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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., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

The aim of this study was to describe the endotracheal respiratory flora in a population of adults suffering from neuromuscular or neurological disorders requiring a long-term tracheostomy and to identify risk factors for colonization. We conducted a prospective and single-center observational study among patients with chronic tracheostomy admitted for planned respiratory assessment between February 2015 and December 2016. Data were collected from patient interview and medical charts with a standardized questionnaire. A tracheal aspiration was performed for each patient. Humidifiers were analysed for bacteriological contamination. Overall 77 tracheal aspirates (TA) were obtained from patients included. Pathogenic bacteria were found in 90% of cases (69/77) with a majority of Pseudomonas aeruginosa (32/77, 41%), Staphylococcus aureus (34/77, 44%) and Serratia marcesens. (22/79, 38%) Amoxicillin + Clavulanic-acid and Cefotaxime were adapted for respectively in only 28% and 35% of the subjects due to the natural resistance of organisms. No pathogenic bacteria were isolated from humidifier samples. Risk factors significantly associated with P. aeruginosa colonization were residence in a medical-care home (p = 0.01, OR = 3.8 [1.1; 15.1]) and the presence of a cuff (p = 0.003, OR = 4.4 [1.1; 20.6]). Significant quantities of pathogenic bacteria are frequently isolated from TA of tracheostomised patients in the absence of infection. The frequent resistance of these pathogens to Amoxicillin + Clavulanic-acid precludes the use of this antibiotic in the empiric treatment of pneumonia in this population., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Many patients with DMD undergo tracheostomy. Tracheostomy is associated with certain complications, however its effect on prognosis is not known., Methods: The relationship between type of mechanical ventilation and survival at 12 years was evaluated in a prospective cohort of patients with Duchenne muscular dystrophy followed in a French reference center for Neuromuscular Diseases. Cox proportional-hazards regressions were used to estimate the hazard ratios associated with risk of switching from non-invasive to invasive ventilation, and with risk of death., Results: One hundred and fifty patients were included. Initial use of invasive ventilation was associated with an episode of acute respiratory failure (p < 0.0001) and with a severe clinical status (p < 0.05). Risk of death was associated with swallowing disorders (2.51, IC [1.12-5.66], p < 0.03) and cardiac failure (p < 0.05) but not with type of mechanical ventilation., Conclusion: Switching to invasive ventilation is appropriate when non-invasive ventilation is ineffective., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Background: The objective was to determine whether optoelectronic plethysmography (OEP) can detect asymmetric ventilation related to unilateral or asymmetric diaphragmatic weakness, suggesting usefulness as a diagnostic tool., Methods: Thirteen patients with suspected asymmetric diaphragmatic weakness based on dyspnea and hemidiaphragm elevation on the chest radiograph were studied as well as three patients with maltase acid deficiency (a cause of symmetrical diaphragmatic weakness). The transdiaphragmatic pressure response to unilateral magnetic stimulation (lateral twitch transdiaphragmatic pressure [latPdiTw]) and the diaphragm compound muscle action potentials (CMAPs) elicited by transcutaneous electrical stimulation of each phrenic nerve as well as OEP were performed., Results: The CMAPs and latPdiTw showed unilateral or predominantly unilateral diaphragmatic weakness in nine of the 13 patients. By OEP, the affected side of the thorax and abdomen contributed &lt; 45% of the inspiratory capacity in each of these nine patients, whereas no asymmetry was noted in the other four patients or in the three patients with maltase acid deficiency. All patients preferred OEP over CMAP or latPdiTw., Conclusions: OEP detected asymmetric ventilation in all patients diagnosed with unilateral diaphragm weakness and in no patients without this diagnosis. Thus, OEP is an effective noninvasive alternative that is preferred by the patients over CMAP response and latPdiTw.

Objective: Communication is a major issue for patients with tracheostomy who are supported by mechanical ventilation. The use of positive end-expiratory pressure (PEEP) may restore speech during expiration; however, the optimal PEEP level for speech may vary individually. We aimed to improve speech quality with an individually adjusted PEEP level delivered under the patient's control to ensure optimal respiratory comfort., Methods: Optimal PEEP level (PEEPeff), defined as the PEEP level that allows complete expiration through the upper airways, was determined for 12 patients with neuromuscular disease who are supported by mechanical ventilation. Speech and respiratory parameters were studied without PEEP, with PEEPeff, and for an intermediate PEEP level. Flow and airway pressure were measured. Microphone speech recordings were subjected to both quantitative and qualitative assessments of speech, including an intelligibility score, a perceptual score, and an evaluation of prosody determined by two speech therapists blinded to PEEP condition., Results: Text reading time, phonation flow, use of the respiratory cycle for phonation, and speech comfort significantly improved with increasing PEEP, whereas qualitative parameters remained unchanged. This resulted mostly from the increase of the expiratory volume through the upper airways available for speech for all patients combined, with a rise in respiratory rate for nine patients. Respiratory comfort remained stable despite high levels of PEEPeff (median, 10.0 cm H2O; interquartile range, 9.5-12.0 cm H₂O)., Conclusions: Patient-controlled PEEP allowed for the use of high levels of PEEP with good respiratory tolerance and significant improvement in speech (enabling phonation during the entire respiratory cycle in most patients). The device studied could be implemented in home ventilators to improve speech and, therefore, autonomy of patients with tracheostomy., Trial Registry: ClinicalTrials.gov; No.: NCT01479959; URL: clinicaltrials.gov.

We hypothesized that peak values of oesophageal (Poes) and transdiaphragmatic pressure (Pdi) swings during a maximal sniff manoeuvre and a maximal static inspiratory manoeuvre (Muller manoeuvre) are comparable or give complementary information for assessing diaphragmatic and global inspiratory muscle strength. We studied 98 patients with suspected diaphragmatic dysfunction. Poes and Pdi swings were measured during maximal sniff manoeuvres (sniff), maximal Muller manoeuvres (max), and cervical magnetic phrenic nerve stimulation (cervical Tw). Eighty eight patients were able to perform both volitional manoeuvres. Among them, mean Poes sniff was significantly higher than mean Poes max (48.7+/-28.7 cm H(2)O vs. 42.9+/-27.4 cm H(2)O, p<0.05) and mean Pdi sniff was higher than mean Pdi max (49.2+/-35.1cm H(2)O vs. 42.9+/-33.3 cm H(2)O, respectively, p=0.05). Cervical Pdi Tw correlated better with Pdi sniff (p<0.0001, r=0.62) than with Pdi max (p<0.0001, r=0.44). Poes and Pdi swings were greatest during the sniff manoeuvre in 42 patients (48%) and during the Muller manoeuvre in 29 patients (33%). Among the 17 remaining patients, nine had the greatest Poes swing during a maximal sniff manoeuvre and the greatest Pdi swing during a maximal static inspiratory manoeuvre; the opposite occurred in the other eight patients. The combination of Muller manoeuvre and sniff manoeuvre increased the diagnosis of normal diaphragmatic strength from 18 patients (20%) to 21 patients (24%), and the additional analysis of cervical Pdi Tw further increased the diagnosis of normal diaphragmatic strength to 27 patients (31%). In conclusion, though sniff manoeuvre gave significantly higher values than Muller manoeuvre, both volitional manoeuvres and cervical Pdi Tw are complementary and should be used in combination to evaluate diaphragmatic muscle strength.