High-flow nasal oxygen versus conventional oxygen therapy in patients with COVID-19 pneumonia and mild hypoxaemia: a randomised controlled trial.

RATIONALE: In patients with COVID-19 pneumonia and mild hypoxaemia, the clinical benefit of high-flow nasal oxygen (HFNO) remains unclear. We aimed to examine whether HFNO compared with conventional oxygen therapy (COT) could prevent escalation of respiratory support in this patient population. METHODS: In this multicentre, randomised, parallel-group, open-label trial, patients with COVID-19 pneumonia and peripheral oxygen saturation (SpO2) ≤92% who required oxygen therapy were randomised to HFNO or COT. The primary outcome was the rate of escalation of respiratory support (ie, continuous positive airway pressure, non-invasive ventilation or invasive mechanical ventilation) within 28 days. Among secondary outcomes, clinical recovery was defined as the improvement in oxygenation (SpO2 ≥96% with fractional inspired oxygen (FiO2) ≤30% or partial pressure of arterial carbon dioxide/FiO2 ratio >300 mm Hg). RESULTS: Among 364 randomised patients, 55 (30.3%) of 181 patients assigned to HFNO and 70 (38.6%) of 181 patients assigned to COT underwent escalation of respiratory support, with no significant difference between groups (absolute risk difference -8.2% (95% CI -18% to +1.4%); RR 0.79 (95% CI 0.59 to 1.05); p=0.09). There was no significant difference in clinical recovery (69.1% vs 60.8%; absolute risk difference 8.2% (95% CI -1.5% to +18.0%), RR 1.14 (95% CI 0.98 to 1.32)), intensive care unit admission (7.7% vs 11.0%, absolute risk difference -3.3% (95% CI -9.3% to +2.6%)), and in hospital length of stay (11 (IQR 8-17) vs 11 (IQR 7-20) days, absolute risk difference -1.0% (95% CI -3.1% to +1.1%)). CONCLUSIONS: Among patients with COVID-19 pneumonia and mild hypoxaemia, the use of HFNO did not significantly reduce the likelihood of escalation of respiratory support. TRIAL REGISTRATION NUMBER: NCT04655638.

Building a home ventilation programme: population, equipment, delivery and cost.

Home mechanical ventilation (HMV) improves quality of life and survival in patients with neuromuscular disorders (NMD). Developing countries may benefit from published evidence regarding the prevalence, cost of equipment, technical issues and organisation of HMV in NMD, facilitating the development of local turn-key HMV programmes. Unfortunately, such evidence is scattered in the existing literature. We searched Medline for publications in English and French from 2005 to 2020. This narrative review analyses 24 international programmes of HMV. The estimated prevalence (min-max) of HMV is ±7.3/100 000 population (1.2-47), all disorders combined. The prevalence of HMV is associated with the gross domestic product per capita in these 24 countries. The prevalence of NMD is about 30/100 000 population, of which ±10% would use HMV. Nocturnal (8/24 hour), discontinuous (8-16/24 hours) and continuous (>16/24 hours) ventilation is likely to concern about 60%, 20% and 20% of NMD patients using HMV. A minimal budget of about 168€/patient/year (504€/100 000 population), including the cost of equipment solely, should address the cost of HMV equipment in low-income countries. When services and maintenance are included, the budget can drastically increase up to between 3232 and 5760€/patient/year. Emerging programmes of HMV in developing countries reveal the positive impact of international cooperation. Today, at least 12 new middle, and low-income countries are developing HMV programmes. This review with updated data on prevalence, technical issues, cost of equipment and services for HMV should trigger objective dialogues between the stakeholders (patient associations, healthcare professionals and politicians); potentially leading to the production of workable strategies for the development of HMV in patients with NMD living in developing countries.

ERS clinical practice guidelines: high-flow nasal cannula in acute respiratory failure.

BACKGROUND: High-flow nasal cannula (HFNC) has become a frequently used noninvasive form of respiratory support in acute settings; however, evidence supporting its use has only recently emerged. These guidelines provide evidence-based recommendations for the use of HFNC alongside other noninvasive forms of respiratory support in adults with acute respiratory failure (ARF). MATERIALS AND METHODOLOGY: The European Respiratory Society task force panel included expert clinicians and methodologists in pulmonology and intensive care medicine. The task force used the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) methods to summarise evidence and develop clinical recommendations for the use of HFNC alongside conventional oxygen therapy (COT) and noninvasive ventilation (NIV) for the management of adults in acute settings with ARF. RESULTS: The task force developed eight conditional recommendations, suggesting the use of 1) HFNC over COT in hypoxaemic ARF; 2) HFNC over NIV in hypoxaemic ARF; 3) HFNC over COT during breaks from NIV; 4) either HFNC or COT in post-operative patients at low risk of pulmonary complications; 5) either HFNC or NIV in post-operative patients at high risk of pulmonary complications; 6) HFNC over COT in nonsurgical patients at low risk of extubation failure; 7) NIV over HFNC for patients at high risk of extubation failure unless there are relative or absolute contraindications to NIV; and 8) trialling NIV prior to use of HFNC in patients with COPD and hypercapnic ARF. CONCLUSIONS: HFNC is a valuable intervention in adults with ARF. These conditional recommendations can assist clinicians in choosing the most appropriate form of noninvasive respiratory support to provide to patients in different acute settings.

Estimating Amyotrophic Lateral Sclerosis and Motor Neuron Disease Prevalence in Portugal Using a Pharmaco-Epidemiological Approach and a Bayesian Multiparameter Evidence Synthesis Model.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a rare and progressive neurodegenerative disease involving the upper and lower motor neurons. It is also the most common and the one with the worst prognosis among the motor neuron diseases (MND). ALS invariably progresses to respiratory failure, which is an essential factor affecting the prognosis of this disease. Its prevalence in the world is heterogeneous and, in many countries, is even unknown, since national registries are not mandatory or comprehensive enough. Worldwide, the ALS/MND prevalence is estimated between 4 and 8 cases per 100,000 inhabitants, but in Portugal the prevalence was never studied. Because ALS and MND are rare diseases, population-based studies are very difficult to perform. In Portugal, there are no systematic patient registries. OBJECTIVE: We aimed to obtain the best available indirect estimates of ALS/MND prevalence, using a pharmaco-epidemiological approach. METHOD: We developed a Bayesian multiparameter evidence synthesis model based on nationwide data of riluzole consumption, a drug highly specific for ALS/MND, combined with data from a nationwide hospital administrative database, data from the national institute of statistics, and data from other scientific articles focused on ALS/MND epidemiology, to estimate ALS/MND prevalence in Portugal. RESULTS: We found an estimated ALS/MND prevalence in Portugal steadily increasing from 6.74 per 100,000 inhabitants (Bayesian 95% Credible Interval [95% CI] 5.39-9.37) in 2009 to 10.32 (95% CI 8.27-14.27) in 2016. In 2016, the estimated ALS/MND prevalence was higher in men, 12.08 per 100,000 (9.66-17.15), than in women, 8.56 (6.84-12.32). Regarding age groups, the estimated prevalence per 100,000 inhabitants were, in 2016 for women, 1.19 (0.78-1.85) for the <50 years' group, 8.48 (6.00-12.76) for the 51-60 group, 23.47 (18.05-33.88) for the 61-70 group, 28.77 (22.02-41.31) for the 71-80 group, and 14.45 (9.97-21.63) for the >80 group. For men, the prevalence estimates were 1.90 (1.32-2.84), 12.89 (9.44-19.16), 32.18 (24.91-45.74), 48.85 (38.72-71.40), and 31.27 (21.73-46.41), respectively, for each age group. We also observed a relevant variability across the country, with prevalence estimates, in 2016, of 9.31 cases per 100,000 inhabitants (7.45-12.86) in the Northern region of Portugal, 11.15 (8.9-15.34) in the Centre region, 10.74 (8.6-14.82) in Lisbon and Alentejo regions, and 5.55 (4.35-7.83) in the Algarve region. CONCLUSION: Overall, and even though we must account for the limitations of the indirect methods and models used for prevalence estimation, we probably have a very high ALS/MND prevalence in Portugal. It would be important to create registries, particularly in rare diseases, for better organization and distribution of healthcare services and resources, particularly at the level of ventilatory support.

Validation of the Multi-INdependence Dimensions (MIND) questionnaire for prolonged mechanically ventilated subjects.

BACKGROUND: Evaluating severity of illness of patients with prolonged mechanical ventilation (PMV) is important to adopt the best appropriate care management for each individual. Yet, no severity-of-illness scoring system has been specifically designed for this type of patients. The aim of this study was to develop and validate a new instrument, the Multi-INdependence Dimensions (MIND) questionnaire designed to comprehensively measure the severity of illness of patients under PMV. METHODS: The validation of the MIND questionnaire was performed during a longitudinal observational study conducted with PMV subjects in weaning facilities in three countries (Argentina, Colombia and Germany). The questionnaire validity was tested in 3 stages: 1) Specification of components, with description of item responses, inter-item and Cronbach alpha correlations; 2) Creation of the composite scores; 3) Measurement properties determination including test-retest reliability after 30 days, clinical validity (Medical Research Council (MRC) muscle strength score, Sepsis-related Organ Failure Assessment (SOFA), Glasgow Coma Scale (GCS), Dependence Nursing Scale and EuroQol-5 Dimension evaluated at inclusion), and ability to detect change. RESULTS: A total of 128 subjects participated in the validation study. Eleven component scores and four composite scores were created. MIND scores significantly correlated with MRC muscle strength, SOFA, DNS, GCS and EQ-5D, supporting the validity of the new scores. Intraclass Correlation Coefficient greater than 0.82 were observed for all composite scores, indicating good test-retest reliability. MIND scores were able to detect improvement in subject severity of illness. CONCLUSION: The MIND questionnaire is a valid and reliable instrument for measuring comprehensively the multiple dimensions characterizing the severity of illness of PMV patients. TRIAL REGISTRATION: NCT02255058 .

Mouthpiece ventilation and complementary techniques in patients with neuromuscular disease: A brief clinical review and update.

Noninvasive ventilatory support (NVS) is sometimes reported as suboptimal in patients with neuromuscular disease (NMD). The reasons for this include inadequate ventilator settings and/or lack of interface tolerance. NVS has been used for many years in patients with NMD disorders as a viable alternative to continuous ventilatory support via a tracheostomy tube. The mouthpiece ventilation (MPV) is a ventilatory mode that is used as daytime ventilatory support in combination with other ventilatory modalities and interfaces for nocturnal NVS. However, there is still a poor understanding of this method's benefits compared with other modalities. This review aims to highlight the indications and advantages along with the disadvantages of MPV.

Tele-monitoring of ventilator-dependent patients: a European Respiratory Society Statement.

The estimated prevalence of ventilator-dependent individuals in Europe is 6.6 per 100 000 people. The increasing number and costs of these complex patients make present health organisations largely insufficient to face their needs. As a consequence, their burden lays mostly over families. The need to reduce healthcare costs and to increase safety has prompted the development of tele-monitoring for home ventilatory assistance.A European Respiratory Society Task Force produced a literature research based statement on commonly accepted clinical criteria for indications, follow-up, equipment, facilities, legal and economic issues of tele-monitoring of these patients.Many remote health monitoring systems are available, ensuring safety, feasibility, effectiveness, sustainability and flexibility to face different patients' needs. The legal problems associated with tele-monitoring are still controversial. National and European Union (EU) governments should develop guidelines and ethical, legal, regulatory, technical, administrative standards for remote medicine. The economic advantages, if any, of this new approach must be compared to a "gold standard" of home care that is very variable among different European countries and within each European country.Much more research is needed before considering tele-monitoring a real improvement in the management of these patients.

Randomised short-term trial of high-span versus low-span APAP for treating sleep apnoea.

PURPOSE: Auto-titrating continuous positive airway pressure (APAP) devices were developed to improve treatment efficacy and compliance in patients with obstructive sleep apnoea syndrome (OSAS). Since there are insufficient data on the optimal pressure range setting, we aimed to compare the adherence, efficacy and tolerability of treatment with high-span versus low-span APAP. METHODS: Seventy-six newly diagnosed OSAS patients fulfilling the treatment criteria were randomised to receive high-span (HS, range 4-15cmH2O, n = 38) or low-span (LS, range 8-12cmH2O, n = 38) APAP. Patients were assessed at 1 and 3 months. RESULTS: Median Epworth sleepiness scale (ESS) was 13 (IQR, 6-16) and median apnoea-hypopnoea index (AHI) was 35.9 (IQR, 27.6-56.3). There were no significant differences in baseline demographic and clinical characteristics between groups. Overall, no significant differences were found at the first month assessment. After 3 months of therapy, we found again no differences in residual AHI or ESS. However, the group HS proved less adherent than group LS, respectively, with median 87 % (IQR, 60.5-97.5) versus 94 % (IQR, 80.0-98.3) of the nights using ≥4 h (P = 0.014) and mean (±SD) usage 5.7 ± 1.6 versus 6.4 ± 1.2 h/night (P = 0.049). The group HS reported more frequently nasal congestion, excessive oronasal dryness and nocturnal awakenings of at least moderate intensity, the latter with statistical significance (P = 0.005). CONCLUSIONS: Both pressure ranges appear to be equally effective to correct AHI and to improve symptoms. Though, patients with high-span APAP were less compliant to treatment, raising issues about the tolerability of wide pressure range settings of these devices.

Adherence to APAP in obstructive sleep apnea syndrome: effectiveness of a motivational intervention.

BACKGROUND: Obstructive sleep apnea syndrome (OSAS) is a serious disorder with significant health consequences. Treatment adherence to auto-titrating positive airway pressure (APAP) is often below expectations. We investigated the effectiveness of a brief educational intervention using motivational strategies in treatment adherence among patients with OSAS. METHODS: The study followed a randomized, controlled design and included 61 patients diagnosed with OSAS, meeting the criteria for APAP therapy. Patients recruited from a Sleep Disorders Unit were randomly allocated to an intervention group (IG) and two control groups (CG1 and CG2). In the IG motivational strategies were applied according to patient's motivation, assessed by the degree of confidence and conviction. In the CG1, participants received exclusively standardized information, and in the CG2, routine procedures were followed. Assessment included the Apnea Hypopnea Index (AHI), the Epworth Sleepiness Scale (ESS), and sociodemographic and clinical information. Adherence to treatment was measured after 1 (T1) and 2 months (T2) through the APAP software. RESULTS: The IG presented higher adherence to APAP-percentage of days of use >4 h (89.8 % p = 0.013), mean effective use per effective day (6.2 p = 0.000), and lower AHI (2.7 p = 0.019) at T2 when compared with the other two groups. Confidence was higher in the IG group at T2 than at T1 (p = 0.000). The ESS presented a significant reduction (p = 0.000) in the IG and in the CG1 (p = 0.008), but was higher in the CG2 (p = 0.015). CONCLUSIONS: Brief interventions using motivational strategies can improve a patient's adherence to APAP.

Obstructive sleep apnea representations, self-efficacy and family coping regarding APAP adherence: a longitudinal study.

Adherence is still an important issue considering new advances in Obstructive Sleep Apnea (OSA) treatment, as automatic positive airway pressure (APAP). The aim of the present study was to identify and explore relationships between identified predictors of adherence, over time. After overnight sleep study and OSA diagnosis and during a six-month APAP treatment period, a total of 153 patients underwent a three time psychological protocol evaluation. Generalized estimating equations were applied to analyzed repeated measurements in the same individuals. Results show that 40% of patients were poorly adherent and 60% were adherent after six months of treatment. The results confirmed a predictive value of age, self-efficacy, decisional balance index and health-related quality of life (HRQoL) in APAP adherence. Furthermore, the results revealed an interaction between time and illness cognitive representations, and self-efficacy and family coping, in explaining adherence patterns over time. Therefore, understanding the causality of theoretically derived constructs is crucial to predict the continuity of APAP adherence.

InspireGBs - Inspiratory muscle training in people with Guillain-Barré syndrome: A feasibility study.

BACKGROUND: Guillain-Barré syndrome (GBS) is a rare immune-mediated peripheral nerve disease often preceded by infections. Respiratory muscle weakness is a common complication in this population, leading to decreased vital capacity, weakened coughing ability, atelectasis, and pulmonary infections. Inspiratory muscle training (IMT) has been widely used to enhance inspiratory muscle strength and pulmonary function in various diseases; however, its application in GBS is unknown. OBJECTIVE: To assess the safety, feasibility, and preliminary effectiveness of an IMT protocol-InspireGBs-in people with GBS. METHODS: A pre/post feasibility study was conducted. Feasibility was determined by participant recruitment/retention, adherence, time spent in each session, and adverse events. Secondary outcome was inspiratory muscle strength. InspireGBs consisted of twice daily sessions 5 times/week, three sets of 10 breaths in each session, for 6 weeks. Initial resistance was set at 50% of participant's baseline maximal inspiratory pressure (MIP) and with a weekly increase of 10% calculated from the previous week's training intensity, if tolerated, otherwise the increase was 5%. RESULTS: Eleven patients (63% male; 63 ± 9 years) were recruited from inpatient rehabilitation and 10 completed the study. Recruitment and retention rates were high (79% and 91%, respectively). Excellent adherence rate (96%) was obtained with no reported adverse effects or safety issues. Sessions lasted from 4 to 6 minutes. The MIP improved (pretraining: 39 [26.5-50] cm H2O vs. posttraining: 61 [56.3-64.5] cm H2O, p = .005 and pretraining: 38 [30.5-53.8] % of predicted vs. posttraining: 60 [54.28-71.58] % of predicted, p = .009) with the InspireGBs. CONCLUSION: InspireGBs is safe, feasible, and may be effective in improving inspiratory muscle strength in individuals with GBS. A randomized controlled trial is now needed to strengthen these findings.

Precision in Practice: Enhancing MI-E Therapy Evaluation for Improved Patient Outcomes, Excluding Speech and Swallowing. Reply to Allen et al. Comment on "Chatwin et al. Waves of Precision: A Practical Guide for Reviewing New Tools to Evaluate Mechanical In-Exsufflation Efficacy in Neuromuscular Disorders. J. Clin. Med. 2024, 13, 2643".

We would like to thank Allen et al [...].

Waves of Precision: A Practical Guide for Reviewing New Tools to Evaluate Mechanical In-Exsufflation Efficacy in Neuromuscular Disorders.

Mechanical insufflation-exsufflation (MI-E) is essential for secretion clearance, especially in neuromuscular disorders. For the best outcomes, initiation of MI-E should be started at the correct time with regular evaluation to the response to treatment. Typically, cough peak flow has been used to evaluate cough effectiveness with and without MI-E. This review highlights the limitations of this and discussed other tools to evaluate MI-E efficacy in this rapidly developing field. Such tools include the interpretation of parameters (like pressure, flow and volumes) that derive from the MI-E device and external methods to evaluate upper airway closure. In this review we pinpoint the differences between different devices in the market and discuss new tools to better titrate MI-E and detect pathological responses of the upper airway. We discuss the importance of point of care ultrasound (POCUS), transnasal fiberoptic laryngoscopy and wave form analysis in this setting. To improve clinical practice newer generation MI-E devices should allow real-time evaluation of waveforms and standardize some of the derived parameters.

Measurement properties of the incremental step test for people with chronic obstructive pulmonary disease: a cross-sectional study.

OBJECTIVES: The new incremental step test (IST) is a field test that was developed for people with chronic obstructive pulmonary disease (COPD), based on the characteristics of the incremental shuttle walk test (ISWT); however, its measurement properties still need to be determined. We aimed, first, to assess the construct validity (through the comparison with the ISWT), within-day reliability and measurement error of the IST in people with COPD; and, second, to identify whether the participants have a learning effect in the IST. DESIGN: Cross-sectional study, conducted according to COnsensus-based Standards for the selection of health status Measurement INstruments guidelines. SETTING: A family health unit in Portugal, April 2022 to June 2023. PARTICIPANTS AND ANALYSIS: 63 participants (67.5±10.5 years) attended two sessions to perform two IST and two ISWT, separately. Spearman's correlations were used to compare the best performances between the IST and the ISWT. Intraclass correlation coefficient (ICC2,1) was used for reliability, and the SE of measurement (SEM), minimal detectable change at 95% CI (MDC95) and Bland and Altman 95% limits of agreement (LoA) were used for measurement error. The learning effect was explored with the Wilcoxon signed-rank test. RESULTS: The IST was significant and strongly correlated with the ISWT (0.72<ρ<0.74, p<0.001), presented an ICC2,1 of 0.95 (95% CI 0.92 to 0.97), SEM=11.7 (18.9%), MDC95=32.4 (52.2%) and the LoA were -33.61 to 31.48 for the number of steps. No difference was observed between the number of steps of the two attempts of the IST (p>0.05). CONCLUSIONS: The IST can be suggested as a valid and reliable test to assess exercise capacity in people with COPD, with no learning effect when two IST are performed on the same day. The measurement error of the IST is considered indeterminate. TRIAL REGISTRATION NUMBER: NCT04715659.

Effectiveness, Adherence and Safety of Home High Flow Nasal Cannula in Chronic Respiratory Disease and Respiratory Insufficiency: A Systematic Review.

INTRODUCTION: The effectiveness of home high flow nasal cannula (HFNC) for the treatment of chronic respiratory failure in patients with chronic respiratory diseases (CRDs) has not been summarized. We aimed to conduct a systematic review of the effectiveness, adherence, and safety of HFNC in the long-term treatment of patients with chronic respiratory diseases and respiratory failure. METHODS: A systematic review was conducted. PubMed, Web of science, and SCOPUS were search up to August 2023. Long-term HFNC studies (≥4 weeks) reporting dyspnea; exacerbations, hospitalizations; peripheral oxygen saturation (SpO2), comfort; patient experience, health-related quality of life or partial pressure of carbon dioxide (paCO2) were included. RESULTS: Thirteen articles (701 patients) based on 10 studies were selected: randomized control trials (n=3), randomized crossover trials (n=2), crossover (n=3) and retrospective (n=2) studies. COPD (n=6), bronchiectasis (n=2), COPD/bronchiectasis (n=1) and ILD (n=1) were the underlined CRDs. HFNC reduced exacerbations when compared to usual care/home respiratory therapies (n=6). Quality of life outcomes were also in favor of HFNC in patients with COPD and bronchiectasis (n=6). HFNC had significant effects on hospitalizations, paCO2, and lung function. Adherence ranged from 5.2 to 8.6h/day (n=5). Three studies reported no events, 3 non-serious events and 2 no differences compared with other home respiratory therapies. CONCLUSIONS: HFNC seems more effective than usual care or other home respiratory therapies in reducing exacerbations and improving quality of life in patients with COPD and bronchiectasis, while presenting good adherence and being safe. Its apparently superior effectiveness needs to be better studied in future real-world pragmatic trials.

Measurement of thoraco-abdominal synchrony using respiratory inductance plethysmography: technical aspects and a proposal to overcome its limitations.

BACKGROUND: Thoraco-abdominal asynchrony (TAA) is usually assessed by respiratory inductance plethysmography. The main parameter used for its assessment is the calculation of the phase angle based on Lissajous plots. However, there are some mathematical limitations to its use. RESEARCH DESIGN AND METHODS: Sequences of five breaths were selected from a) normal subjects, b) COPD patients, both at rest and during exercise, and c) patients with obstructive apnea syndrome. Automated analysis was performed calculating phase angle, loop rotation (clockwise or counterclockwise), global phase delay and loop area. TAA severity was estimated quantitatively and in subgroups. RESULTS: 2290 cycles were analyzed (55% clockwise rotation). Phase angle ranged from -86.90 to + 88.4 degrees, while global phase delay ranged from -179.75 to + 178.54. Despite a good correlation with global phase delay (p < 0.01, ANOVA test), phase angle and loop area were not able to correctly classify breaths with severe deviation and paradoxical movements (p=ns, Bonferroni post hoc test). CONCLUSIONS: Global phase delay covers the whole spectrum of TAA situations in a single value. It may be a relevant parameter for diagnosis and follow-up of clinical conditions leading to TAA. CLINICAL TRIAL REGISTRATION: The trial from which the traces were obtained was registered at ClinicalTrials.gov ;(identifier: NCT04597606).

A new characterization of adherence patterns to auto-adjusting positive airway pressure in severe obstructive sleep apnea syndrome: clinical and psychological determinants.

PURPOSE: The aim of this study was to examine the joint role of demographic, clinical, psychological and family coping variables as predictors of adherence patterns to auto-adjusting positive airway pressure (APAP). METHODS: A total of 153 patients diagnosed with obstructive sleep apnea syndrome (OSAS) were assessed during a 6-months APAP treatment period. All patients underwent psychological evaluation prior to treatment (T1) and 1 to 3 months (T2) and 4 to 6 months (T3) post-APAP treatment. Of these, 107 patients maintained a stable adherence pattern to APAP during the treatment period. RESULTS: Forty-seven percent were poorly adherent, 27 % were moderately adherent and 26 % were optimally adherent OSAS patients. Several factors distinguished the three adherence patterns and some of these emerged as the main predictors. In T1, the first model included age, apnea-hypopnea index, outcome expectations and coping spiritual support, as main predictors to distinguish adherence patterns. In T2 and T3, two models emerged adjusted to the variables of model 1 that included leakage, self-efficacy, mobilizing family acquire/accept support and reframing in model 2 and self-efficacy in model 3. Generally, the areas under the ROC curve, presented a good discrimination. CONCLUSIONS: Findings revealed an integrative heuristic model that accounted for the joint influence of demographic, clinical, psychological, and family coping factors on poor, moderate, and optimal adherence patterns.

Genetics correlates with lung function and nocturnal ventilation in myotonic dystrophy.

BACKGROUND: Dystrophia myotonica (DM) is the most frequent adult-onset muscular dystrophy. Type 1 is caused by the cytosine-thymine-guanine (CTG) repeat expansion in the DM protein kinase gene. Respiratory muscle weakness and altered central ventilatory control lead to hypercapnia and lung volume restriction. PURPOSE: This study aims to review the respiratory involvement in DM patients and study its relation with genetics. METHODS: Retrospective study of patients with DM referred for respiratory assessment was made. Noninvasive ventilation (NIV) was considered to daytime hypercapnia or symptoms of nocturnal hypoventilation. RESULTS: Forty-two consecutive patients (37.9 ± 13.6 years) were evaluated. Mean CTG length was 642.8 ± 439.2 repeats. In the first evaluation, mean forced vital capacity (FVC) was 74.4 ± 20.2 %, maximal expiratory pressure (MEP) 35 ± 16 %, maximal inspiratory pressure 52 ± 23 %, peak cough flow (PCF) 327.3 ± 97.7 L/min, arterial pressure of oxygen 79.7 ± 11.3 mmHg, arterial pressure of carbon dioxide 45.5 ± 6.2 mmHg, overnight minimal peripheral oxygen saturation (SpO2) 79.6 ± 11.6 %, and apnea-hypopnea index 13.9 ± 9.9. CTG length was found to be related with MEP (r = -0.67; p = 0.001) and SpO2 (r = -0.37; p = 0.039). NIV was started in 25 patients. Ventilated patients had lower FVC (2.19 to 3.21 L; p < 0.001) and PCF (285.3 to 388.5 L/min; p = 0.003) and more CTG repeats (826.6 to 388.5 repeats; p = 0.02). NIV compliance was poor in seven patients (28 %) and related with hypercapnia (r = 0.87; p = 0.002) and inspiratory positive airway pressure setting (r = 0.65; p = 0.009). Ventilation improved symptoms and nocturnal hypoventilation. Comparing the first and last evaluations, only PCF was significantly lower (275.0 to 310.8 L/min; p = 0.019). CONCLUSIONS: Ventilatory insufficiency is very common in patients with DM and CTG length may be useful to predict it. Prolonged NIV improves symptoms, nocturnal hypoventilation and maintains daily blood gases. Routine evaluation of PCF should not be forgotten and assisted coughing training provided.

Effectiveness of a group education session on adherence with APAP in obstructive sleep apnea--a randomized controlled study.

PURPOSE: Auto-titrating continuous positive airway pressure (APAP) is an effective treatment for obstructive sleep apnea/hypopnea syndrome (OSAHS). We investigated whether a single group education session on APAP therapy is effective in promoting adherence among patients with OSAHS. METHODS: This prospective, randomized, controlled, parallel group study included patients newly diagnosed with OSAHS who met criteria for APAP therapy. Patients were randomized into a study group and a control group. All patients in the study group were assigned to a single group education session, 1 month after beginning APAP therapy. RESULTS: We evaluated 146 patients. The median percentage of APAP usage days was 88.3 %, with a median duration per day of use of 6.02 h; 59 % were classified as adherent. Overall, no significant difference in adherence was seen between the study and the control groups. Analyzing patient subgroups, the group session significantly improved APAP adherence among males and patients who were younger (<65 years old), obese (BMI ≥ 35 kg/m(2)), non-sleepy (Epworth sleepiness scale ≤ 11), smokers or past smokers, had hypertension or nocturia and those with non-severe OSAHS. CONCLUSION: To maximize the impact of group education sessions and, by that, saving resources, it may be important to select patients likely to benefit from these sessions.

Does one size fit all? An update on chronic ventilatory support in different respiratory illnesses.

Home noninvasive ventilation (HNIV) improves outcomes in different disease categories. In this article, we discuss indications for when and how to initiate HNIV in COPD, obesity hypoventilation syndrome (OHS) and neuromuscular disorders (NMD). While in COPD, significant diurnal hypercapnia and high-intensity HNIV are essential ingredients for success, in NMD and OHS, early respiratory changes are best detected during sleep through oxy-capnography associated (or not) with respiratory polygraphy. In COPD and OHS, it is crucial to consider the coexistence of obstructive sleep apnoea because treatment with continuous positive airway pressure may be the simplest and most effective treatment that should be proposed even in hypercapnic patients as first-line therapy. In NMD, the need for continuous HNIV and eventual switching to tracheostomy ventilation makes this group's management more challenging. Achieving successful HNIV by improving quality of sleep, quality of life and keeping a good adherence to the therapy is a challenge, above all in COPD patients. In OHS patients, on top of HNIV, initiation of other interventions such as weight loss management is crucial. More resources should be invested in improving all these aspects. Telemonitoring represents a promising method to improve titration and follow-up of HNIV.