pulse oximetry performed at home. The aim of our study was to evaluate the effect of seasonality and age on the results of at-home pulse oximetry performed in children referred for suspected OSA., Methods: We retrospectively studied 781 children (64.3% Males), aged 4.9 ± 2.5 years. For all patients, we evaluated both pulse oximetry metrics and the McGill Oximetry Score. Variables for seasonal groups were assessed using Kruskal-Wallis test. A logistic regression model was performed to assess the relationship between patients' main characteristics, season period and the likelihood to have an abnormal McGill Oximetry Score., Results: Patients recorded during winter were significantly younger (p < 0.02), nadir SpO 2 was significantly lower (p < 0.002) and DI 4 significantly higher than during others seasons (p < 0.005). Moreover, patients recorded during winter were nearly 2 times more likely to have an abnormal MOS (aOR 1.949). The logistic regression showed that also younger age (p < 0.0001) was associated with a higher risk to find an abnormal pulse oximetry., Conclusions: In our study, the winter season confirms to be a critical period for pulse-oximetry and it should be taken into account by clinicians for a correct interpretation of tests. Our data show that also younger age affects the prevalence of abnormal at-home pulse oximetry in children.