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OBJECTIVE: The aims of this multi-center study were to describe the characteristics of children receiving long-term home mechanical ventilation (HMV) in İstanbul and to compare the patients receiving non-invasive and invasive ventilation. MATERIAL AND METHODS: This cross-sectional multicenter study included all children receiving long-term HMV followed by admission to six tertiary hospitals. The data were collected between May 2020 and May 2021. Demographic data and data regarding HMV were collected from the patient charts. RESULTS: The study included 416 participants. The most common diagnoses were neuromuscular (35.1%) and neurological diseases (25.7%). Among the patients, 49.5% (n = 206) received non-invasive ventilation (NIV), whereas 50.5% (n = 210) received invasive ventilation. The median age at initiation was significantly younger in the invasive ventilation group than in the NIV group (10 vs. 41 months, P < 0.001). Most subjects in the NIV group (81.1%) received ventilation support only during sleep, whereas most subjects in the invasive ventilation group (55.7%) received continuous ventilator support (P < 0.001). In addition to ventilation support, 41.9% of the subjects in the invasive ventilation group and 28.6% in the NIV group received oxygen supplementation (P = 0.002). Within the last year, 59.1% (n = 246) of the subjects were hospitalized. The risk factors for hospitalization were invasive ventilation, continuous ventilatory support, oxygen supplementation, tube feeding, and swallowing dysfunction (P = 0.002, 0.009,

Abstract Background Non-invasive ventilation (NIV) uses positive pressure to assist people with respiratory muscle weakness or severe respiratory compromise to breathe. Most people use this treatment during sleep when breathing is most susceptible to instability. The benefits of using NIV in motor neurone disease (MND) are well-established. However, uptake and usage are low (~ 19%) and there is no consensus on how to best implement NIV in MND in Australia. Consequently, clinical practice models are highly variable. Our team has recently provided evidence that specific and individualised NIV titration using a sleep study (polysomnography; PSG) leads to better outcomes in people with MND. However, for this clinical practice model to result in sustained benefits, evidence of effectiveness across multiple sites, as well as culture and practice change, must occur. Methods A two-arm, assessor-blinded, individual participant randomised controlled trial in MND care centres across Australia will be undertaken. Two-hundred and forty-four participants will be randomised (1:1) to either the intervention group (PSG-assisted commencement of NIV settings; PSG) or a control group (sham PSG). Participants will be asked to use their NIV device for 7 weeks and will then return for follow-up assessments. Respiratory, sleep and patient-reported outcome measures will be collected at baseline and follow-up. The primary aim is to determine if the proportion of participants using NIV for > 4 h/day during the intervention period is higher in the PSG than the control group. A process evaluation, health economic evaluation and 12-month cohort follow-up will be undertaken and reported separately. Discussion The results of this trial will demonstrate the effects of PSG-assisted titration of NIV on usage of NIV in people with MND. We hypothesise that the PSG intervention will improve synchrony between the user and the machine, which will lead to greater NIV usage compared to the control group. Trial registration ClinicalTrials.gov NCT05136222. Registered on November 25, 2021.

Severe respiratory failure, resulting from a wide range of acute and chronic diseases such as chronic obstructive pulmonary diseases, obstructive sleep apnoea, obesity hypoventilation syndrome (Pickwickian syndrome), COVID-19, bilateral pneumonitis, kyphoscoliosis, acute respiratory distress syndrome, amyotrophic lateral sclerosis, Guillain-Barré syndrome, and others, poses a significant challenge for healthcare systems worldwide. Treating severe respiratory failure often requires long-term or lifelong respiratory support, which places a heavy burden on both patients and healthcare systems. Despite the availability of free comprehensive care for patients with chronic kidney failure and heart failure, those requiring long-term respiratory support are often deprived of adequate medical and social assistance. This gap in care leads to frequent hospitalizations, disability, and premature death of patients with severe respiratory failure, further straining healthcare resources and increasing social tension. The solution of the problem is an organisation of ambulatory centre for homecare respiratory support for patients with severe chronic respiratory failure. Homecare respiratory support demonstrated worldwide cost-effectiveness by significantly enhancing patients clinical and social outcomes, restoring their work capacity, and reducing the need for hospitalizations. This article emphasizes the necessity of a comprehensive homecare respiratory support system and highlights how inadequate assistance can result in frequent hospitalizations, increased disability, and premature death, thereby imposing an additional strain on the healthcare system.

Abstract The newly developed portable oxygen concentrator with an auto-demand oxygen delivery system (auto-DODS) automatically switches between 3 sensitivities according to the negative pressure gradient of inhalation and supplies oxygen only during inhalation. The aim of this study was to verify the effectiveness and safety of auto-demand devices compared with a continuous flow oxygen concentrator, during sleep, in a randomized crossover noninferiority trial. We alternatively used an auto-DODS or a continuous-flow oxygen concentrator, all night on separate days for HOT (Home Oxygen Therapy) patients with nocturnal hypoxemic symptoms. The primary endpoints were the mean value of oxygen saturation (SpO2) over the total sleep time. The secondary endpoints included the efficacy endpoints and the safety endpoints. Regarding the primary endpoint, the difference in SpO2 between the auto-DODS and continuous flow was 0.835%. Since the upper limit of this difference did not exceed 2.8, which was set as the noninferiority margin, it was shown that the auto-DODS did not reduce SpO2 by at least 2.8% on average compared to continuous flow. No equipment failure or exacerbation of disease was observed, confirming the safety of the auto-DODS during the night.

Abstract Background Studies on long-term invasive mechanical ventilation (IMV) via tracheostomy in chronic respiratory insufficiency are limited. The aim of this study was to clarify the use of HIMV (home invasive mechanical ventilation) within the Finnish population and to analyze the characteristics and survival rate of HIMV patients from 2015 to 2022. Methods Data on HIMV patients was collected annually from all Finnish Hospital District patient registries between January 1, 2015, and December 31, 2022. Data included basic demographic data of the patients, underlying diagnosis, time from diagnosis to HIMV initiation, treatment duration, and mortality. Results This study included 179 patients. In 2015, there were 107 HIMV patients, and as of December 31, 2022, there were 95 patients. During the eight-year follow-up period, 84 patients (46.9%) died and there were 67 new patients between 2015 and2022. The prevalence of HIMV treatment in Finland was 2.4/100,000 on January 1,2015, and 1.8/ 100 000 on December 31, 2022. The average number of years living with HIMV for deceased patients at death was 10.1 ± 10.5 years largely depending on the underlying diagnosis. Of all the HIMV treatments, 32% were elective. Conclusions HIMV is a rare treatment in Finland, and based on our 8-year follow-up, prevalence of HIMV is diminishing. Given the high demands, and significant costs associated with HIMV, it is essential to prepare for long treatment, when planning HIMV. It is also advisable to prolong non-invasive ventilation (NIV) treatments for as long as possible.

Abstract Introduction 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. Conclusion 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.

Abstract 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. Conclusion 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.

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.

Abstract Background The number of patients depending on home mechanical ventilation (HMV) has increased substantially in Germany in recent years. These patients receive long-term care in different nursing facilities (nursing home, shared living community, private home). However, there are limited data available on the quality of care of HMV patients. The aim of the OVER-BEAS project was to identify quality indicators (QIs) of HMV care using an evidence-based approach. Methods A multidisciplinary board consisting of professionals and experts of HMV provision compiled a set of QIs between March and September 2019. In a structured, transparent process a set of QIs covering structures, processes and outcome of HMV patient’s care were proposed and evaluated based on the best available evidence. QIs were defined as relevant, reliable and valid measurements of the quality of HMV care and furthermore to be comprehensive and applicable in practice. Results The experts proposed 40 QIs and consented a final set of 26 QIs. Based on the final set, questionnaires to document the QIs were developed: (1) to assess the quality and describe the structure of the nursing facility; and (2) to gather information on patient-related processes and outcomes. The feasibility of the questionnaires was tested in 5 nursing facilities treating HMV patients. The remarks from the nursing specialists were categorised in three groups: (1) term missing accuracy, (2) problem of understanding, and (3) not documented or documented elsewhere. Mean documentation time by the nursing specialists for one patient was 15 min. Based on this feedback, the questionnaires were finalised. Conclusions We proposed a set of QIs relating to long-term HMV care and developed two questionnaires to collect this information. In a pilot study, we found the set of questionnaires to be feasible in assessing the quality of HMV care according to current evidence. The development of standardised evidence-based QIs to evaluate HMV care is a step towards implementing a standardised quality assurance program to document the quality of care of HMV patients.

Abstract Background Non-invasive positive pressure ventilation (NIPPV) has emerged as a recognized and effective long-term therapeutic approach for individuals suffering from chronic respiratory insufficiency resulting from various diverse disorders. Nevertheless, providing home non-invasive positive pressure ventilation encounters several challenges, including compliance, training inadequacies, and limited resources. Therefore, the objective of this study was to observe the impact of home non-invasive positive pressure ventilation on patients with chronic respiratory failure, specifically focusing on its effects on the mean pulmonary artery pressure, diaphragmatic excursion, and associated complications. Results The study included a total of 48 patients, consisting of 26 males (54.1%) and 22 females (45.9%). The baseline mean pulmonary artery pressure (PAP) was found to be 39.79 ± 7.51. Additionally, the baseline diaphragmatic excursion in quiet breathing was measured to be 1.80 ± 0.38 cm, while the baseline diaphragmatic excursion in deep breathing was recorded as 4.35 ± 0.99 cm. Following the implementation of home non-invasive ventilation, specifically bilevel-positive airway pressure or continuous positive airway pressure, significant improvements were observed in the aforementioned parameters. The most commonly reported complications among the patients included skin ulcers and aerophagia. Furthermore, mortality rate of 6.3% was observed. Conclusions The use of home non-invasive positive pressure ventilation has been shown to be a successful and sustainable therapeutic approach for persons suffering from chronic respiratory failure. This therapy method has significantly improved PAP, diaphragm force, and overall mortality rates. The use of ultrasonography to evaluate pulmonary pressure or diaphragmatic excursion is considered a beneficial and straightforward approach for the follow-up of subjects with chronic respiratory failure.

ObjectiveTo evaluate the incidence, outcome, and prognostic factors of prolonged mechanical ventilation (PMV) in children in Mainland China.MethodsA 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.ResultsOut 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

Abstract Introduction The symptoms of obesity hypoventilation syndrome (OHS) may be present for years with concomitant progressive comorbidities, and the condition is frequently diagnosed late as a result of acute‐on‐chronic hypercapnic respiratory failure. Although some data exist on intensive care unit (ICU) prevalence, mortality and morbidity of OHS, little is known about the ICU mortality of these chronic respiratory failure patients during the COVID‐19 pandemic. Methods We performed a cross‐sectional observational study in five Hungarian Intensive Care Units for 4 months during the COVID‐19 pandemic. All ICU patients were screened for OHS risk factors by treating physicians. Risk factors were defined as obesity (body mass index [BMI] ≥ 30 kg/m2) and at least one of the following: Epworth Sleepiness Score ≥ 6; symptoms of right heart failure; daytime or night‐time hypoxemia; presence of loud snoring; witnessed apnoea. We calculated prevalence, mortality and factors associated with unfavourable outcome. Results A total of 904 ICU patients were screened for OHS risk factors. Overall 79 (8.74 ± 5.53%) patients were reported to have met the criteria for suspected OHS with a mortality rate of 40.5%; 69% (54 patients) of the cohort displayed at least 3 symptoms related to OHS before their acute illness. COVID‐19 infection was associated with higher mortality in OHS‐suspected patients, independently of actual BMI. Conclusion Despite the increased risk of obese patients, suspected OHS did not show higher prevalence than expected during the COVID‐19 pandemic in critically ill patients. COVID‐19 infection however was a risk for mortality in these patients, independent of actual BMI.

Background and objective: Non-invasive ventilation (NIV) improves survival of patients with chronic respiratory failure (CRF). Most often, pressure settings are made to normalize arterial blood gases. However, this objective is not always achieved due to intolerance to increased pressure or poor compliance. Few studies have assessed the effect of persistent hypercapnia on ventilated patients’ survival. Data from the Pays de la Loire Respiratory Health Research Institute cohort were analyzed to answer this question. Study design and methods: NIV-treated adults enrolled between 2009 and 2019 were divided into 5 subgroups: obesity-hypoventilation syndrome (OHS), COPD, obese COPD, neuromuscular disease (NMD) and chest wall disease (CWD). PaCO2 correction was defined as the achievement of a PaCO2 < 6 kPa or a 20% decrease in baseline PaCO₂ in COPD patients. The endpoint was all-cause mortality. Follow-up was censored in case of NIV discontinuation. Results: Data from 431 patients were analyzed. Median survival was 103 months and 148 patients died. Overall, PaCO2 correction was achieved in 74% of patients. Bivariate analysis did not show any survival difference between patients who achievedPaCO₂ correction and those who remained hypercapnic: overall population: p = 0.74; COPD: p = 0.97; obese COPD: p = 0.28; OHS: p = 0.93; NMD: p = 0.84; CWD: p = 0.28. Conclusion: Moderate residual hypercapnia under NIV does not negatively impact survival in CRF patients. In individuals with poor tolerance of pressure increases, residual hypercapnia can therefore be tolerated under long-term NIV. Larger studies, especially with a higher number of patients with residual PaCO2 > 7 kPa, are needed to confirm these results.

The aim of this pilot retrospective study was to test the Rehabilitation complexity scale (RCS-E v13) in patients from 15 Italian pulmonary rehabilitation (PR) units and correlate RCS-E v13 to the most used clinical and functional outcome measures. At admission and discharge, clinical data [comorbidities with the Cumulative Illness Rating Scale (CIRS)], Barthel Index (BI), Barthel Index dyspnea (BI-d), COPD Assessment Test (CAT), and six-minute walking test (6MWT) were collected, and RCS-E total score calculated. Two-hundred nineteen patients [30.6% COPD, 43.4% chronic respiratory failure (CRF), and 26% with invasive ventilation (IV)], aged 69.9 (11.2) years were considered. RCS-E at admission 8.63 (1.69), 11.06 (2.50), 16.56 (2.97) and discharge 0.84 (1.02), 2.19 (1.5), 7.09 (1.47)] for COPD, CRF and IV respectively were statistically differed among groups (ANOVA p =

Abstract Background Chronic respiratory conditions are a prominent public health issue and thus, building a patient registry might facilitate both policy decision making and improvement of clinical management processes. Hellenic Registry of patients with Home Mechanical Ventilation (HR-HMV) was initiated in 2017 and a web-based platform is used to support patient data collection. Eighteen hospital departments (including sleep labs) across Greece participate in this initiative, focusing on recording data for both children and adult patients supported by mechanical ventilation at home, including patients with Sleep Apnea–Hypopnea Syndrome (SAHS) under Positive Airway Pressure (PAP) therapy. Methods The HR-HMV initiative ultimately aims to provide a database for evidence-based care and policy making in this specific domain. To this end, a web information system was developed and data were manually collected by clinics and hospital departments. Legal and privacy issues (such as General Data Protection Rule compliance and technical information security measures) have been considered while designing the web application. Based on the collected data, an exploratory statistical report of SAHS patients in Greece is presented. Results Eleven out of the eighteen participating clinics and hospital departments have contributed with data by the time of the current study. More than 5000 adult and children patient records have been collected so far, the vast majority of which (i.e., 4900 patients) diagnosed with SAHS. Conclusion The development and maintenance of patient registries is a valuable tool for policy decision making, observational/epidemiological research and beyond (e.g., health technology assessment procedures). However, as all data collection and processing approaches, registries are also related with potential biases. Along these lines, strengths and limitations must be considered when interpreting the collected data, and continuous validation of the collected clinical data per se should be emphasized. Especially for Greece, where the lack of national registries is eminent, we argue that HR-HMV could be a useful tool for the development and the update of related policies regarding the healthcare services for patients with home mechanical ventilation support and SAHS patients, which could be useful for related initiatives at a European level as well.

Purpose: Diabetic retinopathy (DR) is a hypoxic retinal disease, but so far, the association with systemic hypoxia is poorly understood. Hence, the aim of this study was to evaluate cross-sectional and longitudinal associations between DR and chronic respiratory failure (CRF) in a national cohort. Design: Cross-sectional and 5-year longitudinal register-based cohort study. Methods: Between 2013 and 2018, we included patients with diabetes from the Danish Registry of Diabetic Retinopathy, who were each age and sex matched with five controls without diabetes. At index date, the prevalence of CRF was compared between cases and controls, and the longitudinal relationship between DR and CRF was assessed in a five-year follow-up. Results: At baseline, we identified 1,980 and 9,990 patients with CRF among 205,970 cases and 1,003,170 controls. The prevalence of CRF was higher among cases than controls (OR 1.75, 95% CI 1.65–1.86), but no difference between cases with and without DR was found.During follow-up, we identified 1,726 and 5,177 events of CRF among cases and controls, respectively. The incidence of CRF was higher among both cases with and without DR compared to controls (DR level 0: HR 1.24, 95% CI 1.16–1.33, DR level 1–4: HR 1.86, 95% CI 1.63–2.12), and higher among cases with DR compared to cases without DR (HR 1.54, 95% CI 1.38–1.72). Conclusion: In this study based on nationwide data, we found an increased risk of present and incident CRF in patients with diabetes with or without DR, and we identified DR as a predictor of future CRF.

Abstract Background Chronic respiratory failure (CRF) can be treated at home with non-invasive ventilation (NIV) and/or long-term oxygen (LTOT). The prevalence of these treatments is largely unknown. We aimed to clarify the prevalence and indications of the treatments, and the three-year mortality of the treated patients in the Helsinki University Hospital (HUH) area in Finland. Methods In this retrospective study we analyzed the prevalence of adult CRF patients treated with NIV and/or LTOT on 1.1.2018 and followed these patients until 1.1.2021. Data collected included the underlying diagnosis, patient characteristics, information on treatment initiation and from the last follow-up visit, and mortality during the three-year follow-up. Patients with home invasive mechanical ventilation or sleep apnea were excluded. Results On 1.1.2018, we had a total of 815 patients treated with NIV and/or LTOT in the Helsinki University Hospital (HUH) area, with a population of 1.4 million. The prevalence of NIV was 35.4 per 100,000, of LTOT 24.6 per 100,000 and of the treatments combined 60.0 per 100,000. Almost half, 44.5%, were treated with NIV, 41.0% with LTOT, and 14.4% underwent both. The most common diagnostic groups were chronic obstructive pulmonary disease (COPD) (33.3%) and obesity-hypoventilation syndrome (OHS) (26.6%). The three-year mortality in all patients was 45.2%. In the COPD and OHS groups the mortality was 61.3% and 21.2%. In NIV treated patients, the treatment durations varied from COPD patients 5.3 years to restrictive chest wall disease patients 11.4 years. The age-adjusted Charlson co-morbidity index (ACCI) median for all patients was 3.0. Conclusions NIV and LTOT are common treatments in CRF. The prevalence in HUH area was comparable to other western countries. As the ACCI index shows, the treated patients were fragile, with multiple co-morbidities, and their mortality was high. Treatment duration and survival vary greatly depending on the underlying diagnosis.

Abstract Background With the growing practice of home mechanical ventilation, there is a need to identify biological markers for adequate follow-up. Red cell distribution width (RDW) is a promising candidate because it is convenient, objective and may reflect treatment effect over a long period of time. The aim of this study was to explore the possible role of RDW as a marker for home mechanical ventilation in real-life, unselected chronic respiratory patient populations. Methods First, we identified characteristic RDW values for mixed case, unselected chronic respiratory failure and home mechanical ventilated patients through retrospective review within our institutional database. Next, we conducted a prospective observational study to identify RDW changes during the first six months of optimized home mechanical ventilation treatment. Adult patients starting home mechanical ventilation were included. Factors affecting RDW change during the first 6 months of treatment were analysed. Results RDW was elevated in both chronic respiratory failure and home mechanical ventilation patients compared to healthy individuals in the retrospective review. In the prospective study of 70 patients, we found that 55.4% of patients starting home mechanical ventilation have abnormal RDW values which are reduced from 14.7 (IQR = 13.2–16.2)% to 13.5 (IQR = 13.1–14.6)% during the first 6 months of HMV treatment (p

Chronic respiratory failure is a common complication of neuromuscular disease. The use of noninvasive ventilation and mechanically assisted cough can reduce symptoms of hypoventilation, slow lung function decline, improve sleep quality, and in some cases prolong survival in patients with neuromuscular disease. In this article, we review indications for the initiation of noninvasive ventilation and mechanically assisted cough as well as provide recommendations for settings and titration. We discuss the evidence supporting the use of noninvasive ventilation as an adjunct to rehabilitation in patients with neuromuscular disease. Lastly, we review the ethical considerations that are relevant to decisions regarding initiation and cessation of noninvasive ventilation. While noninvasive ventilation and mechanically assisted cough have become standards of care in many forms of neuromuscular disease, most current recommendations are based on expert opinion rather than much-needed data from prospective clinical trials and we emphasize topics requiring future research.

IntroductionAir pollution has a significant impact on the morbidity and mortality of various respiratory diseases. However, this has not been widely studied in diffuse interstitial lung diseases, specifically in idiopathic pulmonary fibrosis.ObjectiveIn this study we aimed to assess the relationship between four major air pollutants individually [carbon monoxide (CO), nitrogen dioxide (NO2), ozone (O3), and nitrogen oxides (NOx)] and the development of chronic respiratory failure, hospitalization due to respiratory causes and mortality in patients with idiopathic pulmonary fibrosis.MethodsWe conducted an exploratory retrospective panel study from 2011 to 2020 in 69 patients with idiopathic pulmonary fibrosis from the pulmonary medicine department of a tertiary hospital. Based on their geocoded residential address, levels of each pollutant were estimated 1, 3, 6, 12, and 36 months prior to each event (chronic respiratory failure, hospital admission and mortality). Data was collected from the air quality monitoring stations of the Community of Madrid located

Abstract Background Home mechanical ventilation (HMV) is a viable and effective strategy for patients with chronic respiratory failure (CRF). The Chilean Ministry of Health started a program for adults in 2008. Methods This study examined the following data from a prospective cohort of patients with CRF admitted to the national HMV program: characteristics, mode of admission, quality of life, time in the program and survival. Results A total of 1105 patients were included. The median age was 59 years (44–58). Women accounted for 58.1% of the sample. The average body mass index (BMI) was 34.9 (26–46) kg/m2. A total of 76.2% of patients started HMV in the stable chronic mode, while 23.8% initiated HMV in the acute mode. A total of 99 patients were transferred from the children's program. There were 1047 patients on non-invasive ventilation and 58 patients on invasive ventilation. The median baseline PaCO2 level was 58.2 (52–65) mmHg. The device usage time was 7.3 h/d (5.8–8.8), and the time in HMV was 21.6 (12.2–49.5) months. The diagnoses were COPD (35%), obesity hypoventilation syndrome (OHS; 23.9%), neuromuscular disease (NMD; 16.3%), non-cystic fibrosis bronchiectasis or tuberculosis (non-CF BC or TBC; 8.3%), scoliosis (5.9%) and amyotrophic lateral sclerosis (ALS; 5.24%). The baseline score on the Severe Respiratory Insufficiency questionnaire (SRI) was 47 (± 17.9) points and significantly improved over time. The lowest 1- and 3-year survival rates were observed in the ALS group, and the lowest 9-year survival rate was observed in the non-CF BC or TB and COPD groups. The best survival rates at 9 years were OHS, scoliosis and NMD. In 2017, there were 701 patients in the children's program and 722 in the adult´s program, with a prevalence of 10.4 per 100,000 inhabitants. Conclusion The most common diagnoses were COPD and OHS. The best survival was observed in patients with OHS, scoliosis and NMD. The SRI score improved significantly in the follow-up of patients with HMV. The prevalence of HMV was 10.4 per 100,000 inhabitants. Trial registration This study was approved by and registered at the ethics committee of North Metropolitan Health Service of Santiago, Chile (N° 018/2021).

Background: 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. Objective: 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. Design: 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. Methods: 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. Results: 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. Conclusions: 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.

Abstract Background Various health‐related quality‐of‐life (HRQOL) tools are used to evaluate patients with chronic respiratory failure (CRF), but there is a relative lack of tools available for the evaluation of social support and treatment in these patients. The present study focused on the development of a systematic patient‐reported outcome measure (PROM) tool for use in patients with CRF. Methods The CRF‐PROM scale conceptual framework and item bank were generated after reviewing the corresponding literature and HRQOL scales, interviewing CRF patients and focus groups. After creation of the initial scale, the items in the scale were selected through two item selection theories, and the final scale was created. The reliability, validity and feasibility of the final scale were assessed. Results The CRF‐PROM scale includes four domains (i.e., physiological domain, psychological domain, social domain and therapeutic domain) and 10 dimensions. After the item selection process, the final scale included 50 items. Cronbach's α coefficients, which were all above 0.7, indicated the reliability of the scale. The results of structural validity met the relevant standards of confirmatory factor analysis. The response rates of the preinvestigation and the formal investigation were 93.3% and 97.6%, respectively. Conclusions The CRF‐PROM scale developed in the present study is effective and reliable. It could be used widely in the posthospital management of patients, in CRF studies and in clinical trials of new medical products and interventions. Patient or Public Contribution Participants from eight different hospitals and communities participated in the development or validation phase of the CRF‐PROM scale.

Maximilian Wollsching-Strobel, Sarah Bettina Schwarz, Tim Mathes, Daniel Sebastian Majorski, Pouya Heidari, Doreen Kroppen, Friederike Sophie Magnet, Wolfram Windisch Cologne Merheim Hospital, Department of Pneumology, Kliniken der Stadt Köln gGmbH, Witten/Herdecke University, Faculty of Health/School of Medicine, Cologne and Witten, North Rhine-Westphalia, GermanyCorrespondence: Wolfram WindischCologne Merheim Hospital, Department of Pneumology, Kliniken der Stadt Köln gGmbH, Witten/Herdecke University, Faculty of Health/School of Medicine, Ostmerheimer Str. 200, Cologne and Witten, North Rhine-Westphalia, D-51109, GermanyTel +49 221 8907 18929Fax +49 221 8907 8305Email windischw@kliniken-koeln.dePurpose: To assess the influence of anemia on health-related quality of life (HRQL) in COPD patients receiving long-term non-invasive ventilation (NIV).Patients and Methods: In this prospective single-center cohort study, COPD patients on long-term NIV were analyzed between June 2015 and May 2020. Linear multiple regression analyses were performed using the results of the Severe Respiratory Insufficiency Questionnaire (SRI) along with the following variables: sex, age, body mass index, duration of NIV, exacerbation history (≤ 1 versus > 1 in the previous year), the updated Charlson comorbidity index, hemoglobin levels and anemia (WHO criteria).Results: Anemia was identified in 32.8% (N=128). Anemia (mean difference − 8.4, 95% CI − 2.0/-14.9 SRI points, P=0.011) and exacerbations (mean difference − 9.9, 95% CI − 4.3/-15.5 SRI points, P=0.001) each had a negative impact on SRI summary scores. Exacerbations were negatively associated with six out of seven SRI subscale scores, while anemia was negatively associated with four out of seven. SRI summary scores dropped by 1.5 points for every g/dl of hemoglobin (P=0.08). No other variables had an influence on the SRI scores.Conclusion: The present study has shown that within a cohort of COPD patients undergoing long-term NIV, one-third were identified as anemic. Furthermore, anemia, like exacerbation history, was found to have a considerable negative impact on HRQL that is specific to patients with chronic respiratory failure.Clinical Trial Registration: German Clinical Trials Registry (DRKS00008759).Keywords: chronic respiratory failure, exacerbations, hemoglobin, non-invasive ventilation, severe respiratory insufficiency questionnaire

Sarah Gephine,1,2 Patrick Mucci,1 Jean-Marie Grosbois,3 François Maltais,2 Didier Saey2 1University Lille, University Artois, University Littoral Côte d’Opale, ULR 7369 - URePSSS - Unité de Recherche Pluridisciplinaire Sport Santé Société, Lille, F-59000, France; 2Centre de Recherche, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Université Laval, Québec, Canada; 3Formaction Santé, Pérenchies, FranceCorrespondence: Didier SaeyInstitut Universitaire de Cardiologie et de Pneumologie de Québec, Université Laval, 2725 Chemin Sainte-Foy, Québec, QC, G1V 4G5 CanadaEmail Didier.saey@rea.ulaval.caBackground: The prevalence of physical frailty and its clinical characteristics in advanced chronic obstructive pulmonary disease (COPD) is unknown, as well as the usefulness of functional capacity tests to screen for physical frailty. The aim of the study was to evaluate the proportion and clinical portrait of COPD patients with chronic respiratory failure exhibiting physical frailty at the time of referral to home-based pulmonary rehabilitation. We also evaluate the usefulness of the short physical performance battery (SPPB) and timed-up and go (TUG) as potential screening tools for physical frailty. Finally, we evaluated the specific contribution of gait speed to the frailty Fried total score.Methods: This was a prospective observational study in which physical frailty was defined using Fried criteria (body mass loss, exhaustion, low physical activity, slower walking and weakness). Clinical portrait was documented from daily physical activity, exercise tolerance, functional capacity, anxiety and depressive symptoms, health-related quality of life, and fatigue scores. The ability of the SPPB and TUG to predict physical frailty was investigated using receiver operating characteristic curves. Contribution of each Fried criteria was evaluated with a principal component analysis (PCA).Results: Amongst the 44 included participants (FEV1, 33 ± 13% of predicted), 19 were physically frail. Frail individuals had lower daily steps number, exercise tolerance and functional capacity, and higher fatigue, anxiety, and depressive symptom scores (p< 0.05) compared to non-frail individuals. SPPB and TUG did not have an acceptable detection accuracy for screening physical frailty. PCA indicated that gait speed was the main contributor to the Fried total score of physical frailty.Conclusion: Physical frailty affects a large proportion of COPD patients with chronic respiratory failure starting a home-based intervention and was associated with worse clinical status. Although the present results need to be confirmed by adequately powered studies, gait speed seems to have the potential to become a simple screening tool for physical frailty in this population.Keywords: frailty, chronic respiratory failure, pulmonary rehabilitation, gait speed

Purpose: To investigate the long-term changes in body weight and serum albumin levels in patients with respiratory failure, and those with chronic heart failure, who were treated with home long-term oxygen therapy (LTOT) to understand the current status and contribute to future measures. Methods: Patients with chronic obstructive pulmonary disease (COPD), those with interstitial pneumonia (IP), and those with chronic heart failure (CHF) undergoing home LTOT for 6 months or more between January 2011 and January 2019 were included in the study. Body weight and serum albumin levels were assessed at the start of home LTOT and at the end of the observation period, a minimum of 6 months after commencing home LTOT. Results: Sixty-two patients (29 COPDs, 23 IPs, and 10 CHFs) were included. In COPD patients and IP patients, body weight decreased (P = 0.0017, P = 0.0018, respectively, Wilcoxon signed-rank test). Serum albumin levels decreased in IP patients (P = 0.0185) but not in COPD patients. There was neither significant decrease in body weight nor serum albumin levels in patients with CHF. Conclusion: Chronic respiratory failure patients who have home LTOT were likely to have a decreased nutritional status. In order to provide prolonged home LTOT, medical staff need to pay close attention to the nutritional status of patients receiving home LTOT.

Michele Vitacca,1 Alberto Malovini,2 Bruno Balbi,3 Maria Aliani,4 Serena Cirio,5 Antonio Spanevello,6,7 Claudio Fracchia,8 Mauro Maniscalco,9 Giacomo Corica,10 Nicolino Ambrosino,8 Mara Paneroni1 1Istituti Clinici Scientifici Maugeri IRCCS, Respiratory Rehabilitation Unit of the Institute of Lumezzane, Brescia, Italy; 2Istituti Clinici Scientifici Maugeri IRCCS, Laboratory of Informatics and Systems Engineering for Clinical Research of the Institute of Pavia, Pavia, Italy; 3Istituti Clinici Scientifici Maugeri IRCCS, Respiratory Rehabilitation Unit of the Institute of Veruno, Novara, Italy; 4Istituti Clinici Scientifici Maugeri IRCCS, Respiratory Rehabilitation Unit of the Institute of Cassano Delle Murge, Bari, Italy; 5Istituti Clinici Scientifici Maugeri IRCCS, Respiratory Rehabilitation Unit of the Institute of Pavia, Pavia, Italy; 6Istituti Clinici Scientifici Maugeri IRCCS, Respiratory Rehabilitation Unit of the Institute of Tradate, Varese, Italy; 7University of Insubria, MACRO, Varese, Italy; 8Istituti Clinici Scientifici Maugeri IRCCS, Respiratory Rehabilitation Unit of the Institute of Montescano, Pavia, Italy; 9Istituti Clinici Scientifici Maugeri IRCCS, Respiratory Rehabilitation Unit of the Institute of Telese, Benevento, Italy; 10Istituti Clinici Scientifici Maugeri IRCCS, Health Directorate of the Institute of Lumezzane, Brescia, ItalyCorrespondence: Michele VitaccaIstituti Clinici Scientifici Maugeri IRCCS, Via Salvatore Maugeri, 4, Pavia 27100, ItalyEmail michele.vitacca@icsmaugeri.itBackground: The Barthel Index dyspnea (BId) is responsive to physiological changes and pulmonary rehabilitation in patients with chronic obstructive pulmonary disease (COPD). However, the minimum clinically important difference (MCID) has not been established yet.Aim: To identify the MCID of BId in patients with COPD stratified according to the presence of chronic respiratory failure (CRF) or not.Materials and Methods: Using the Medical Research Council (MRC) score as an anchor, receiver operating characteristic curves and quantile regression were retrospectively evaluated before and after pulmonary rehabilitation in 2327 patients with COPD (1151 of them with CRF).Results: The median post-rehabilitation changes in BId for all patients were − 10 (interquartile range = − 17 to − 3, p< 0.001), correlating significantly with changes in MRC (r = 0.57, 95% CI = 0.53 to 0.59, p< 0.001). Comparing different methods of assessment, the MCID ranged from − 6.5 to − 9 points for patients without and − 7.5 to − 12 points for patients with CRF.Conclusion: The most conservative estimate of the MCID is − 9 points in patients with COPD, without and − 12 in those with CRF. This estimate may be useful in the clinical interpretation of data, particularly in response to intervention studies.Keywords: activities of daily life, breathlessness, dyspnea, chronic respiratory failure, exercise training, health related quality of life, rehabilitation

Abstract Background It has been shown that home mechanical ventilation improves quality of life, but it has not been widely studied which particular patient groups benefit the most from starting this type of therapy. The purpose of this prospective observational study was to evaluate quality of life change patterns 6 months after initiation of home mechanical ventilation in patients suffering from chronic respiratory failure using patient reported outcomes. Methods We enrolled 74 chronic respiratory failure patients starting invasive or noninvasive home mechanical ventilation through the Semmelweis University Home Mechanical Ventilation Program. Quality of life was evaluated at baseline and at 6 months after initiation of home mechanical ventilation using the Severe Respiratory Insufficiency Questionnaire. Results Overall quality of life showed 10.5% improvement 6 months after initiation of home mechanical ventilation (p

Objective: to evaluate the expression levels of miRNA (miR-27, miR-101, miR-142, miR-339 and miR-424) and its relationship with clinical and laboratory parameters in lung transplant recipients. Materials and methods. The study included 57 lung recipients aged 10 to 74 years (35 ± 15), including six children (9%) – four boys 10, 12, 13 and 17 years and girls 13 and 14 years old – and 51 adult recipients, including 30 men (62.5%). The control group was made up of 14 healthy individuals that were not significantly different by gender and age. Expression levels of the microRNAs studied in blood plasma were determined via quantitative polymerase chain reaction (PCR). Correlations of miRNA expression levels with complete blood count and biochemical blood test indicators were analyzed. Results. Patients with end-stage chronic respiratory failure (potential lung recipients) were found to have significantly higher expression levels of miR-27, miR-101 and miR-339 in plasma than the healthy individuals (p = 0.02, p = 0.03 and p = 0.01, respectively). The expression level of miR-339 correlated with the age of potential lung recipients (p = 0.04). It was a negative correlation (r = –0.46). The expression levels of the other four miRNAs were age independent. The average expression level of miR-424 in lung recipients in the long-term period after lung transplant was higher than in waitlisted patients (p = 0.03). Analysis of the relationship between miRNA expression levels and external respiration function in the long-term post-transplant period showed that miR-142 expression level (r = 0.61; p = 0.04) positively correlates with the Tiffeneau-Pinelli index. This strong correlation, which exceeds 85%, indicates the presence of restrictive lung diseases. A year and more after transplantation, it was found that in the recipients, there were close positive correlations between miR-27, miR-142, miR-424 expression levels and blood leukocyte concentration, as well as between the miR-142 expression level and the sCD40L concentration during this period. Conclusion. A comparative study of the expression level of miRNAs (miR-27, miR-101, miR-142, miR-339 and miR-424) in the blood plasma of patients suffering from end-stage chronic lung diseases of various origin and in lung recipients enables us to conclude that further studies of the miRNA panels are needed in order to assess their effectiveness as potential molecular and genetic markers of post-transplant complications.

MicroRNAs are small RNA molecules stable in blood serum (plasma) samples. Their level of expression is associated with the severity and nature of physiological and pathological processes in the body.Aim: to evaluate the expression levels of five microRNAs (miR-27, miR-101, miR-142, miR-339 and miR-424) in potential lung recipients with end-stage chronic lung diseases of various etiologies.Materials and methods. The study included 16 patients with end-stage chronic lung diseases (potential lung recipients) aged 4 to 74 years (average 36 ± 18). Among them were two children (12.5%) – girls aged 4 and 14 years, and 14 adults aged from 21 to 74 (40 ± 16) years – 6 men (42.9%) and 8 women. The control group consisted of 12 healthy individuals. The main diseases that caused severe respiratory failure were: cystic fibrosis (n = 5), primary pulmonary hypertension (PPH; n = 4), pulmonary fibrosis of various etiologies (idiopathic pulmonary fibrosis – 1; pulmonary fibrosis associated with exogenous allergic alveolitis – 1; radiation-induced pulmonary fibrosis – 1), lymphangioleiomyomatosis (n = 2), histiocytosis (n = 1) and pulmonary emphysema (n = 1). MicroRNA expression was detected through real-time PCR. The level of microRNA expression in plasma was estimated in accordance with instructions for reagent kits (Qiagen, USA).Results. The levels of miR-27, miR-101 and miR-339 in potential lung recipients were significantly higher than in healthy individuals. The levels differed depending on the etiology of diseases: the levels of miR-27, miR-101, miR-142 and miR-339 were higher in patients with cystic fibrosis than in healthy individuals; in patients with other lung diseases, only miR-101 levels where higher than in healthy individuals. The miR-424 level in healthy individuals did not differ from that in potential lung recipients or in subgroups.Conclusion. Results obtained show the features of a number of microRNA levels (miR-27, miR-101, miR-142, and miR-339) under certain lung diseases and suggest a possibility of a diagnostic value in patients with chronic respiratory failure during pre-transplant examination.

Esophageal pressure (Pes) monitoring is performed during polysomnography (PSG) with a thin, water-filled catheter connected to a transducer. The resulting quantitative assessment of respiratory effort can aid in the accurate diagnosis of sleep-related breathing disorders. This was a prospective observational study using Pes in PSG for thirty patients with chronic respiratory failure (CRF) conducted in the Department of Pulmonary, Critical Care and Sleep Medicine at a tertiary care center of North India. Sleep Scoring was done by conventional method and using esophageal manometry and compared- Polysomnography normal without esophageal manometry recording (PSGN) and polysomnography with esophageal manometry scoring (PSGE). AHI index was similar in both groups. However, RERAs were diagnosed easily using Pes resulting in significant increase in RDI and even reclassification in terms of severity of sleep apnea. Besides, Pes was also useful to distinguish obstructive from central hypopnea which cannot be distinguished by routine PSG which can help guide therapy particularly in chronic respiratory failure patients with hypoventilation. Such patients with hypoventilation often require bilevel positive airway pressure as ventilatory support. Central hypopneas and apneas with hypercapnia may require a higher-pressure support, a backup rate or even advanced volume assured modes of ventilation. Thus, it can be concluded that Pes in PSG remains a safe and generally well-tolerated procedure. Use of Pes aids to detect RERA and thereby respiratory disturbance index (RDI); a better marker of Sleep related breathing disorder rather than AHI. It also helps in differentiate between obstructive and central hypopnea.

This review focuses on the delivery of non-invasive ventilation—i.e., intermittent positive-pressure ventilation—in children lasting more than 3 months. Several recent reviews have brought to light a dramatic escalation in the use of long-term non-invasive ventilation in children over the last 30 years. This is due both to the growing number of children receiving care for complex and severe diseases necessitating respiratory support and to the availability of LT-NIV equipment that can be used at home. While significant gaps in availability persist for smaller children and especially infants, home LT-NIV for children with chronic respiratory insufficiency has improved their quality of life and decreased the overall cost of care. While long-term NIV is usually delivered during sleep, it can also be delivered 24 h a day in selected patients. Close collaboration between the hospital complex-care team, the home LT-NIV program, and family caregivers is of the utmost importance for successful home LT-NIV. Long-term NIV is indicated for respiratory disorders responsible for chronic alveolar hypoventilation, with the aim to increase life expectancy and maximize quality of life. LT-NIV is considered for conditions that affect respiratory-muscle performance (alterations in central respiratory drive or neuromuscular function) and/or impose an excessive respiratory load (airway obstruction, lung disease, or chest-wall anomalies). Relative contraindications for LT-NIV include the inability of the local medical infrastructure to support home LT-NIV and poor motivation or inability of the patient/caregivers to cooperate or understand recommendations. Anatomic abnormalities that interfere with interface fitting, inability to protect the lower airways due to excessive airway secretions and/or severely impaired swallowing, or failure of LT-NIV to support respiration can lead to considering invasive ventilation via tracheostomy. Of note, providing home LT-NIV during the COVID 19 pandemic has become more challenging. This is due both to the disruption of medical systems and the fear of contaminating care providers and family with aerosols generated by a patient positive for SARS-CoV-2 during NIV. Delay in initiating LT-NIV, decreased frequency of home visits by the home ventilation program, and decreased availability of polysomnography and oximetry/transcutaneous PCO2 monitoring are observed. Teleconsultations and telemonitoring are being developed to mitigate these challenges.

Home mechanical ventilation (HMV) is a method of treatment in children with sleep-disordered breathing (SDB) and alveolar hypoventilation regardless of primary disease. The goal of the study was to describe the changes in the HMV program in Serbia during the last two decades. Cross-sectional retrospective study included data from the national HMV database from 2001 until 2019. HMV was initiated in clinically stable patients after the failure to wean from mechanical ventilation succeeded acute respiratory deterioration or electively after the confirmation of SDB and alveolar hypoventilation by sleep study or continuous transcutaneous capnometry and oximetry. The study included 105 patients (50 ventilated noninvasively and 55 ventilated invasively via tracheostomy). The median age at the time of HMV initiation was 6.2 years (range: 0.3–18 years). Invasive ventilation had been initiated significantly earlier than noninvasive ventilation (NIV) (p < 0.01), without difference in duration of ventilatory support (p = 0.95). Patients on NIV were significantly older (p < 0.01) than those ventilated invasively (13 and 1.5 years, respectively). Average waiting time on equipment had been shortened significantly—from 6.3 months until 2010 to 1 month at the end of the study (p < 0.01). Only 6.6% of patients had obstructive sleep apnea syndrome (OSAS) requiring HMV. During the study period, 24% patients died, mostly due to uncontrolled infection or progression of underlying disease. Availability and shortened waiting time for the equipment accompanied by advanced overall health care led to substantial improvements in the national HMV program. However, future improvements should be directed to systematic evaluation of SDB in patients with OSAS, early diagnosis of nocturnal hypoventilation, and subsequent timely initiation of chronic ventilation.

INTRODUCTION[|]Domiciliary non-invasive mechanical ventilation (NIMV) devices are increasingly used in patients with chronic obstructive pulmonary disease (COPD) related chronic respiratory failure (CRF). In this study, device compliance of COPD patients with and without emphysema was assessed.[¤]METHODS[|]In an observational cross-sectional study, 2014-2018, who applied to respiratory intensive care unit (ICU) outpatient clinic using domiciliary NIMV with chronic respiratory failure diagnosis patients with COPD who had a thorax CT within the past year were taken into the study. Demographic information, comorbidities, NIMV mode and duration of use, respiratory function test, arterial blood gas, whole blood count, C-reactive protein (CRP) and complications were recorded from outpatient clinic records. Both groups were compared in terms of the parameters examined.[¤]RESULTS[|]Forty patients (M, 75%) with a median age of 66 were included in the study. There was no difference between the groups in terms of NIMV compliance and NIMV pressures in the study. In the emphysema group, active smoking was statistically higher (p=0.026) and FEV1, FEV1/FVC and PEF25-75 were found to be lower. The comorbidities of both groups were similar and as a complication redness of the skin due to mask pressure was found in a patient in the emphysema group. There was no difference between groups in terms of arterial blood gas and inflammatory markers.[¤]DISCUSSION AND CONCLUSION[|]Because of the clinical benefit for patients, using of the NIMV is one of the ideal hours for home use. COPD subtypes do not make a difference on NIMV compliance. [¤]

Abstract Gene expression profiles of patients with progressive sarcoidosis, most of whom had evidence of fibrosis on imaging, have been reported to be similar to those of patients with inflammatory hypersensitivity pneumonitis, while expression profiles in progressive sarcoidosis did not resemble those of idiopathic pulmonary fibrosis. However, it is not known whether specific parenchymal features discerned on computed tomography (CT) imaging can predict development of fibrosis in pulmonary fibrosis. We herein describe a rare case of pulmonary sarcoidosis with honeycomb lung‐like structures developing as a result of concentration of traction bronchiectasis distally, predominantly in both lower lung fields, which developed through shrinkage of consolidations comprising a “central–peripheral band” detected in a woman in her 60s, with non‐caseating epithelioid granuloma. To our knowledge, this is the first case demonstrating the distinctive morphology and developmental process of honeycomb lung‐like structures in fibrotic pulmonary sarcoidosis.

Abstract Background Home mechanical ventilation is an established treatment for chronic respiratory failure resulting in improved survival and quality of life. Technological advancement, evolving health care reimbursement systems and newly implemented national guidelines result in increased utilization worldwide. Prevalence shows great geographical variations and data on East-Central European practice has been scarce to date. The aim of the current study was to evaluate prevalence and characteristics of home mechanical ventilation in Hungary. Methods We conducted a nationwide study using an online survey focusing on patients receiving ventilatory support at home. The survey focused on characterization of the site (affiliation, type), experience with home mechanical ventilation, number of patients treated, indication for home mechanical ventilation (disease type), description of home mechanical ventilation (invasive/noninvasive, ventilation hours, duration of ventilation) and description of the care provided (type of follow up visits, hospitalization need, reimbursement). Results Our survey uncovered a total of 384 patients amounting to a prevalence of 3.9/100,000 in Hungary. 10.4% of patients received invasive, while 89.6% received noninvasive ventilation. The most frequent diagnosis was central hypopnea syndromes (60%), while pulmonary (20%), neuromuscular (11%) and chest wall disorders (7%) were less frequent indications. Daily ventilation need was less than 8 h in 74.2%, between 8 and 16 h in 15.4% and more than 16 h in 10.4% of patients reported. When comparing sites with a limited ( 50 patients) case number, we found the former had significantly higher ratio of neuromuscular conditions, were more likely to ventilate invasively, with more than 16 h/day ventilation need and were more likely to provide home visits and readmit patients (p

Birsen Ocakli, Eylem Acarturk, Emine Aksoy, Sinem Gungor, Fulya Ciyiltepe, Selahattin Oztas, Ipek Ozmen, Meltem Coban Agca, Cuneyt Salturk, Nalan Adiguzel, Zuhal Karakurt Department of Chest Diseases, Sureyyapasa Chest Diseases and Thoracic Surgery Training and Research Hospital, Istanbul, Turkey Purpose: The aim of this study was to evaluate the impact of exposure to biomass smoke vs cigarette smoke on serum inflammatory markers and pulmonary function parameters in patients with chronic respiratory failure (CRF).Patients and methods: A total of 106 patients with CRF divided into age and gender-matched groups of cigarette-smoke exposure (n=55, mean [SD] age: 71.0 [12.0] years, 92.7% were females) and biomass smoke exposure (n=51, mean [SD] age: 73.0 [11.0] years, 94.1% were females) were included in this retrospective study. Data on patient demographics (age and gender), inflammatory markers, including neutrophil-to-lymphocyte ratio, C-reactive protein, platelet/mean platelet volume ratio, arterial blood gas analysis, and pulmonary function test findings, including forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), and FEV1/FVC were obtained from medical records.Results: Carbon dioxide partial pressure levels were significantly higher in the biomass smoke exposure than in the cigarette smoke exposure group (mean [SD] 51.0 [8.0] vs 47.0 [8.0] mmHg, p=0.026, respectively). Spirometry revealed similarly low levels for FEV1 (%) (38.0 [16.0] vs 40.0 [12.0]%) and FVC (%) (45.0 [19.0] vs 39.0 [19.0]%) in cigarette-smoke and biomass smoke exposure groups, whereas biomass smoke exposure was associated with significantly higher FEV1/FVC (75.0 [14.0] vs 58.0 [12.0]%, p=0.001), lower FVC (mL) (mean [SD] 744.0 [410.0] vs 1,063.0 [592.0] mL, p=0.035) and lower percentage of patients with FEV1/FVC 70% and more prominent loss of vital capacity than cigarette smoke exposure seems to indicate the likelihood of at least 18 years of biomass exposure to be sufficiently high to be responsible for both obstructive and restrictive pulmonary diseases. Keywords: biomass exposure, cigarette smoke, inflammatory markers, spirometry, chronic respiratory failure

Eylem Acartürk Tunçay, Zuhal Karakurt, Emine Aksoy, Cuneyt Saltürk, Sinem Gungor, Nezihe Ciftaslan, İlim Irmak, Dilek Yavuz, Birsen Ocakli, Nalan Adıgüzel Respiratory Intensive Care Unit, Sureyyapaşa Chest Diseases and Thoracic Surgery Education and Research Hospital, University of Health Sciences, Istanbul, Turkey Aim: Increased dyspnea, sputum volume, and purulence are subjective symptoms in COPD patients. To diagnose COPD exacerbations with chronic respiratory failure (CRF) and to assess the requirement for antibiotic treatment, physicians require more objective criteria. We aimed to investigate whether neutrophil-to-lymphocyte ratio (NLR) can be used as an infectious exacerbation marker in COPD patients with CRF.Patients and methods: This retrospective cross-sectional study was performed in the intensive care outpatient clinic of a tertiary training hospital between 2014 and 2015. Patients admitted with CRF due to COPD and who had complete blood count (CBC) results were enrolled. CBC results and C-reactive protein (CRP) levels were obtained from the hospital online database. The “modified exacerbation model (MEM)” was defined as follows: exacerbation A, leukocytes ≥12,000/mm3, CRP >10 mg/dL; exacerbation B, leukocytes ≥10,000/mm3, CRP >10 mg/dL; exacerbation C, leukocytes ≥10,000/mm3, CRP >8 mg/dL; exacerbation D, leukocytes ≥10,000/mm3, CRP >5 mg/dL. The cutoff value of NLR was defined for each model. Patients were split into two groups based on the NLR cutoff value according to the “NLR exacerbation model” and further subgrouped according to peripheral eosinophil percentage (eosinophils ≥2% and

Luqian Zhou,1,* Xiaoying Li,1,* Lili Guan,1,* Jianhua Chen,1 Bingpeng Guo,1 Weiliang Wu,1 Yating Huo,1 Ziqing Zhou,1 Zhenyu Liang,1 Yuqi Zhou,2 Jie Tan,3 Xin Chen,4 Yuanlin Song,5 Rongchang Chen1 1Department of Respiratory Medicine, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Disease, The First Affiliated Hospital of Guangzhou Medical University, 2Department of Respiratory Medicine, The Third Affiliated Hospital of Sun-Yat Sen University, 3Department of Respiratory Medicine, The First Affiliated Hospital, School of Clinical Medicine of Guangzhou Pharmaceutical University, 4Department of Respiratory Medicine, Zhujiang Hospital of Southern Medical University, Guangzhou, 5Department of Pulmonary Medicine, Zhongshan Hospital, Fudan University, Shanghai, People’s Republic of China *These authors contributed equally to this work Background: The benefits of noninvasive positive pressure ventilation (NPPV) in patients with hypercapnic COPD are controversial. It is presumed that methodology and appropriate use of NIV ventilator might be crucial for the outcomes. With the new built-in software, the performance of NIV can be monitored at home, which can guarantee the compliance and appropriate use. This study investigated effects of home use of NIV in hypercapnia in COPD patients using the NIV ventilator with built-in software for monitoring. Methods: The current multicenter prospective, randomized, controlled trial enrolled patients with stable GOLD stages III and IV hypercapnic COPD. Patients were randomly assigned via a computer-generated randomization sequence, with a block size of four patients, to continue optimized treatment (control group) or to receive additional NPPV (intervention group) for 3 months. The primary outcome was arterial carbon dioxide pressure (PaCO2). Data were derived from built-in software and analyzed every 4 weeks. Analysis was carried out with the intention to treat. This study is registered with ClinicalTrials.gov, number NCT02499718. Results: Patients were recruited from 20 respiratory units in China from October 1, 2015, and recruitment was terminated with a record of the vital statistics on May 31, 2016. A total of 115 patients were randomly assigned to the NPPV group (n=57) or the control group (n=58). Patients complied well with NPPV therapy (mean [± standard deviation] day use 5.6±1.4 h). The mean estimation of leaks was 37.99±13.71 L/min. The changes in PaCO2 (-10.41±0.97 vs -4.32±0.68 mmHg, P=0.03) and 6-min walk distance (6MWD) (38.2% vs 18.2%, P=0.02) were statistically significant in the NPPV group versus the control group. COPD assessment test (CAT) showed a positive trend (P=0.06) in favor of the NPPV group. Pulmonary function and dyspnea were not different between groups. Conclusion: Ventilators equipped with built-in software provided methodology for monitoring NIV use at home, which could facilitate the improvement of compliance and quality control of NIV use. It was shown that three months use of NIV at home could reduce the PaCO2 and improve exercise tolerance (6MWD) in chronic hypercapnic COPD patients. Keywords: COPD, noninvasive positive pressure ventilation, long-term oxygen therapy, chronic respiratory failure, built-in software

Objective: In this study it was aimed to share experiences and to draw attention to the problems about home mechanical ventilation (HMV) which is used in the treatment of patients with chronic respiratory failure and is increasingly becoming widespread all over the world. Materials and Methods: The medical records of the patients having HMV due to chronic respiratory failure between January 2009 and March 2016 at Erciyes University Medical Faculty Pediatric Intensive Care Unit were retrospectively analyzed. Results: Twenty nine patients (14 males, 15 females) with a median age of 29 months (8.5-127.5) to start HMV application were included in this study. The median hospitalization time of the patients was 75 days (44-115) and the median follow up time after discharge was 110 days (52.5-510). Twenty-five (86.2%) patients had been followed up due to neurologic diseases, 4 patients (13.8%) due to lung diseases. Twenty five (86.2%) patients (13 males, 12 females) with a median age of 17 month (8-93) received invasive mechanical ventilation and 4 (13.8%) patients (2 males, 2 females) with a median age of 162 month (73.5-189) received non-invasive mechanical ventilation. Conclusion: In our country pediatric intensive care bed capacity is limited and HMV is a good option for alleviating the burden of chronic patients.

Abstract Information and Communication Technologies applied to health care and advances in sensor and data transmission technology allowed tele-medicine based programs of care also for patients with respiratory diseases. Different sensors, transmission devices and interventions are used in tele-medicine for some indications. Patients suffering from Chronic Obstructive Pulmonary Disease, asthma, neuromuscular diseases, ventilator assisted individuals and those undergoing pulmonary rehabilitation programs may benefit from this approach. The legal problems are still unsolved. Economic advantages for health care systems, though potentially high, are still poorly investigated. Despite the hopes, we need more evidence before this modality can be considered as a real progress in the management of patients with respiratory diseases. On one hand, these technologies can improve the care of patients with difficult access to services, particularly those in rural/remote areas, on the other hand, there is the risk that they will be used only to reduce standard services in health systems of developed countries.

Hilal Altinoz,1 Nalan Adiguzel,2 Cuneyt Salturk,2 Gokay Gungor,2 Ozlem Mocin,2 Huriye Berk Takir,2 Feyza Kargin,2 Merih Balci,2 Oner Dikensoy,1 Zuhal Karakurt2 1Pulmonary Division, Acibadem University School of Medicine, 2Sureyyapasa Thoracic Diseases and Thoracic Surgery Training Hospital, Istanbul, Turkey Abstract: Cachexia is known to be a deteriorating factor for survival of patients with chronic obstructive pulmonary disease (COPD), but data related to obesity are limited. We observed that obese patients with COPD prescribed long-term noninvasive mechanical ventilation (NIMV) had better survival rate compared to nonobese patients. Therefore, we conducted a retrospective observational cohort study. Archives of Thoracic Diseases Training Hospital were sought between 2008 and 2013. All the subjects were prescribed domiciliary NIMV for chronic respiratory failure secondary to COPD. Subjects were grouped according to their body mass index (BMI). The first group consisted of subjects with BMI between 20 and 30 kg/m2, and the second group consisted of subjects with BMI >30 kg/m2. Data obtained at the first month’s visit for the following parameters were recorded: age, sex, comorbid diseases, smoking history, pulmonary function test, 6-minute walk test (6-MWT), and arterial blood gas analysis. Hospital admissions were recorded before and after the domiciliary NIMV usage. Mortality rate was searched from the electronic database. Overall, 118 subjects were enrolled. Thirty-eight subjects had BMI between 20 and 30 kg/m2, while 80 subjects had BMI >30 kg/m2. The mean age was 65.8±9.4 years, and 81% were male. The median follow-up time was 26 months and mortality rates were 32% and 34% for obese and nonobese subjects (P=0.67). Improvement in 6-MWT was protective against mortality. In conclusion, survival of obese patients with COPD using domiciliary NIMV was found to be better than those of nonobese patients, and the improvement in 6-MWT in such patients was found to be related to a better survival. Keywords: chronic respiratory failure, survival, body mass index, mortality, therapy

Background: Clinically, multidrug-resistant Acinetobacter baumannii (MDR-AB) recurrence is found in some patients although identified as successfully eradicated. We aim to discover the characteristics of patients with MDR-AB recurrence in the respiratory tract. Methods: We retrospectively collected 106 chronic respiratory failure patients with MDR-AB harvest in pulmonary secretion culture. Results: MDR-AB was successfully eradicated in 69 patients. Diabetes mellitus (p = 0.030, odds ratio [OR]: 2.7, 95% confidence interval [CI]: 1.1–6.4) and acute respiratory distress syndrome (p = 0.001, OR = 4.8, 95% CI: 1.8–12.7) reduce the MDR-AB eradication rate. Besides, a classification of colonization or infection was made beyond the 69 MDR-AB eradicated patients. In the colonization group, diabetes mellitus (p = 0.009; OR = 5.1, 95% CI: 1.5–17.6) is the only independent factor to increase the recurrence rate. Glycated hemoglobin level is also analyzed for each group to investigate diabetes control effect, but no significant difference found. Conclusions: Diabetes mellitus is a risk factor of MDR-AB recurrence among MDR-AB-colonized patients; the impact of localized pneumonia patch in MDR-AB-infected patients requires further study to be clarified.

Objectives. The possibility of prolonging and augmenting the quality of life of respiratory patients in end-stage disease and in particular neuromuscular disease patients depends on an increased level of care. Amyotrophic lateral sclerosis (ALS) is a neurological disease characterized by a fast progressive impairment of respiratory function which leads to mechanical ventilation and high burden of care. A specifically designed questionnaire was administered to caregivers to analyze the level of care provided to these patients in our districts. Patients and methods. The study population consisted of 70 patients (44 males and 26 females). Of the 70 patients, 46 were being treated with non-invasive ventilation and 24 with invasive mechanical ventilation. The questionnaire was divided in two parts: questions 1 to 13 investigated life conditions and 14 to 22 the quality of care provided. Results. The patients studied present a heavy burden of care and this is in relation to the quantity of respiratory aids prescribed. The symptom which created statistically significant problems for care was breathlessness, as is often reported in the literature. The presence of homecare tended to create conditions of greater serenity for the caregivers. Conclusions. This study shows that the burden of care in end-stage ALS patients is very onerous, and it can help to point out problems not sufficiently contemplated in healthcare planning.

Background: For patients with chronic respiratory failure (CRF) who are treated with noninvasive positive pressure ventilation (NPPV), a little is known regarding the effects of low-intensity NPPV (LI-NPPV) on the clinical course of CRF and the frequency of adjustments in these patients. Objectives: This study investigated the effects of LI-NPPV on the clinical course of patients with CRF as compared with patients who were treated with conventional NPPV (C-NPPV) and determined how frequently NPPV was adjusted during therapy. Methods: Clinical data from 21 patients who received long-term NPPV were retrospectively analyzed. Patients were categorized into two groups based on the level of initial pressure support (PS): C-NPPV group (PS ≥ 10 cm H2O) and LI-NPPV group (PS < 10 cm H2O). Results: Patients in the LI-NPPV group had significantly more exacerbations of CRF (P < 0.05). There was no significant difference in the number of patients who required adjustments of NPPV settings between the two groups. There was no significant difference in PaCO2levels 1 month after the start of NPPV between the two groups; however, PaCO2levels were significantly lower after 1 year in the C-group (P < 0.001). Seventy-one percent of LI-NPPV patients and 43% of C-NPPV patients needed NPPV adjustments. Conclusions: Attention should be paid to CRF patients who are initially administered LI-NPPV; they should be carefully observed because they can develop more exacerbations of CRF than patients undergoing C-NPPV. If possible, higher initial PS should be administered to prevent CRF exacerbations.

Few studies have analyzed the prevalence and accessibility of home mechanical ventilation (HMV) in Italy. We aimed to investigate the prevalence and prescription variability of HMV as well as of long-term oxygen therapy (LTOT) and continuous positive airway pressure (CPAP), in the Lombardy Region. Prescribing rates of HMV (both noninvasive and tracheostomies), CPAP (auto-CPAP, CPAP/other sleep machines) and LTOT (liquid-O2, O2-gas, concentrators) in the 15 Local Healthcare districts of Lombardy were gathered from billing data for 2012 and compared. Crude rates (per 100,000 population) and rates for the different healthcare districts were calculated. In 2012, 6325 patients were on HMV (crude prescription rate: 63/100,000) with a high variation across districts (8/100,000 in Milano 1 vs 150/100,000 in Pavia). There were 14,237 patients on CPAP (crude prescription rate: 142/100,000; CPAP/other sleep machines 95.3% vs auto-CPAP 4.7%) with also high intra-regional variation (56/100,000 in Mantova vs. 260/100,000 in Pavia). There were 21,826 patients on LTOT (prescription rate: 217/100,000 rate; liquid-O2 94%, O2-gas 2.08%, O2-concentrators 3.8%), with again high intra-regional variation (100/100,000 in Bergamo vs 410/100,000 in Valle Camonica). The crude rate of HMV prescriptions in Lombardy is very high, with a high intra-regional variability in prescribing HMV, LTOT and CPAP which is partly explainable by the accessibility to specialist centers with HMV/sleep-study facilities. Analysis of administrative data and variability mapping can help identify areas of reduced access for an improved standardization of services. An audit among Health Payer and prescribers to interpret the described huge variability could be welcomed.

Introduction: Nocturnal Non-invasive Positive Pressure Ventilation (NPPV) is the treatment of choice in patients with chronic hypercapnic respiratory failure due to hypoventilation. Continuous oxygen saturation measured with a pulse oximeter provides a surrogate measure of arterial oxygen saturation but does not completely reflect ventilation. Currently, Partial Pressure of Arterial (PaCO2) measured by arterial blood analysis is used for estimating the adequacy of ventilatory support and serves as the gold standard Aim: To examine the safety, feasibility and utility of cutaneous capnography to re-titrate the non-invasive positive pressure ventilation settings in patients with chronic hypercapnic respiratory failure due to hypoventilation. Materials and Methods: Twelve patients with chronic hypercapnic respiratory failure prospectively underwent complete polysomnography and cutaneous capnography measurement on the ear lobe. Non-invasive ventilation pressures were adjusted with the aim of normalizing cutaneous carbon dioxide or at least reducing it by 10 to 15 mmHg. Sensor drift for cutaneous carbon dioxide of 0.7 mmHg per hour was integrated in the analysis. Results: Mean baseline cutaneous carbon dioxide was 45.4 + 6.5 mmHg and drift corrected awake value was 45.1 + 8.3 mmHg. The correlation of baseline cutaneous carbon dioxide and the corrected awake cutaneous carbon dioxide with arterial blood gas values were 0.91 and 0.85 respectively. Inspiratory positive airway pressures were changed in nine patients (75%) and expiratory positive airway pressures in eight patients (66%). Epworth sleepiness score before and after the study showed no change in five patients, improvement in six patients and deterioration in one patient. Conclusion: Cutaneous capnography is feasible and permits the optimization of non-invasive ventilation pressure settings in patients with chronic hypercapnic respiratory failure due to hypoventilation. Continuous cutaneous capnography might serve as an important additional tool to complement diurnal arterial carbon dioxide tension values.

Introduction: We present a case of facioscapulohumeral muscular dystrophy (FSHD) with a diaphragm paralysis as the primary cause of ventilatory failure. FSHD is an autosomal dominant inherited disorder with a restricted pattern of weakness. Although respiratory weakness is a relatively unknown in FSHD, it is not uncommon. Methods: We report on the clinical findings of a 68-year old male who presented with severe dyspnea while supine. Results: Supplementing our clinical findings with laboratory, electrophysiological and radiological performances led to the diagnosis of diaphragm paralysis. Arterial blood gas in sitting position without supplemental oxygen showed a mild hypercapnia. His sleep improved after starting non-invasive ventilation and his daytime sleepiness disappeared. Discussion: We conclude that in patients with FSHD who have symptoms of nocturnal hypoventilation, an adequate assessment of the diaphragm is recommended. This is of great importance as we know that nocturnal hypoventilation can be treated effectively by non-invasive ventilation.