Respiratory sequelae are a frequent cause of morbidity and mortality in children with NMD. Impaired cough strength and resulting airway clearance as well as sleep disordered breathing are the two main categories of respiratory sequelae. Routine clinical evaluation and diagnostic testing by pulmonologists is an important pillar of the multidisciplinary care required for children with NMD. Regular surveillance for respiratory disease and timely implementation of treatment including pulmonary clearance techniques as well as ventilation can prevent respiratory related morbidity including hospital admissions and improve survival. Additionally, novel disease modifying therapies for some NMDs are now available which has significantly improved the clinical trajectories of patients resulting in a paradigm shift in clinical care. Pulmonologists are \'learning\' the new natural history for these diseases and adjusting clinical management accordingly.

Home non-invasive ventilation (NIV) is used to treat patients with chronic respiratory failure (CRF). However, knowledge on the prevalence and impact of multimorbid aetiology of CRF, patterns of NIV use, and survival of these patients is limited. Our aim was to analyse the multiple aetiologies of CRF, patterns of NIV use and the outcome of those patients. We conducted a retrospective analysis of 1,281 patients treated with home-NIV between 2004 and 2014 in Turku University Hospital, Finland. The patients were divided into nine disease categories: obstructive airways disease (16 %); obesity hypoventilation syndrome (11 %); neuromuscular disease (10 %); chest wall diseases (4 %); sleep apnoea (26 %); interstitial lung diseases (3 %); malignancy (2 %); other (3 %) and acute (8 %), which refers to the patients who did not fulfil criteria of CRF. In addition, multiple aetiologies of CRF were found in 17 %. Mean adherence to home-NIV was 6.0 ± 4.4 h/d and median treatment duration 410 (120-1021) days. Adherence, treatment duration or survival did not significantly differ between patients with either single or multiple causative diseases leading to CRF. Median survival was 4.5 years (95 % CI 3.6 to 5.4). The main reasons for discontinuing NIV were death (56 %) and lack of motivation (19 %). We conclude that home-NIV is used in a variety of diseases. CRF of multiple aetiologies is prevalent and not limited to chronic obstructive lung disease and obstructive sleep apnoea overlap syndrome. However, the adherence to home-NIV or survival did not differ between patients with a single or multiple diseases causing CRF, but the survival of the home-NIV patients differed according to the underlying aetiology of CRF.

UNASSIGNED: To evaluate the incidence, outcome, and prognostic factors of prolonged mechanical ventilation (PMV) in children in Mainland China.
UNASSIGNED: A prospective study was conducted in 11 pediatric intensive care units (PICUs) from May 1, 2021, to April 30, 2022. All pediatric patients on mechanical ventilation meeting the criteria for PMV were included in the study.
UNASSIGNED: Out of 5,292 patients receiving mechanical ventilation, 278 children met the criteria for PMV (5.3%). After excluding case with incomplete data or lost to follow-up, the study included 250 patients. Among them, 115 were successfully weaned from mechanical ventilation, 90 died, and 45 were still on mechanical ventilation. The 6-month survival rate was 64%. The primary associated conditions of PMV were lower airway diseases (36%), central nervous system diseases (32%), and neuromuscular diseases (14%). The stepwise multiple logistic regression analysis indicated that the utilization of vasoactive agents and an elevated pediatric logistic organ dysfunction-2 (PELOD-2) score on the day of PMV diagnosis were significantly associated with an increased of PMV death. Specifically, the odds ratio (OR) for vasoactive agent use was 2.86; (95% CI: 0.15-0.84; P = 0.018), and for the PELOD-2 score, it was 1.37; 95% CI: 1.17-1.61; P < .001). Conversely, early rehabilitation intervention was negatively associated with the risk of PMV death (OR = 0.45; 95% CI: 0.22-0.93; P = .032). Furthermore, the tracheotomy timing emerged as an independent predictor of failure to wean from PMV, with an OR of 1.08, (95% CI: 1.01-1.16; P = .030).
UNASSIGNED: The study revealed a 5.3% incidence of PMV in children requiring mechanical ventilation in China. The use of vasoactive agents and a higher PELOD-2 score at PMV diagnosis were significantly associated with an increased risk of PMV death, whereas early rehabilitation intervention was identified as crucial for improving patient outcomes. The timing of tracheostomy was identified as a high-risk factor for failure to wean from mechanical ventilation.

BACKGROUND: Long-term oxygen therapy (LTOT) is reported to improve survival in patients with chronic respiratory failure. We aimed to describe effectiveness, burden, and cost of illness of patients treated with portable oxygen concentrators (POC) compared to other LTOT options.
METHODS: This retrospective comparative analysis included adult patients with chronic respiratory insufficiency and failure (CRF) upon a first delivery of LTOT between 2014 and 2019 and followed until December 2020, based on the French national healthcare database SNDS. Patients using POC, alone or in combination, were compared with patients using stationary concentrators alone (aSC), or compressed tanks (CTC) or liquid oxygen (LO2), matched on the basis of age, gender, comorbidities, and stationary concentrator use.
RESULTS: Among 244,719 LTOT patients (mean age 75 ± 12, 48% women) included, 38% used aSC, 46% mobile oxygen in the form of LO2 (29%) and POC (18%), whereas 9% used CTC. The risk of death over the 72-month follow-up was estimated to be 13%, 15%, and 12% lower for patients in the POC group compared to aSC, CTC, and LO2, respectively. In the POC group yearly mean total costs per patient were 5% higher and 4% lower compared to aSC and CTC groups, respectively, and comparable in the LO2 group. The incremental cost-effectiveness ratio (ICER) of POC was €8895, €6288, and €13,152 per year of life gained compared to aSC, CTC, and LO2, respectively.
CONCLUSIONS: Within the POC group, we detected an association between higher mobility (POCs autonomy higher than 5 h), improved survival, lower costs, and ICER - €6 238, compared to lower mobility POCs users.

UNASSIGNED: Although people receiving invasive home mechanical ventilation through a tracheostomy are facing both physical and mental health challenges, healthcare services often focus mainly on physical symptoms. To ensure well-functioning treatment and care for people receiving tracheostomy ventilation in a home setting, their mental health needs to be promoted and seen as an integral part of their health in general.
UNASSIGNED: This scoping review aimed to provide a summary of the current knowledge on the mental health of people receiving invasive home mechanical ventilation through a tracheostomy.
UNASSIGNED: A scoping review of published and gray literature based on the framework developed by Arksey and O\'Malley and refined by the JBI was performed. The Preferred Reporting Items for Systematic Reviews and Meta-analyses Extension for Scoping Reviews checklist was used for reporting the findings.
UNASSIGNED: A literature search was conducted by two researchers independently in the PubMed, CINAHL and PsycINFO databases. Additional searches for gray literature were conducted in Google, Google Scholar, websites of selected organisations, and the reference lists of included studies. The software system Covidence was used in the study selection process. For critical appraisal, the Mixed Methods Appraisal Tool was used.
UNASSIGNED: Thirteen studies were included in this review, of which six used qualitative, six quantitative, and one mixed methods. The majority of studies were authored in Europe (n = 10), followed by the Americas (n = 2) and the Western Pacific (n = 1). Mental health was investigated both directly and indirectly (61.5% vs. 38.5%). Categorizing the reported mental health outcomes, we found that emotional well-being was reported widely across the studies (n = 13), while psychological well-being (n = 5) and social well-being (n = 4) were less widely reported.
UNASSIGNED: The mental health of people receiving home tracheostomy ventilation has received some scholarly attention. A heterogeneity of mental health outcomes was reported in the literature with emotional well-being being an important mental health area both in relation to the sub-components positive affect and quality of life appraisal. Mental health outcomes in relation to psychological well-being and social well-being were fragmented and only sparsely investigated.

BACKGROUND: Children with advanced pulmonary disease due to cystic fibrosis (CF) are at risk of acute respiratory failure due to pulmonary exacerbations leading to their admission to pediatric intensive care units (PICU). The objectives of this study were to determine short and medium-term outcomes of children with CF admitted to PICU for acute respiratory failure due to pulmonary exacerbation and to identify prognosis factors.
METHODS: This retrospective monocentric study included patients less than 18 years old admitted to the PICU of a French university hospital between 2000 and 2020. Cox proportional hazard regression methods were used to determine prognosis factors of mortality or lung transplant.
RESULTS: Prior to PICU admission, the 29 patients included (median age 13.5 years) had a severe lung disease (median Forced Expiratory Volume in 1 s percentage predicted at 29%). Mortality rates were respectively 17%, 31%, 34%, 41% at discharge and at 3, 12 and 36 months post-discharge. Survival rates free of lung transplant were 34%, 32%, 24% and 17% respectively. Risk factors associated with mortality or lung transplant using the univariate analysis were female sex and higher pCO2 and chloride levels at PICU admission, and following pre admission characteristics: home respiratory and nutritional support, registration on lung transplant list and Stenotrophomonas Maltophilia bronchial colonization.
CONCLUSIONS: Children with CF admitted to PICU for acute respiratory failure secondary to pulmonary exacerbations are at high risk of death, both in the short and medium terms. Lung transplant is their main chance of survival and should be considered early.

OBJECTIVE: Venous blood gases (VBGs) are not consistently considered suitable surrogates for arterial blood gases (ABGs) in assessing acute respiratory failure due to variable measurement error. The physiological stability of patients with chronic ventilatory failure may lead to improved agreement in this setting.
METHODS: Adults requiring ABGs for sleep or ventilation titration studies had VBGs drawn before or after each ABG, in a randomized order. Veno-arterial correlation and agreement were examined for carbon dioxide tension (PCO2), pH, oxygen tension (PO2), and oxygen saturation (SO2).
RESULTS: We analyzed 115 VBG-ABG pairs from 61 patients. Arterial and venous measures were correlated (P < .05) for PCO2 (r = .84) and pH (r = .72), but not for PO2 or SO2. Adjusted mean veno-arterial differences (95% limits of agreement) were +5.0 mmHg (-4.4 to +14.4) for PCO2; -0.02 (-0.09 to +0.04) for pH; -34.3 mmHg (-78.5 to +10.0) for PO2; and -23.9% (-61.3 to +13.5) for SO2. VBGs obtained from the dorsal hand demonstrated a lower mean PCO2 veno-arterial difference (P < .01). A venous PCO2 threshold of ≥ 45.8 mmHg was > 95% sensitive for arterial hypercapnia, so measurements below this can exclude the diagnosis without an ABG. A venous PCO2 threshold of ≥ 53.7 mmHg was > 95% specific for arterial hypercapnia, so such readings can be assumed diagnostic. The area under the receiver operating characteristic curve of 0.91 indicated high discriminatory capacity.
CONCLUSIONS: A venous PCO2 < 45.8 mmHg or ≥ 53.7 mmHg would exclude or diagnose hypercapnia, respectively, in patients referred for sleep studies, but VBGs are poor surrogates for ABGs where precision is important.
BACKGROUND: Registry: Australian New Zealand Clinical Trials Register; Name: A comparison of arterial and blood gas analyses in sleep studies; URL: https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=372717; Identifier: ACTRN12617000562370.
BACKGROUND: Lindstrom SJ, McDonald CF, Howard ME, et al. Venous blood gases in the assessment of respiratory failure in patients undergoing sleep studies: a randomized study. J Clin Sleep Med. 2024;20(8):1259-1266.

This state-of-the-art review provides an overview of the history of home mechanical ventilation (HMV), including early descriptions of mechanical ventilation from ancient and Renaissance perspectives and the mass development of ventilators designed for long-term use during the poliomyelitis epidemic. Seminal data from key clinical trials supports the application of HMV in certain patients with chronic obstructive pulmonary disease, neuromuscular disease and obesity-related respiratory failure. Innovative engineering coupled with refined physiological understanding now permits widespread delivery of home mechanical ventilation to a global population, using portable devices with advanced ventilatory modes and telemonitoring capabilities. Exponential growth in digital technology continues, and ongoing research is needed to understand how to harness clinical and physiological data to benefit patients and healthcare services in a clinically- and cost-effective manner.

OBJECTIVE: Noninvasive ventilation (NIV) is an effective treatment for chronic respiratory failure (CRF). Patient-centered outcomes need to be evaluated regularly and the S3-NIV questionnaire seems an applicable tool. We translated this short, self-administered questionnaire into a Dutch version and tested its construct validity and reliability.
METHODS: An observational study was conducted, including 127 stable long-term NIV users with CRF or complex sleep related breathing disorders due to different underlying diseases: chronic obstructive pulmonary disease (25%), slowly progressive neuromuscular disorders (35%), rapidly progressive neuromuscular disorders (12%) and \'other disorders\' (28%) including complex sleep apnea and obesity hypoventilation syndrome. Construct validity and reliability were tested.
RESULTS: The Dutch version of the questionnaire was obtained after a translation and back-translation process. Internal consistency of the total score was good (Cronbach\'s α coefficient of 0.78) as well as for the \'respiratory symptoms\' subdomain and the \'sleep and side effects\' subdomain (Cronbach\'s α coefficient of 0.78 and 0.69, respectively). The reproducibility was excellent with an intraclass correlation of 0.89 (95% CI 0.87-0.93). Construct validity was good for the \'respiratory symptoms\' subdomain.
CONCLUSIONS: The Dutch S3-NIV questionnaire is a reliable and valid tool to evaluate symptoms, sleep, and NIV related side effects in long-term NIV users.