Characteristics and predictors of outcome in children with severe acute bronchiolitis: A 10-yearexperience.

BACKGROUND: Severe acute bronchiolitis (SAB) can be life-threatening for infants and may be responsible for the congestion of intensive care units (ICU) during epidemics. We aimed to study the clinical and paraclinical characteristics of patients with SAB requiring a transfer to the ICU in order to examine their outcomes and to identify the predictors of a stay of ≥7 days and/or death. METHODS: This was a cross-sectional retrospective study including infants aged ≤12 months transferred to the ICU for their first episode of SAB between 1 January 2010 and 31 December 2019. RESULTS: We collected data on 380 patients with a median age of 1.75 months. They had a history of prematurity (20.53 %), low birth weight (18.68 %), parental atopy (12.89 %), and comorbidity (7.37 %, mainly congenital heart disease [5 %]). The leading cause of transfer was hypoxemia and increased oxygen requirements (49.73 %). The patients required mechanical ventilation (MV) in 63.42 % of the cases and noninvasive ventilation (NIV) in 67.63 %. NIV has supplanted MV over the years. Its use has increased from 40.4 % in 2010 to 96 % in 2019 compared with 83.84 % and 42 % for MV. A total of 14 (3.68 %) patients died. The independent predictors of a stay of ≥7 days and/or death were young age ≤2 months (p = 0.002), failure to thrive (p = 0.006), apnea (p = 0.045), dehydration (p = 0.018), the presence of biological inflammatory reaction (p = 0.002), isolation of respiratory syncytial virus (p < 0.001), and bacterial coinfection (p = 0.013).NIV was a protective factor (p < 0.001). A severity score ranging from 0 to 17 was established with an optimal cut-off value of 5 points. CONCLUSION: Specific caution is needed in patients with these severity predictors. The generalization ofNIV in general pediatrics departments would improve SAB management and reduce transfers to the ICU.

Swallowing during provision of helmet ventilation: Review and provisional multidisciplinary guidance.

Use of noninvasive ventilation provided by a helmet increased globally during and after the COVID-19 pandemic. This approach may reduce need for intubation and its associated clinical complications in critically ill patients. Use of helmet interface minimizes virus aerosolization while enabling verbal communication, oral feeding and coughing/expectoration of secretions during its administration. Although improved oral hydration is a recognized benefit of helmet NIV, relatively little is known about the safety and efficiency of swallowing during helmet NIV. Risk of aspiration is a key consideration given the fragile pulmonary status of critically ill patients requiring respiratory support, and therefore the decision to initiate oral intake is best made based on multidisciplinary input. We reviewed the current published evidence on NIV and its effects on upper airway physiology and swallowing function. We then presented a case example demonstrating preservation of swallowing performance with helmet NIV. Last, we offer provisional multidisciplinary guidance for clinical practice, and provide directions for future research.

Emergency medicine updates: Endotracheal intubation.

INTRODUCTION: Airway management including endotracheal intubation (ETI) is a key skill for emergency clinicians. Therefore, it is important for emergency clinicians to be aware of the current evidence regarding the identification and management of patients requiring ETI. OBJECTIVE: This paper evaluates key evidence-based updates concerning ETI for the emergency clinician. DISCUSSION: ETI is commonly performed in the emergency department (ED) setting but has many nuanced components. There are several tools that have been used to predict a difficult airway which incorporate anatomic and physiologic features. While helpful, these tools should not be used in isolation. Preoxygenation and apneic oxygenation are recommended to reduce the risk of desaturation and patient decompensation, particularly with noninvasive ventilation in critically ill patients. Induction and neuromuscular blocking medications should be tailored to the clinical scenario. Video laryngoscopy is superior to direct laryngoscopy among novice users, while both techniques are reasonable among more experienced clinicians. Recent literature suggests using a bougie during the first attempt. Point-of-care ultrasound is helpful for confirming correct placement and depth of the endotracheal tube. CONCLUSIONS: An understanding of literature updates can improve the ED care of patients requiring emergent intubation.

Oxygen Delivery Devices in Postoperative Patients: Proper Selection of Patients Matters!

How to cite this article: Bhattacharya D, Esquinas AM, Mandal M. Oxygen Delivery Devices in Postoperative Patients: Proper Selection of Patients Matters! Indian J Crit Care Med 2024;28(8):802.

Initiation of noninvasive ventilation in patients with amyotrophic lateral sclerosis.

INTRODUCTION/AIMS: Noninvasive ventilation (NIV) has been shown to improve survival and symptom burden in patients with amyotrophic lateral sclerosis (ALS). However, limited data exist regarding the clinical and physiological parameters at the time of NIV initiation. This study aimed to describe the clinical characteristics and respiratory physiological markers in a cohort of ALS patients with chronic respiratory failure. METHODS: This is a single-center retrospective cohort study of patients with ALS assessed for NIV initiation between February 2012 and January 2021. NIV was initiated based on insurance eligibility criteria: daytime hypercapnia, defined by partial pressure of carbon dioxide (PaCO2) >45 mm Hg using diurnal transcutaneous CO2 (TcCO2) as a surrogate, a maximal inspiratory pressure (MIP) <60 cmH2O or forced vital capacity (FVC) <50% predicted normal. RESULTS: We identified 335 patients with ALS and chronic respiratory failure referred to an outpatient home ventilation clinic for NIV initiation. The mean age was 64 years ±11; 151 (45%) were female, 326 (97%) were white, and 100 (29%) had bulbar-onset ALS. At the time of NIV initiation, the mean FVC was 64% ± 19%, the mean MIP; 41 cmH2O ± 17, and diurnal TcCO2; 40 ± 6 mmHg. The most common reasons for NIV initiation were MIP <60 cmH2O (58%) and multiple concomitant indications (28%). Within 1 year of NIV initiation, 126 (37%) patients were deceased. DISCUSSION: We found that impairment in inspiratory force was the most common reason for NIV initiation and often preceded significant declines in FVC.

Interfaces for Home Noninvasive Ventilation.

The choice of interface used to deliver noninvasive ventilation (NIV) is a critical element in successfully and safely establishing home NIV in people with sleep hypoventilation syndromes. Both patient-related and equipment-related factors need to be considered when selecting an interface. Recognizing specific issues that can occur with a particular style of mask is important when troubleshooting NIV problems and attempting to minimize side effects. Access to a range of mask styles and designs to use on a rotational basis is especially important for patients using NIV on a more continuous basis, those at risk of developing pressure areas, and children.

Palliative Care and Noninvasive Ventilation.

Palliative care is important for many patients who require noninvasive ventilation. The particular needs of patients with neuromuscular disease and chronic obstructive pulmonary disease are explored. Advance care planning is explored with tips for undertaking this important communication task. Brief comments regarding symptom burden, weaning, voluntary assisted dying, and self-care are included.

The Use of High-Flow Nasal Cannula in the Emergency Department and a Comparison of Its Efficacy With Noninvasive Ventilation.

BACKGROUND:  High-flow nasal cannula (HFNC) oxygenation has emerged as a convenient and handy oxygenation mode over the past few years, especially during the COVID-19 pandemic. HFNC is designed to provide humidified oxygen at high flow rates to subjects in a much more patient-compliant method. Noninvasive ventilation (NIV) has been a powerful tool in treating dyspneic patients of different etiologies, yielding positive outcomes over many decades. HFNC has the potential to serve as an alternative to NIV for acutely breathless patients, offering better patient compliance. METHODS:  A prospective observational study was conducted with a population size of 100 patients. The patients were randomly assigned to HFNC and NIV groups and further compared based on the clinical criteria, arterial oxygen pressure (PaO2)/fraction of inspired oxygen (FiO2) ratios, and modified Borg score. Simple proportions, mean, standard deviation, and chi-square tests were used. The chi-square test was applied to determine the association between the two attributes. RESULTS:  Both HFNC and NIV subset populations have shown substantial improvement in their clinical criteria in terms of respiratory rate, heart rate, oxygen saturation, PaO2/FiO2 ratios, and modified Borg score over two and six hours with statistically significant improvement in oxygen saturations among HFNC subset in comparison to NIV subset (at two hours, p = 0.004; at six hours, p = 0.022). Secondary outcomes like the need for intubation (14% in HFNC, 22% in NIV) and mortality (4% in HFNC, 6% in NIV group) were noted, which were statistically insignificant in comparing their efficacy. CONCLUSION:  The study concluded that HFNC resulted in better clinical parameters than NIV, but the difference was statistically insignificant except for oxygen saturation. Similarly, HFNC resulted in a decreased need for intubation and less mortality compared to NIV.

Cardiopulmonary Management for Severe Aortic Valve Stenosis With Noninvasive Ventilation Using a Nasopharyngeal Tube.

The nasopharyngeal tube (NT) is an effective interface for noninvasive ventilation (NIV). In cases of severe heart failure, assistance with noninvasive positive-pressure ventilation (NPPV) effectively reduces afterload and alleviates respiratory effort. We present the case of a three-day-old male neonate diagnosed with severe aortic valve stenosis (AS). In respiratory management, extubation was delayed due to increased respiratory effort and afterload, so this patient was extubated and managed with NPPV using an NT. An uncuffed endotracheal tube was inserted, initiating NIV with a positive end-expiratory pressure of 8 cmH2O. The patient exhibited stable vital signs post-extubation and was weaned off NPPV and transferred to the general ward. In this case of severe AS, the use of NT as an interface for NPPV demonstrated efficacy in respiratory and circulatory management. This approach could have shortened the duration of mechanical ventilation and facilitated safe postoperative care, highlighting the potential benefits of NT in managing severe heart failure.

Low Pressure Heliox-based Rebreather System to Reduce Work of Breathing and Conserve Gas.

Background: To test the ability of a low-pressure, low-flow, Heliox-based rebreathing system to reduce work of breathing and conserve gas while preserving CO2 concentration, temperature, and humidity at physiological levels in a bench study.Methods: We performed a bench study of a novel low-pressure, low-flow, noninvasive Heliox rebreathing system with CO2 scrubber that was connected to an artificial lung simulator with careful monitoring of flow, pressure, work of breathing, oxygen (O2), carbon-dioxide (CO2), temperature, and humidity levels. Multiple runs of breathing were performed while manipulating levels of resistance (5 - 30 cm H2O/L/sec), gas mixtures (room air, 79% Helium 21% O2, and 70% Helium and 30% O2), and leak levels (ultra-low, low, and high).Results: We found significant reductions in work of breathing (up to 64%) while conserving gas with estimates of up to 54-fold reduction in medical gas wastage (P<0.001). Specifically, at resistances of 5, 10, 20, and 30 cm H2O/L/sec we demonstrated 64%, 57%, 36%, and 7% reduction in work of breathing (P<0.0001). Gas wastage was reduced by 10- to 54-fold while the end-tidal CO2 concentration, humidity, and temperature were maintained by the device at physiological levels.Conclusions: In a bench-test, a low-pressure, low-flow, noninvasive Heliox rebreathing system with CO2 scrubber reduced work of breathing and conserved gas while preserving CO2 concentration, temperature, and humidity at physiological levels. Future studies in human subjects need to be performed to determine whether reduction of work of breathing and gas conservation can be achieved.

Predictors for prescription of noninvasive ventilation in the postoperative period of cardiac surgery: a systematic review.

INTRODUCTION: The postoperative (PO) period after cardiac surgery is associated with the occurrence of respiratory complications. Noninvasive positive pressure ventilation (NIPPV) is largely used as a ventilatory support strategy after the interruption of invasive mechanical ventilation. However, the variables associated with NIPPV prescription are unclear. OBJECTIVE: To describe the literature on predictors of NIPPV prescription in patients during the PO period of cardiac surgery. MATERIALS AND METHODS: This systematic review was registered on the International Prospective Register of Systematic Reviews (PROSPERO) platform in December 2021 (CRD42021291973). Bibliographic searches were performed in February 2022 using the PubMed, Lilacs, Embase and PEDro databases, with no year or language restrictions. The Predictors for the prescription of NIPPV were considered among patients who achieved curative NIPPV. RESULTS: A total of 349 articles were identified, of which four were deemed eligible and were included in this review. Three studies were retrospective studies, and one was a prospective safety pilot study. The total sample size in each study ranged from 109 to 1657 subjects, with a total of 3456 participants, of whom 283 realized NIPPV. Curative NIPPV was the only form of NIPPV in 75% of the studies, which presented this form of prescription in 5-9% of the total sample size, with men around 65 years old being the majority of the participants receiving curative NIPPV. The main indication for curative NIPPV was acute respiratory failure. Only one study realized prophylactic NIPPV (28% of 32 participants). The main predictors for the prescription of curative NIPPV in the PO period of cardiac surgery observed in this study were elevated body mass index (BMI), hypercapnia, PO lung injury, cardiogenic oedema and pneumonia. CONCLUSIONS: BMI and lung alterations related to gas exchange disturbances are major predictors for NIPPV prescription in patients during the PO period of cardiac surgery. The identification of these predictors can benefit clinical decision-making regarding the prescription of NIPPV and help conserve human and material resources, thereby preventing the indiscriminate use of NIPPV.

Rehabilitation Strategies for Patients With Spinal Muscular Atrophy in the Era of Disease-Modifying Therapy.

The impact of disease-modifying therapy ranges from cure to no impact with a wide range of intermediates. In cases where the intermediate group reaches a plateau after the acquisition of some muscle strength, it is necessary to set a functional level appropriate for increased motor power and establish a long-term exercise plan to maintain it. As the disease status stabilizes and the life span increases, early nonsurgical interventions are required, such as using a standing frame to prevent joint contracture, applying a spinal brace at the early stage of scoliosis, and maintaining sitting postures that exaggerate lumbar lordosis. In cases where scoliosis and hip displacement occur and progress even after conservative managements are implemented, early referral to surgery should be considered. Oromotor activity and swallowing function are influenced not only by the effects of disease-modifying drugs, but also by post-birth experience and training. Therefore, although the feeding tube cannot be removed, it is necessary to make efforts to simulate the infant feeding development while maintaining partial oral feeding. Since the application period of non-invasive ventilators has increased, it has become more important to prevent long-term complications such as facial abrasion, skin allergy, orthodontic deformities, and maxillary flattening caused by the interface. Dual ventilator mode or interface can also be utilized.

Factors associated with noninvasive ventilation usage in patients with hypoventilation disorders.

Study Objectives: The objective of this study was to investigate the association between demographic, clinical, and interface factors and noninvasive ventilation (NIV) usage. Methods: A retrospective cohort analysis of 478 patients prescribed NIV from 2013 to 2021 was performed. Demographic factors, clinical indications for NIV, and interface factors were collected, and linear regression was conducted to evaluate the association between these variables and NIV usage (hour/night). Results: The average usage of the cohort was 6.5 hour/night ±â€…4.6, with an average age of 57 years ±â€…16 and body mass index (BMI) of 40.5kg/m2 ±â€…14.7. The cohort was mostly male (n = 290, 60.6%). The most common indications for NIV prescription were high-pressure requirement for obstructive sleep apnea (HPR, n = 190, 39.7%), neuromuscular disease (NMD, n = 140, 29.3%), and obesity hypoventilation syndrome (OHS, n = 111, 23.2%). A diagnosis of NMD was a significant predictor of higher NIV usage (8.0 ±â€…6.1 hour/night) in multivariate analysis (p = .036). The HPR subcohort had the lowest usage of all indications. Age and BMI did not predict usage. A nasal interface (p < .01) and lower expiratory positive airway pressure (EPAP) setting (p < .001) were associated with increased NIV usage. Conclusions: This study highlights the multifaceted nature of NIV usage. Where demographic factors were not consistent predictors of usage, interface, and clinical indication were associated with usage. These findings highlight that the HPR users are a group at risk of low usage.

Noninvasive ventilation and laser-assisted unilateral posterior cordotomy as novel multidisciplinary approaches for Charcot-Marie-Tooth disease 4B vocal cord paralysis: a case report.

BACKGROUND: Charcot-Marie-Tooth disease (CMT) is one of the most common inherited neuropathies. The disease is generally characterized by sensory loss most prominent in distal extremities, muscle weakness, and muscle wasting. There is still no effective therapy for Charcot-Marie-Tooth disease. CASE PRESENTATION: The patient is a 6-year-old Iranian girl, of Fars ethnicity, who was admitted with a chief complaint of hoarseness and an impression of Charcot-Marie-Tooth disease type 4B. She was initially treated with noninvasive ventilation and, after a year, electively underwent cordotomy as a novel therapeutic approach. CONCLUSIONS: Charcot-Marie-Tooth disease type 4B is a less common but important cause of stridor. Noninvasive ventilation treatment and unilateral posterior cordotomy can be utilized for hereditary neuropathies.

Nasal high flow or noninvasive ventilation? navigating hypercapnic COPD exacerbation treatment: A randomized noninferiority clinical trial.

BACKGROUND: Noninvasive ventilation (NIV) has been the cornerstone for managing acute exacerbations of COPD (AECOPD) with hypercapnic respiratory failure. Nasal high flow (NHF) oxygen therapy has emerged as a potential alternative, offering a more tolerable modality with promising outcomes. The aim of the present study was to evaluate whether NHF respiratory support is noninferior to NIV with respect to treatment failure, in patients with mild-to-moderate hypercapnic AECOPD. METHODS: In this multi-center, randomized, noninferiority trial, 105 patients with AECOPD and respiratory failure type II were enrolled. Participants were randomly assigned to receive either NHF therapy or NIV. The primary endpoint was the frequency of treatment failure, defined as the need for intubation and invasive mechanical ventilation or a switch to the alternative treatment group. Secondary endpoints included changes in respiratory parameters, patient comfort indicators, and the occurrence of complications. RESULTS: The findings revealed no significant difference in the primary outcome between the groups, with a treatment failure rate of 19.6 % (10 out of 51) in the NHF group and 14.8 % (8 out of 54) in the NIV group. Interestingly, NHF users reported significantly lower levels of dyspnea and discomfort at multiple follow-up points. Despite the differences in patient comfort, respiratory parameters such as respiratory rate, arterial blood gases, and use of accessory muscles of respiration showed no significant disparities between the groups throughout the study period. CONCLUSIONS: NHF therapy was similar to NIV in preventing treatment failure among patients with hypercapnic AECOPD, offering a viable alternative with enhanced comfort. TRIAL REGISTRATION: The study was prospectively registered in ClinicalTrials.gov (Identifier: NCT03466385) on March 15, 2018.

Deventilation Syndrome in Patients with Chronic Obstructive Pulmonary Disease Using Nocturnal Noninvasive Ventilation: What Are the Underlying Mechanisms?

INTRODUCTION: Patients with chronic obstructive pulmonary disease (COPD) commonly experience severe dyspnea after discontinuation of nocturnal noninvasive ventilation (NIV), known as deventilation syndrome (DVS), which negatively affects quality of life. Despite various hypotheses, the precise mechanisms of DVS remain unknown. METHODS: An observational pilot study was performed monitoring 16 stable COPD patients before, during, and after an afternoon nap on NIV. Seven patients experienced DVS (Borg Dyspnea Scale ≥5), while nine served as controls (Borg Dyspnea Scale ≤2). Hyperinflation was evaluated through inspiratory capacity (IC) measurements and end-expiratory lung impedance (EELI) via electrical impedance tomography. Respiratory muscle activity was assessed by diaphragmatic surface electromyography (sEMG). RESULTS: Post-NIV dyspnea scores were significantly higher in the DVS group (5 [3-7] vs. 0 [0-1.5], p < 0.001). IC values were lower in the DVS group compared to controls, both pre-NIV (54 [41-63] vs. 88 [72-94] %pred., p = 0.006) and post-NIV (45 [40-59] vs. 76 [65-82] %pred., p = 0.005), while no intergroup difference was seen in IC changes pre- and post-NIV. EELI values after NIV indicated a tendency towards lower values in controls and higher values in DVS patients. sEMG amplitudes were higher in the DVS group within the first 5-min post-NIV (221 [112-294] vs. 100 [58-177]% of baseline, p = 0.030). CONCLUSION: This study suggests that it is unlikely that DVS originates from the inability to create diaphragmatic muscle activity after NIV. Instead, NIV-induced hyperinflation in individuals with static hyperinflation may play a significant role. Addressing hyperinflation holds promise in preventing DVS symptoms in COPD patients.

Nasal High-Frequency Oscillatory Ventilation Use in Romanian Neonatal Intensive Care Units-The Results of a Recent Survey.

BACKGROUND: Nasal high-frequency oscillatory ventilation (nHFOV) has emerged as an effective initial and rescue noninvasive respiratory support mode for preterm infants with respiratory distress syndrome (RDS); however, little is known about nHFOV use in Romanian neonatal intensive care units (NICUs). OBJECTIVES: We aimed to identify the usage extent and clinical application of nHFOV in Romania. METHODS: A structured web-based questionnaire was designed to find the rate of nHFOV use and knowledge of this new method of noninvasive respiratory support in Romanian level III NICUs. Using multiple-choice, open-ended, and yes/no questions, we collected information on the NICU's size, noninvasive respiratory support modes used, nHFOV use, indications, settings, nasal interfaces, secondary effects, and equipment used. Descriptive statistics and comparisons were performed using IBM SPSS Statistics 26.0. RESULTS: A total of 21/23 (91.3%) leaders from level III NICUs (median [IQR] number of beds of 10 [10-17.5]) responded to the survey. The most frequently used noninvasive ventilation modes were CPAP mode on mechanical ventilators (76.2%), followed by NIPPV (76.2%); heated, humidified high-flow nasal cannula (HHHFNC) (61.9%); and nHFOV (11/21 units; 52.4%). A total of 5/11 units reported frequent nHFOV use (in two or more newborns/month) in both term and preterm infants. The main indications reported for nHFOV use were CPAP failure (90.9%), hypercapnia (81.8%), and bronchopulmonary dysplasia (72.7%). Face/nasal masks and short binasal prongs are the most commonly used nasal interfaces (90.9% and 72.7%, respectively). Air leaks at the interface level (90.9%), thick secretions (81.8%), and airway obstruction (63.6%) were the most frequently mentioned adverse effects of nHFOV. Only three of the NICUs had a written protocol for nHFOV use. Most units not yet using nHFOV cited lack of equipment, experience, training, or insufficient information and evidence for the clinical use and outcome of nHFOV use in neonates as the main reasons for not implementing this noninvasive respiratory mode. CONCLUSIONS: Our survey showed that nHFOV is already used in more than half of the Romanian level III NICUs to support term and preterm infants with respiratory distress despite a lack of consensus regarding indications and settings during nHFOV.

Feasibility of synchronized high flow nasal cannula.

BACKGROUND: A high-flow nasal cannula (cHFNC) delivers flow continuously (during inspiration and expiration). Using the diaphragm electrical activity (Edi), synchronizing HFNC could be an alternative (cycling high/low flow on inspiration/expiration, respectively). The objective of this study was to demonstrate the feasibility of synchronized HFNC (sHFNC) and compare it to cHFNC. METHODS: Different levels of cHFNC and sHFNC (4, 6, 8, and 10 liters per minute [LPM], with 2 LPM on expiration for sHFNC) were compared in eight rabbits (mean weight 3.16 kg), before and after acute lung injury (pre-ALI and post-ALI). Edi, tracheal pressure (Ptr), esophageal pressure (Pes), flow, and arterial CO2 were measured. In addition to the animal study, one 3.52 kg infant received sHFNC and cHFNC using a Servo-U ventilator. RESULTS: In the animal study, there were more pronounced decreases in Edi, reduced Pes swings and reduced PaCO2 at comparable flows during sHFNC compared to cHFNC both pre and post-ALI (p < .05). Baseline (pre-inspiratory) Ptr was 2-7 cmH2O greater during cHFNC (p < .05) indicating more dynamic hyperinflation. In one infant, the ventilator performed as expected, delivering Edi-synchronized high/low flow. CONCLUSION: Synchronizing high flow unloaded breathing, decreased Edi, and reduced PaCO2 in an animal model and is feasible in infants.

Chronic obstructive pulmonary disease and obstructive sleep apnea overlap: who to treat and how?

INTRODUCTION: The co-existence of chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea (OSA), or the overlap syndrome, is common and associated with a distinct pattern of nocturnal hypoxemia and worse clinical outcomes than either disease alone. Consequently, identifying who and how to treat these patients is essential. AREAS COVERED: Treatment is recommended in all patients with OSA and symptoms or systemic hypertension, but determining symptoms attributable to OSA can be challenging in patients with COPD. Treatment should be considered in asymptomatic patients with moderate to severe OSA and COPD with pulmonary hypertension and comorbid cardiovascular and cerebrovascular disease, especially if marked hypoxic burden. CPAP is effective, but in patients with the overlap syndrome and daytime hypercapnia, high-intensity noninvasive ventilation aiming to lower PaCO2 may have additional benefits. Additionally, in those with severe resting daytime hypoxemia, supplemental oxygen improves survival and should be added to positive airway pressure. The role of alternative non-positive airway pressure therapies in the overlap syndrome needs further study. EXPERT OPINION: Both COPD and OSA are heterogeneous disorders with a wide range of disease severity and further research is needed to better characterize and prognosticate patients with the overlap syndrome to personalize treatment.

Concise review of end of life and palliative care in neuromuscular pathologies: still a long pathway ahead.

Introduction: Neuromuscular diseases (NMD) include different types of diseases depending on the deficient component of the motor unit involved. They may all be interested by a progressive and sometimes irreversible pump respiratory failure which unfortunately for some NMD may start soon after the diagnosis. Within this vast group of patients those affected by muscle diseases are a subgroup who comprises patients with an average earlier onset of symptoms compared to other NMD. Indeed it is also important to comprehend not just the patient's burden but also the surrounding families'. Defining the end of life (EoL) phase in these patients is not simple especially in the young patient population. Consequently, the late stage of disease remains poorly defined and challenging. Objectives: The aim of this review is to describe the EoL phase in NMD patients with attention to QoL and psycological status. Methods: The focus would be on one hand on the management of the psychological burden, the communication barriers, and tone of humor. Results: Those topics have been described being crucial in this group of patients as they increase tensions and burden of both patient and family, and between them and the outside world. Thus also causing their social isolation, increasing anxiety and reducing their quality of life. On the other hand the use of cough clearance devices and all the respiratory supports and their withdrawn are carefully evaluated in the view of alleviating respiratory symptoms, improving patient quality of life and above all reaching the patient's goals of care. Conclusions: Although there is no cure, the advent of supportive interventions including multidisciplinary care (MDC) has improved all the aspects of dying for patients affected by NMD; nevertheless there still a long pathway ahead.