Sleep disordered breathing assessment in patient with slowly progressive neuromuscular disease.

BACKGROUND: Sleep-disordered breathing (SDB) is common in patients with neuromuscular diseases (NMD). Focusing on hypercapnia may lead to the neglect of other SDB such as obstructive and/or central sleep apnea syndrome (SAS). Our objectives were to assess the risk of inappropriate SDB management according to different screening strategies and to evaluate the prevalence and determinants of isolated and overlapping sleep apnea in patients with slowly progressive NMD. METHODS: This monocentric, cross-sectional, retrospective study analyzed medical records of adult NMD patients referred to a sleep department. Diagnostic strategies, including respiratory polygraphy (RP), nocturnal transcutaneous capnography (tcCO2), and blood gases (BG), were assessed for their performance in diagnosing SDB. Demographics and pulmonary function test results were compared between patients with or without SDB to identify predictors. RESULTS: Among the 149 patients who underwent a full diagnostic panel (RP + tcCO2 + BG), 109 were diagnosed with SDB. Of these, 33% had isolated SAS, and central apneas were predominant. Using single diagnostic strategies would lead to inappropriate SDB management in two thirds of patients. A combination of 2 diagnostic tools resulted respectively in 21.1, 22.9 and 42.2 % of inappropriate SDB management for RP + tcCO2, RP + BG and tcCO2 + BG. CONCLUSION: The significant prevalence of sleep apnea syndrome in patients with slowly progressive NMD highlights the need for increased awareness among clinicians. Improved diagnostics involve a systematic approach addressing both sleep apnea and diurnal and nocturnal alveolar hypoventilation to avoid inappropriate management and limit the consequences of SDB.

Adherence to continuous positive airway pressure therapy in patients with spinal cord injury and obstructive sleep apnea: trajectories and predictors.

PURPOSE: To identify specific determinants of non-adherence or cessation of continuous positive airway pressure (CPAP) therapy in a population of patients with spinal cord injuries (SCI). METHODS: Retrospective analysis of data from patients with SCI who underwent a full night supervised polysomnography between 2015 and 2021 and presented with moderate to severe obstructive sleep apnea (OSA) and for whom CPAP was indicated. Adherence was studied at 1, 6, and 12 months. Univariate and multivariate analyses were performed to identify factors associated with non-adherence (< 4 h per night or CPAP cessation). Factors studied were demographic and disease-related data and both subjective and objective sleep parameters. RESULTS: A total of 60 patients were included (40% cervical SCI). In univariate analysis, the only predictive parameters of non-adherence observed at 1, 6, and 12 months were the average use of CPAP on the 1st night (p = 0.02) and over the 1st week (p ≤ 0.001). A complete lesion (AIS-A) was predictive of non-adherence at 1 and 6 months (p = 0.02 at 6 months), while mask leakage was associated with non-adherence at 12 months (p = 0.02). Upper limb autonomy and the presence of family caregivers did not appear to be protective. In multivariate analysis, only the average use in the first week remained predictive of adherence (> 4 h) in the short, medium and long term. CONCLUSION: In patients with SCI and OSA, the 1st week of CPAP treatment seems to be determinant of short-, medium-, and long-term CPAP adherence. Support for SCI patients from the start of treatment is essential and may help avoid treatment failures.

Prognosis of Right Ventricular Systolic Dysfunction in Patients With Duchenne Muscular Dystrophy.

Background Chronic respiratory failure and heart involvement may occur in Duchenne muscular dystrophy. We aimed to assess the prognostic value of the right ventricular (RV) systolic dysfunction in patients with Duchenne muscular dystrophy. Methods and Results We studied 90 genetically proven patients with Duchenne muscular dystrophy from 2010 to 2019, to obtain respiratory function and Doppler echocardiographic RV systolic function. Prognostic value was assessed in terms of death and cardiac events. The median age was 27.5 years, and median forced vital capacity was at 10% of the predicted value: 83 patients (92%) were on home mechanical ventilation. An RV systolic dysfunction was found in 46 patients (51%). In patients without RV dysfunction at inclusion, a left ventricular systolic dysfunction at inclusion was associated with a higher risk of developing RV dysfunction during follow-up with an odds ratio of 4.5 (P=0.03). RV systolic dysfunction was significantly associated with cardiac events, mainly acute heart failure (62%) and cardiogenic shock (23%). In a multivariable Cox model, the adjusted hazard ratio was 4.96 (95% CI [1.09-22.6]; P=0.04). In terms of death, we found a significant difference between patients with RV dysfunction versus patients without RV dysfunction in the Kaplan-Meier curves (log-rank P=0.045). Conclusions RV systolic dysfunction is frequently present in patients with Duchenne muscular dystrophy and is associated with increased risk of cardiac events, irrespective of left ventricular dysfunction and mechanical ventilation. Registration URL: https://www.clinicaltrials.org; unique identifier: NCT02501083.

Characteristics of Patients With Late-Onset Pompe Disease in France: Insights From the French Pompe Registry in 2022.

BACKGROUND AND OBJECTIVES: The French Pompe disease registry was created in 2004 for study of the natural course of the disease in patients. It rapidly became a major tool for assessing the long-term efficacy of enzyme replacement therapy (ERT) after the market release of alglucosidase-alfa. METHODS: Approximately 10 years after publication of the baseline characteristics of the 126 initial patients of the French Late-Onset Pompe Disease registry, we provide here an update of the clinical and biological features of patients included in this registry. RESULTS: We describe 210 patients followed at 31 hospital-based French neuromuscular or metabolic centers. The median age at inclusion was 48.67 ± 14.91 years. The first symptom was progressive lower limb muscle weakness, either isolated (50%) or associated with respiratory symptoms (18%), at a median age of 38 ± 14.9 years. At inclusion, 64% of the patients were able to walk independently and 14% needed a wheelchair. Positive associations were found between motor function measure, manual motor test, and 6-minute walk test (6MWT) results, and these parameters were inversely associated with the time taken to sit up from a lying position at inclusion. Seventy-two patients had been followed for at least 10 years in the registry. Thirty-three patients remained untreated a median of 12 years after symptom onset. The standard ERT dose was administered for 177 patients. DISCUSSION: This update confirms previous findings for the adult population included in the French Pompe disease registry, but with a lower clinical severity at inclusion, suggesting that this rare disease is now diagnosed earlier; thanks to greater awareness among physicians. The 6MWT remains an important method for assessing motor performance and walking ability. The French Pompe disease registry provides an exhaustive, nationwide overview of Pompe disease and can be used to assess individual and global responses to future treatments.

Effect of Intensity of Home Noninvasive Ventilation in Individuals With Neuromuscular and Chest Wall Disorders: A Systematic Review and Meta-Analysis of Individual Participant Data.

INTRODUCTION: Home noninvasive ventilation (NIV), targeting a reduction of carbon dioxide with a combination of sufficient inspiratory support and backup-rate improves outcomes in patients with chronic obstructive pulmonary disease. The aim of this systematic review with individual participant data (IPD) meta-analysis was to evaluate the effects of intensity of home NIV on respiratory outcomes in individuals with slowly progressive neuromuscular (NMD) or chest-wall disorders (CWD). METHODS: Controlled, non-controlled and cohort studies indexed between January-2000 and December-2020 were sought from Medline, Embase and the Cochrane Central Register. Outcomes were diurnal PaCO2, PaO2, daily NIV usage, and interface type (PROSPERO-CRD 42021245121). NIV intensity was defined according to the Z-score of the product of pressure support (or tidal volume) and backup-rate. RESULTS: 16 eligible studies were identified; we obtained IPD for 7 studies (176 participants: 113-NMD; 63-CWD). The reduction in PaCO2 was greater with higher baseline PaCO2. NIV intensity per se was not associated with improved PaCO2 except in individuals with CWD and the most severe baseline hypercapnia. Similar results were found for PaO2. Daily NIV usage was associated with improvement in gas exchange but not with NIV intensity. No association between NIV intensity and interface type was found. CONCLUSION: Following home NIV initiation in NMD or CWD patients, no relationship was observed between NIV intensity and PaCO2, except in individuals with the most severe CWD. The amount of daily NIV usage, rather than intensity, is key to improving hypoventilation in this population during the first few months after introduction of therapy.

Comparison of Four Mechanical Insufflation-Exsufflation Devices: Effect of Simulated Airway Collapse on Cough Peak Flow.

BACKGROUND: Mechanical insufflation-exsufflation (MI-E) devices are used to improve airway clearance in individuals with acute respiratory failure. Some MI-E devices measure cough peak flow (CPF) during MI-E to optimize pressure adjustments. The aim was to compare CPF and effective cough volume (ECV: volume expired/coughed > 3 L/s) measurements between 4 MI-E devices under simulated conditions of stable versus collapsed airway. METHODS: Four MI-E devices were tested on the bench. Each device was connected via a standard circuit to a collapsible tube placed in an airtight chamber that was attached to a lung model with adjustable compliance and resistance. Pressure was measured upstream and downstream the collapsing tube; air flow was measured between the chamber and the lung model. Each device was tested in 2 conditions: collapse condition (0 cm H2O) and no-collapse condition (-70 cm H2O). For each condition, 6 combinations of inspiratory/expiratory pressures were applied. CPF was measured at the "mouth level" by the device built-in flow meter and at the "tracheal level" by a dedicated pneumotachograph. Comparisons were performed with non-parametric tests. RESULTS: CPF values measured at the tracheal level and ECV values differed between devices for each inspiratory/expiratory pressure in the collapse and no-collapse conditions (P < .001). CPF values were significantly lower at the tracheal level in the collapse as compared with the no-collapse condition (P < .001 for each device), whereas they were higher at the mouth level (P < .05) for 3 of the 4 devices. CONCLUSIONS: CPF values differed significantly across MI-E devices, highlighting limitation(s) of using only CPF values to determine cough effectiveness. In simulated of airway collapse, CPF increased at the mouth, whereas it decreased at the tracheal level.

Accuracy of Resting Energy Expenditure Estimation Equations in Polio Survivors.

OBJECTIVE: To evaluate the accuracy of 4 equations validated for the general population to determine resting energy expenditure (REE) in polio survivors. DESIGN: A descriptive, ambispective, single-center observational cohort study of minimal risk care. SETTING: Tertiary university care hospital. PARTICIPANTS: DATAPOL database of polio survivors followed up in a specialist department (N=298). INTERVENTIONS: None. MAIN OUTCOMES MEASURES: REE measurement by indirect calorimetry and estimated REE using 4 equations and comparing the values with indirect calorimetry. Analysis of correlations between measured REE and weight, height, and body mass index (BMI) and indicators of severity of polio sequelae. RESULTS: Of the 298 polio cases in the database between January 2014 and May 2017, 41 were included (19 men and 22 women). Mean±SD BMI was 26.0±5.6 kg/m2 (56.1% below 25). Measured REE correlated significantly and positively with weight and weaker with BMI. Correlations between measured and estimated REE were strong (between 0.49 and 0.59); correlations were strongest for the simplified World Health Organization and the Harris and Benedict equations. However, the equations systematically overestimated REE by more than 20%, especially in men. We calculated a correction factor for the World Health Organization scale: -340.3 kcal/d for women and -618.8 kcal/d for men. CONCLUSION: Analysis of REE is important for polio survivors; The use of estimation equations could lead to the prescription of a nonadapted diet. We determined a correction factor that should be validated in prospective studies.

Noninvasive Ventilation Automated Technologies: A Bench Evaluation of Device Responses to Sleep-Related Respiratory Events.

BACKGROUND: Noninvasive ventilation (NIV) is the reference standard treatment for most situations of chronic respiratory failure. NIV settings must be titrated to both preserve upper-airway patency and control hypoventilation. Automatic adjustment of pressure support (PS) and expiratory positive airway pressure (EPAP) may facilitate the initiation and follow-up of domiciliary NIV. However, whether the automatic-adjustment algorithms embedded into current devices accurately detect, respond to, and score common sleep-related respiratory events remains unclear. METHODS: A bench was set up to simulate central hypopnea (CH), central apnea (CA), obstructive hypopnea (OH), and obstructive apnea (OA). Four home ventilators were evaluated, with their dedicated modes for automatic PS and EPAP adjustment. RESULTS: All 4 devices increased PS during CH, CA, and OH. However, PS adjustment varied widely in magnitude, with tidal volumes within 100 ± 20% of the target being provided by only 3 devices for CH, one for CA, and one for OH. Two devices increased EPAP for OH and 3 for OA, including one that also increased EPAP for CA. Only 2 devices scored residual hypopnea after simulated CA, and only one scored a residual event after OH. One device scored no event. CONCLUSIONS: Current NIV devices differed markedly in their responses to, and reporting of, standardized sleep-related respiratory events. Further improvements in embedded NIV algorithms are needed to allow more widespread out-of-laboratory initiation and follow-up of NIV.

Effect of an enteral amino acid blend on muscle and gut functionality in critically ill patients: a proof-of-concept randomized controlled trial.

BACKGROUND: A defining feature of prolonged critical illness is muscle wasting, leading to impaired recovery. Supplementation with a tailored blend of amino acids may bolster the innate gut defence, promote intestinal mucosa repair and limit muscle loss. METHODS: This was a monocentric, randomized, double-blind, placebo-controlled study that included patients with sepsis or acute respiratory distress syndrome. Patients received a specific combination of five amino acids or placebo mixed with enteral feeding for 21 days. Markers of renal function, gut barrier structure and functionality were collected at baseline and 1, 2, 3 and 8 weeks after randomization. Muscle structure and function were assessed through MRI measurements of the anterior quadriceps volume and by twitch airway pressure. Data were compared between groups relative to the baseline. RESULTS: Thirty-five critically ill patients were randomized. The amino acid blend did not impair urine output, blood creatinine levels or creatinine clearance. Plasma citrulline levels increased significantly along the treatment period in the amino acid group (difference in means [95% CI] 5.86 [1.72; 10.00] nmol/mL P = 0.007). Alanine aminotransferase and alkaline phosphatase concentrations were lower in the amino acid group than in the placebo group at one week (ratio of means 0.5 [0.29; 0.86] (P = 0.015) and 0.73 [0.57; 0.94] (P = 0.015), respectively). Twitch airway pressure and volume of the anterior quadriceps were greater in the amino acid group than in the placebo group 3 weeks after randomization (difference in means 10.6 [0.99; 20.20] cmH20 (P = 0.035) and 3.12 [0.5; 5.73] cm3/kg (P = 0.022), respectively). CONCLUSIONS: Amino acid supplementation increased plasma citrulline levels, reduced alanine aminotransferase and alkaline phosphatase levels, and improved twitch airway pressure and anterior quadriceps volume. Trial registration ClinicalTrials.gov, NCT02968836. Registered November 21, 2016.

A Restructured Hospital Into a One-Building Organization for COVID-19 Patients: A Resilient and Effective Response to the Pandemic.

The COVID-19 pandemic is a unique crisis challenging healthcare institutions as it rapidly overwhelmed hospitals due to a large influx of patients. This major event forced all the components of the healthcare systems to adapt and invent new workflows. Thus, our tertiary care hospital was reorganized entirely. During the cruising phase, additional staff was allocated to a one-building organization comprising an intensive care unit (ICU), an acute care unit, a physical medicine and rehabilitation unit, and a COVID-19 screening area. The transfer of patients from a ward to another was more efficient due to these organizations and pavilion structure. The observed mortality was low in the acute care ward, except in the palliative unit. No nosocomial infection with SARS-CoV-2 was reported in any other building of the hospital since this organization was set up. This type of one-building organization, integrating all the components for comprehensive patient care, seems to be the most appropriate response to pandemics.

Macroglossia: A potentially severe complication of late-onset Pompe disease.

BACKGROUND: Pompe disease is a rare neuromuscular disorder caused by a deficiency of a lysosomal enzyme, acid α-glucosidase. Macroglossia is a classic clinical sign of several inherited myopathies and has also been reported to occur progressively in late-onset Pompe disease (LOPD). METHODS: We describe patients with LOPD and macroglossia included in the French national Pompe disease registry. Clinical, functional, and radiological data were collected during periodic follow-up and analyzed retrospectively. These cases were compared with 15 previously reported cases. RESULTS: Five patients, three females and two males, aged 71-88 years, were included in this study. All but one of the patients suffered from symptoms related to macroglossia before the diagnosis of Pompe disease. Three had localized tongue atrophy and one had significant localized tongue hypertrophy which led to glossectomy 10 years before diagnosis. Two patients had severe dysphagia, one of whom underwent gastrostomy for enteral nutritional support. One patient experienced the persistence of numerous sleep apneas despite nocturnal bilevel positive airway pressure (BiPAP) ventilation. All our patients had dysarthria, and two required speech therapy. Four patients had a tongue hypersignal on magnetic resonance imaging (MRI) T1 sequences. CONCLUSIONS: Detection of macroglossia should be part of the clinical diagnosis and follow-up of patients with LOPD, with a careful evaluation of its main consequences. Macroglossia can have severe functional impacts on speech, swallowing, and sleep. Whole-body MRI with facial sections may facilitate the early diagnosis of Pompe disease with the "bright tongue sign".

Diaphragm Ultrasound in Cardiac Surgery: State of the Art.

In cardiac surgery, patients are at risk of phrenic nerve injury, which leads to diaphragm dysfunction and acute respiratory failure. Diaphragm dysfunction (DD) is relatively frequent in cardiac surgery and particularly affects patients after coronary artery bypass graft. The onset of DD affects patients' prognosis in term of weaning from mechanical ventilation and hospital length of stay. The authors present a narrative review about diaphragm physiology, techniques used to assess diaphragm function, and the clinical application of diaphragm ultrasound in patients undergoing cardiac surgery.

Predictors of Hospitalization and Superinfection in Viral Respiratory Tract Infections Between Influenza and Paramyxoviruses: The SUPERFLUOUS Study.

BACKGROUND: Viral respiratory tract infections (VRTIs) are among the most common diseases, but the risks of superinfection for different virus species have never been compared. METHODS: Multicenter retrospective study conducted among adults who tested positive for VRTIs with reverse-transcription polymerase chain reaction. We compared characteristics between influenza (A or B) and paramyxoviruses (respiratory syncytial virus, parainfluenza virus types 1 and 3, and human metapneumovirus) and identified predictors of superinfection and hospitalization.s. RESULTS: Five hundred ninety patients had VRTI, including 347 (59%) influenza and 243 paramyxovirus infections with comparable rates of superinfections (53% vs 60%). In multivariate analyses, the predictors of superinfections were age >75 years (adjusted odds ratio, 2.37 [95% confidence interval, 1.65-3.40]), chronic respiratory disease (1.79 [1.20-2.67]), and biological abnormalities, including neutrophil count >7000/µL (1.98 [1.34-2.91)], eosinophil count <50/µL (2.53 [1.61-3.98], and procalcitonin level >0.25ng/mL (2.8 [1.65-4.73]). The predictors of hospitalization were age >75 years old (adjusted odds ratio, 3.49 [95% confidence interval, 2.17-5.63]), paramyxovirus infection (2.28 [1.39-3.75]), long-term use of inhaled corticosteroids (2.49 [1.13-5.49]), and biological abnormalities, including neutrophil count >7000/µL (2.38 [1.37-4.12)] and procalcitonin level >0.25ng/mL (2.49 [1.23-5.02]). Kaplan-Meier survival curves showed that influenza-infected patients had a higher mortality rate than those with paramyxovirus infections (8.9% vs 4.5%, respectively; P = .02). CONCLUSIONS: Our study revealed a high rate of superinfection (56%), not related to viral species. However influenza virus was associated with a poorer prognosis than paramyxoviruses, pleading for a broader and large-scale vaccination of individual at risk of VRTIs.

Determinants of diaphragm inspiratory motion, diaphragm thickening, and its performance for predicting respiratory restrictive pattern in Duchenne muscular dystrophy.

INTRODUCTION/AIMS: Respiratory status is a key determinant of prognosis in patients with Duchenne muscular dystrophy (DMD). We aimed to evaluate the determinants of diaphragm ultrasound and its performance in predicting restrictive respiratory patterns in DMD. METHODS: This was a retrospective study of DMD patients followed in our center and admitted for an annual checkup from 2015 to 2018. We included DMD patients who underwent diaphragm ultrasound and pulmonary functional tests. RESULTS: This study included 74 patients with DMD. The right diaphragm thickening fraction (TF) was significantly associated with age (P = .001), Walton score (P = .012), inspiratory capacity (IC) (P = .004), upright forced vital capacity (FVC) (P < .0001), supine FVC (P = .038), and maximal inspiratory pressure (MIP) (P = .002). Right diaphragm excursion was significantly associated with age (P < .0001), steroid use (P = .008), history of spinal fusion (P < .0001), body mass index (BMI) (P = .002), Walton score (P < .0001), IC (P < .0001), upright FVC (P < .0001), supine FVC (P < .0001), and MIP (P < .0001). A right diaphragm TF >28% and a right diaphragm excursion>25.4 mm were associated with an FVC >50% with, respectively, an area under the curve (AUC) of 0.95 (P = .001) and 0.93 (P < .001). A left diaphragm TF >26.8% and a left diaphragm excursion >21.5 mm were associated with an FVC >50% with, respectively, an AUC of 0.95 (P = .011) and 0.97 (P < .001). DISCUSSION: Diaphragm excursion and diaphragm TF can predict restrictive pulmonary insufficiency in DMD.

Protective Recommendations for Non-invasive Ventilation During COVID-19 Pandemic: A Bench Evaluation of the Effects of Instrumental Dead Space on Alveolar Ventilation.

INTRODUCTION: With the current COVID-19 pandemic, concerns have raised regarding the risk for NIV to promote airborne transmission. In case of hospital admission, continuation of therapy in patients undergoing chronic NIV is necessary and several protective circuit configurations have been recommended to reduce the risk of aerosol dissemination. However, all these configurations increase instrumental dead space. We therefore designed this study to evaluate their effects on the tidal volume (VTE) required to preserve stable end-tidal CO2 partial pressure (PETCO2) with constant respiratory rate. METHODS: A bench consisting of a test lung connected to an adult-sized mannequin head was set up. The model was ventilated through usual domiciliary configuration (single limb circuit with facial vented mask) which was used as reference. Then, five different circuit configurations including non-vented facial mask with viral/bacterial filter, modification of leak position, and change from single to double-limb circuit were evaluated. For each configuration, pressure support (PS) was gradually increased to reach reference PETCO2. Resulting VTE was recorded as primary outcome. RESULTS: Reference PETCO2 was 38(0) mmHg, with a PS set at 10 cmH2O, resulting in a VTE of 432(2) mL. Compared to reference, all the configurations evaluated required substantial increase in VTE to preserve alveolar ventilation, ranging from +79(2) to +216(1) mL. CONCLUSIONS: Modifications of NIV configurations in the context of COVID-19 pandemic result in substantial increase of instrumental dead space. Re-evaluation of treatment efficiency and settings is crucial whenever protective measures influencing NIV equipment are considered.

INTRODUCCIÓN: Durante la actual pandemia de COVID-19 ha surgido la preocupación sobre el posible riesgo de que la ventilación no invasiva (VNI) promueva la transmisión aérea. En el caso de ingreso hospitalario, es necesario continuar con el tratamiento de aquellos pacientes tratados con VNI crónica y se han recomendado varias configuraciones protectoras de los circuitos para reducir el riesgo de diseminación por aerosoles. Sin embargo, todas estas configuraciones aumentan el espacio muerto instrumental. Así, diseñamos este estudio para evaluar los efectos de estas configuraciones sobre el volumen corriente (VCE) necesario para mantener estable la presión parcial de CO2 al final del volumen corriente espirado (PETCO2) con una frecuencia respiratoria constante. MÉTODOS: Se construyó un modelo experimental que constaba de un pulmón de prueba conectado a una cabeza de maniquí de tamaño adulto. El modelo recibió ventilación utilizando la configuración domiciliaria habitual (circuito de rama única con máscara facial ventilada), lo que se utilizó como referencia. Después se evaluaron cinco configuraciones diferentes del circuito, incluidas la máscara facial sin ventilación con filtro antiviral/antibacteriano, la modificación de la posición de la fuga y el cambio de circuito de rama única a doble rama. Para cada configuración, la presión de soporte (PS) se incrementó gradualmente hasta alcanzar la PETCO2 de referencia. El VCE resultante se registró como resultado primario. RESULTADOS: La PETCO2 de referencia fue de 38(0) mmHg, con una PS fijada en 10 cmH2O, lo que resultó en un VCE de 432(2) mL. En comparación con la referencia, todas las configuraciones evaluadas requirieron un aumento sustancial del VCE para preservar la ventilación alveolar, en un rango entre +79(2) mL y +216(1) mL. CONCLUSIONES: Las modificaciones de las configuraciones de VNI en el contexto de la pandemia de COVID-19 resultan en un aumento sustancial del espacio muerto instrumental. Reevaluar la eficacia y los ajustes del tratamiento es fundamental cuando se ponen en consideración unas medidas de protección que influyen en el equipo de VNI.

Swallowing dysfunction in patients hospitalised due to a COPD exacerbation: correspondence.

This correspondence deals with physiopathological studies that aimed to understand the underlying mechanisms of breathing-swallowing interaction https://bit.ly/3BKAwx3.

Nutritional status, swallowing disorders, and respiratory prognosis in adult Duchenne muscular dystrophy patients.

Malnutrition and swallowing disorders are common in Duchenne muscular dystrophy (DMD) patients. We assessed, in adult DMD with home mechanical ventilation (HMV) and cough assist device, its prevalence and the relationships with respiratory muscle strength and long-term respiratory prognosis. We reviewed the patients (n = 117, age 18-39 years [median 24]), followed in a reference center, from 2006 to 2015, to obtain clinical baseline, nutritional status, vital capacity (VC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP). The median body mass index (BMI) was low (15.6 kg/m²). Included patients had severe restrictive respiratory function with a median VC of 10.5% [7-17] of the predicted value. All patients were on HMV. Prevalence of malnutrition, swallowing disorders, and gastrostomy were respectively 62%, 34%, and 11%. BMI and serum albumin level were significantly associated with MIP, MEP, and VC. The 1-year/5-years cumulative incidences of respiratory events (pulmonary sepsis and acute respiratory distress) were, respectively, 20.7%/44.5%. Using univariate analysis, predictive factors for respiratory events were swallowing disorders (p = .001), transthyretinemia (p = .034), MIP (p = .039), and MEP (p = .03) but not BMI or albuminemia. Using multivariate analysis, only swallowing disorders remained significantly associated with respiratory events (OR = 4.2, IC 95% 1.31-12.2, p = .01). In conclusion, this study highlights the interrelationships between nutritional intake, swallowing function, airway clearance, and respiratory function in adult DMD. A multidisciplinary approach focusing on these previous factors is essential to optimize DMD patient health.

Mouthpiece ventilation in neuromuscular disorders: Narrative review of technical issues important for clinical success.

In neuromuscular disorders (NMDs), nocturnal non-invasive ventilation (NIV) via a nasal mask is offered when hypercapnic respiratory failure occurs. With disease progression, nocturnal NIV needs to be extended into the daytime. Mouthpiece ventilation (MPV) is an option for daytime NIV. MPV represents a difficult task for home ventilators due to rapidly changing load conditions resulting from intermittent connections and disconnections from MPV circuit. The 252nd ENMC International Expert Workshop, held March 6th to 8th 2020 in Amsterdam, reported general guidelines for management of daytime MPV in NMDs. This report could not present all the detail regarding the technical issues important for clinical success of MPV. Based on the expert workshop discussions and the evidence from existing studies, the current narrative review aims to identify the technical issues of MPV and offers guidance via a decisional algorithm and educational figures providing relevant information that is important for successful implementation of MPV.

Impact of Interface Type on Noninvasive Ventilation Efficacy in Patients With Neuromuscular Disease: A Randomized Cross-Over Trial / Impacto del tipo de mascarilla en la eficacia de la ventilación no invasiva en pacientes con enfermedad neuromuscular: un ensayo clínico aleatorizado cruzado

Background and objective: Around 25% of patients with neuro-muscular diseases (NMD) are treated by home noninvasive ventilation (NIV) through an oronasal mask. However, there is growing evidence that nasal masks require lower NIV pressures and result in fewer residual obstructive events. We hypothesized that nasal masks would improve efficacy and reduce side effects compared to oronasal masks in this population.Methods: open label, cross-over, randomized, study in 2 tertiary care hospitals. Patients with NMD treated by home NIV were randomized for one-week periods to nasal and oronasal interfaces respectively (cross-over). At the end of each period, nocturnal polygraphy (monitoring mouth opening) under NIV, synchronized with transcutaneous partial pressure in CO2 (tcCO2) was performed. Data were collected from the NIV built-in software and NIV side-effects were collected. Intention-to-treat and per protocol analyses were performed. The primary outcome was mean nocturnal SpO2. The secondary outcomes were: percentage of sleep with SpO2<90%, oxygen desaturation index (ODI), mean tcCO2, mean duration of mouth opening during sleep, level of non-intentional leaks and side-effects.Results: Thirty patients with NMD were included. There were no between-group differences for either the primary or secondary outcomes. Post hoc comparisons showed that changing between interfaces reduced NIV efficacy: mean nocturnal SpO2 (p=0.04), ODI (p=0.01), mean tcCO2 (p=0.048), side-effects (p=0.008). (AU)

Antecedentes y objetivo: Alrededor del 25% de los pacientes con enfermedades neuromusculares (ENM) son tratados mediante ventilación no invasiva (VNI) a través de una máscara oronasal. Sin embargo, existen crecientes indicios de que las máscaras nasales requieren presiones de VNI más bajas y resultan en menos eventos obstructivos residuales. Nuestra hipótesis fue que las máscaras nasales mejorarían la eficacia y reducirían los efectos secundarios en comparación con las máscaras oronasales en esta población.Métodos: Estudio abierto, cruzado, aleatorizado en 2 hospitales de atención terciaria. Los pacientes con ENM tratados mediante VNI domiciliaria fueron aleatorizados durante períodos de una semana de duración a las mascarillas nasales y oronasales, alternativamente (cruzado). Al final de cada período se realizó una polisomnografía nocturna (con monitorización de la apertura bucal) con VNI, sincronizada con la medición transcutánea de la presión parcial de CO2 (tcCO2). Los datos se recopilaron utilizando el software integrado en la VNI y se recogieron los efectos secundarios de la VNI. Se realizaron análisis por intención de tratar y por protocolo. El criterio de valoración principal fue la SpO2 nocturna media. Los criterios secundarios fueron: porcentaje de sueño con SpO2<90%, índice de desaturación de oxígeno (IDO), tcCO2 media, duración media de la apertura bucal durante el sueño, nivel de fugas no intencionales y efectos secundarios.Resultados: Se incluyeron 30 pacientes con ENM. No hubo diferencias entre los grupos para los resultados primarios o secundarios. Las comparaciones a posteriori mostraron que cambiar mascarillas reducía la eficacia de la VNI: SpO2 nocturna media (p=0,04), IDO (p=0,01), tcCO2 media (p=0,048) y efectos secundarios (p=0,008). (AU)