Expiratory Pressure Research Articles

Inspiratory muscle training impact on respiratory muscle strength, pulmonary function, and quality of life in children with chest burn: A randomized controlled trial

PurposeRespiratory muscle function is compromised in children recovering from chest wall burns, which potentially leads to more impact on exercise capacity and quality of life. This study investigates the effects of an inspiratory muscle training intervention accompanied with a pulmonary rehabilitation program on respiratory muscle strength, lung function, functional capacity, and quality of life in chest burned children. MethodsForty children with burns, aged from 10 to 18 years old and total body surface area of 30% to 50%, were randomly allocated to the inspiratory muscle training group (IMT- G: n = 20) or control group (CG: n = 20). They received IMT plus pulmonary rehabilitation or pulmonary rehabilitation with sham IMT, respectively for eight weeks. The outcomes were the respiratory muscle strength measured by maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP); lung functions (FEV1, FVC and FEV1/FVC ratio); functional capacity as well as Pediatric Quality of Life to measure physical and psychosocial functioning. outcome measures were assessed at before and after intervention (after eight weeks). ResultsBased on the pre-intervention assessments, we found no significant difference between both groups (p > 0.05). Significant post-intervention differences were reported between both groups in MIP (P = .003), MEP (P = .017), FVC (P = .001), FEV1 (P = .007), FEV1/FVC ratio (P = .028), functional capacity (P = .003), physical domain of QoL (P = .006) and psychological domain of QoL (P = .002) in favor of the IMT group. ConclusionsEight weeks of inspiratory muscle training combined with pulmonary rehabilitation program improved children with chest burns' respiratory muscles strength, lung functions, functional capacity, and quality of life. Inspiratory muscle training may be employed in burn rehabilitation programs. It is a safe and effective therapy in chest burned children.

Long‐term pneumatic stenting with positive expiratory pressure therapy for severe expiratory central airway collapse

AbstractExpiratory central airway collapse (ECAC) comprising excessive central airway collapse (EDAC) and tracheobronchomalacia. Treatment is challenging for severe cases that are not candidates for surgical management. We report a case of severe ECAC successfully managed with continuous positive airway pressure (CPAP) therapy. A 75‐year‐old female patient status post right pneumonectomy, presented with chronic cough. Dynamic bronchoscopy evaluation showed severe EDAC which improved with intraprocedural noninvasive positive pressure (NIPPV) therapy. Due to patients' comorbidities, she was not a candidate to surgical tracheobronchoplasty. Therefore, we attempted pneumatic stenting with long‐term CPAP therapy resulting in improvement of symptoms and functional capacity. The long‐term efficacy of pneumatic stenting has not been clearly established yet. Literature review of management of ECAC with NIPPV consist primarily of case reports and there is only one clinical trial being conducted to assess the efficacy of CPAP therapy in ECAC. While NIPPV arises as a sufficient alternative for management of severe ECAC, larger scale studies are needed to prove the real efficacy of NIPPV in this setting.

Analysis of the effect of positive expiratory pressure therapy device on comprehensive nursing intervention for patients with chronic obstructive pulmonary disease and respiratory failure.

Analysis of the effect of positive expiratory pressure therapy device on comprehensive nursing intervention for patients with chronic obstructive pulmonary disease and respiratory failure.

Association of IGF-1 and IGF-2 genotypes with respiratory muscle strength in individuals with COPD: A cross-sectional study.

Chronic obstructive pulmonary disease is a systemic disease characterized not only by respiratory symptoms but also by physical deconditioning and muscle weakness. One prominent manifestation of this disease is the decline in respiratory muscle strength. Previous studies have linked the genotypes of insulin-like growth factor 1 and 2 (IGF-1 and IGF-2) to muscle weakness in other populations without this disease. However, there is a notable knowledge gap regarding the biological mechanisms underlying respiratory muscle weakness, particularly the role of IGF-1 and IGF-2 genotypes in this pulmonary disease. Therefore, this study aimed to investigate, for the first time, the association between IGF-1 and IGF-2 genotypes with respiratory muscle strength in individuals with chronic obstructive pulmonary disease. In addition, we analyzed the relationship between oxidative stress, chronic inflammation, and vitamin D with respiratory muscle strength. A cross sectional study with 61 individuals with chronic obstructive pulmonary disease. Polymerase chain reaction of gene polymorphisms IGF-1 (rs35767) and IGF-2 (rs3213221) was analyzed. Other variables, related to oxidative stress, inflammation and Vitamin D were dosed from peripheral blood. Maximal inspiratory and expiratory pressure were measured. The genetic polymorphisms were associated with respiratory muscle strength ( 3.0 and 3.5; = 0.57). Specific genotypes of IGF-1 and IGF-2 presented lower maximal inspiratory and expiratory pressure (<0.05 for all). Oxidative stress, inflammatory biomarkers, and vitamin D were not associated with respiratory muscle strength. The polymorphisms of IGF-1 and IGF-2 displayed stronger correlations with respiratory muscle strength compared to blood biomarkers in patients with chronic obstructive pulmonary disease. Specific genotypes of IGF-1 and IGF-2 were associated with reduced respiratory muscle strength in this population.

Reliability and Validity of Maximal Respiratory Pressures.

Maximal respiratory pressure is used to assess the inspiratory and expiratory muscles strength by using maximal inspiratory pressure (PImax) and maximal expiratory pressure (PEmax). This study aimed to summarize and evaluate the reliability and validity of maximal respiratory pressure measurements. This systematic review followed the Consensus-based Standards for the Selection of Health Measurement Instruments (COSMIN) recommendations and was reported by using the PRISMA checklist. Studies published before March 2023 were searched in PubMed and EMBASE databases. A total of 642 studies were identified by using the online search strategy and manual search (602 and 40, respectively). Twenty-three studies were included. The level of evidence for test-retest reliability was moderate for PImax and PEmax (intraclass correlation coefficient > 0.70 for both), inter-rater reliability was low for PImax and very low for PEmax (intraclass correlation coefficient > 0.70 for both), and the measurement error was very low for PImax and PEmax. In addition, concurrent validity presented a high level of evidence for PImax and PEmax (r > 0.80). Only concurrent validity of maximal respiratory pressure measured with the manometers evaluated in this review presented a high level of evidence. The quality of clinical studies by using maximal respiratory pressure would be improved if more high-quality studies on measurement properties, by following well established guidelines and the COSMIN initiative, were available.

Influence of physical exercise on respiratory muscle function in older adults: A systematic review and meta-analysis

Respiratory function decreases with aging. The literature showed that non-ventilatory specific exercise could have a positive impact on respiratory muscles. A systematic literature review was conducted to assess the effects of non-ventilatory specific exercise on maximal inspiratory (MIP) and expiratory pressure (MEP) and peak expiratory flow (PEF) in older adults. The included 9 trials investigated the effects of resistance training, yoga, Pilates, physical activity based on walking, and whole-body vibration training. The meta-analysis showed no statistically significant differences in MIP, MEP, and PEF after implementation of a non-ventilatory specific exercise program in older individuals. Between-study heterogeneity was substantial for MIP and MEP outcomes but it was not statistically significant for PEF. Further RCTs will be necessary to determine the effects of physical exercise interventions. PROSPERO registry CRD42023478262.

The Effects of Various Approaches to Lobectomies on Respiratory Muscle Strength, Diaphragm Thickness, and Exercise Capacity in Lung Cancer

BackgroundThe most common surgery for non-small cell lung cancer is lobectomy, which can be performed through either thoracotomy or video-assisted thoracic surgery (VATS). Insufficient research has examined respiratory muscle function and exercise capacity in lobectomy performed using conventional thoracotomy (CT), muscle-sparing thoracotomy (MST), or VATS. This study aimed to assess and compare respiratory muscle strength, diaphragm thickness, and exercise capacity in lobectomy using CT, MST, and VATS.MethodsThe primary outcomes were changes in respiratory muscle strength, diaphragm thickness, and exercise capacity. Maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) were recorded for respiratory muscle strength. The 6-min walk test (6MWT) was used to assess functional exercise capacity. Diaphragm thickness was measured using B-mode ultrasound.ResultsThe study included 42 individuals with lung cancer who underwent lobectomy via CT (n = 14), MST (n = 14), or VATS (n = 14). Assessments were performed on the day before surgery and on postoperative day 20 (range 17–25 days). The decrease in MIP (p < 0.001), MEP (p = 0.003), 6MWT (p < 0.001) values were lower in the VATS group than in the CT group. The decrease in 6MWT distance was lower in the MST group than in the CT group (p = 0.012). No significant differences were found among the groups in terms of diaphragmatic muscle thickness (p > 0.05).ConclusionThe VATS technique appears superior to the CT technique in terms of preserving respiratory muscle strength and functional exercise capacity. Thoracic surgeons should refer patients to physiotherapists before lobectomy, especially patients undergoing CT. If lobectomy with VATS will be technically difficult, MST may be an option preferable to CT because of its impact on exercise capacity.

Voice handicap and voice-related quality of life in COVID-19 patients

ObjectivesIdentifying voice handicap and voice-related quality of life in patients presenting pulmonary impairment associated with COVID-19 infection, comparing pulmonary parameters between these patients and individuals in the control group, as well as correlating pulmonary parameters to self-assessment questionnaires (IDV-10 and QVV). MethodsThirty-five (35) patients presenting pulmonary impairment with COVID-19 infection were herein selected and compared to 35 individuals who were not affected by COVID-19 infection. Two self-assessment questionnaires were applied (vocal handicap index and voice quality of life protocol). Maximum phonation time Forced Expiratory Pressure (PEF) and Forced Inspiratory Pressure (PIF) were measured and videolaryngoscopy was performed. ResultsThere was statistically significant difference in scores recorded in voice self-assessment questionnaires (IDV-10 and QVV), Expiratory Pressure (PEF) and Forced Inspiratory Pressure (PIF) between patients with pulmonary impairment associated with COVID-19 infection and those in the control group. Correlation between PEF/PIF and scores recorded in voice self-assessment questionnaires was also observed. ConclusionPulmonary impairment associated with COVID-19 infection has worsened voice handicap and voice-related quality of life in the assessed patients, as well as reduced their forced expiratory and inspiratory pressure in comparison to the control group. Level of evidence4.

Inspiratory Muscle Training in Patients Living With Chronic Kidney Disease and Receiving Hemodialysis: Meta-Analysis of Randomized Controlled Trials.

People living with chronic kidney disease (CKD) and receiving hemodialysis (HD) have impaired respiratory muscle strength and endurance. The objective of this study was to systematically review the effects of inspiratory muscle training (IMT) on respiratory muscle strength, functional capacity, lung function, quality of life, endothelial function, and oxidative stress in people living with CKD and receiving HD. An electronic search was conducted from inception to June 2023. Randomized controlled trials that evaluated the effects of IMT on respiratory muscle strength, functional capacity, lung function, endothelial function, quality of life, or oxidative stress in adults living with CKD and receiving HD, compared with control, placebo IMT, or conventional physical therapy, were included. Eight studies were included, totaling 246 people. The meta-analysis showed that IMT increased the maximum inspiratory pressure (MIP) by 22.53cm H2O, the maximum expiratory pressure (MEP) by 19.54cm H2O, and the distance covered in the 6-minute walk test by 77.63m. Changes in lung function and quality of life were not observed. It was not possible to quantitatively analyze data on endothelial function and oxidative stress. IMT improves MIP, MEP, and functional capacity in people living with CKD and receiving HD. IMT did not demonstrate significant results for lung function and quality of life. Effects on endothelial function and oxidative capacity remain uncertain. Inspiratory muscle training improves MIP, MEP, and functional capacity in people living with CKD and receiving HD, compared with conventional physical therapy or controls or placebo intervention. Increases in functional capacity in this population are extremely important because of the relationship with the survival of these people.

Nebulized hypertonic saline and positive expiratory pressure device use in patients with bronchiectasis: Analysis from the United States Bronchiectasis and NTM research registry

BackgroundNebulized Hypertonic saline (HS) and positive expiratory pressure device (PEP) are often used in patients with bronchiectasis. We sought to describe the clinical characteristics in patients using HS and PEP, utilizing a large national database registry. MethodsData from the US Bronchiectasis and NTM Research Registry were used in this study. Patients with a diagnosis of bronchiectasis were included. Eligible patients were assigned to one of four mutually exclusive groups: HS only, PEP only, HS & PEP, or no airway clearance or mucoactive agent. Descriptive statistics were computed for the overall study population and stratified by the four groups. One-way ANOVA and chi-square tests were used to test the difference in the means in continuous variables and the association between categorical variables (respectively) across the four groups. ResultsA total of 2195 patients were included. Of those with bronchiectasis and a productive cough, a greater number of patients utilized HS only vs PEP only (17.5 % vs 9.1 %, p < 0.001). Similar association was found in those with Pseudomonas aeruginosa (22.3 % HS only vs 6.5 % PEP only, p < 0.001). There was a higher number of patients who used HS and PEP therapy in combination vs PEP therapy alone (25.0 % vs 9.1 %, p = 0.002), in those with a productive cough. ConclusionsIn patients with bronchiectasis and a productive cough or Pseudomonas aeruginosa, HS is used more often than PEP alone. There is a need for further analysis to compare these two modalities and explore the factors influencing their utilization.

A follow-up study on respiratory outcomes, quality of life and performance perception of SARS-CoV-2 primary and reinfection in elite athletes: A 9-month prospective study.

The prolonged consequences of SARS-CoV-2 on young elite athletes recovering from primary and reinfection are unclear. This study aimed to assess inspiratory/expiratory muscle strength and respiratory function at the time of spontaneous recovery at 3, 6, and 9months after SARS-CoV-2 primary and reinfection in elite athletes. The study enrolled 25 elite male judoists, including 11 primary infection cases, five reinfection cases, and nine controls from the Türkiye Olympic Preparation Center. Inspiratory/expiratory muscle strength and respiratory function were measured, including maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), forced expiratory volume in 1s (FEV1), forced vital capacity (FVC), FEV1/FVC, and peak expiratory flow (PEF) before and up to 9months after SARS-CoV-2 infection in the early pre-competition preparation phases. The most common symptoms reported by reinfection cases were fatigue (80%), dyspnea (60%), and muscle/joint pain (60%), while primary infection cases reported fatigue (73%), muscle/joint pain (45%), and headache (45%). MIP decreased by -14% and MEP decreased by -13% following the SARS-CoV-2 infection in reinfection cases. Likewise, FEV1 and FVC decreased by -5% and -8%, respectively; consequently, FEV1/FVC increased by 3%. Inspiratory/expiratory muscle strength and respiratory function improved rapidly after 9months of SARS-CoV-2 infection in primary cases, whereas dysfunction persisted in reinfection cases. PEF was unaffected throughout the 9-month follow-up period. Reinfection may lead to further alterations in respiratory system relative to the primary infection, with a suspected restrictive pattern that remains dysfunctional in the third month; however, it improves significantly during a 9-month follow-up period.

The Influence of Lung Function and Respiratory Muscle Strength on Quadriceps Muscle Fatigability in COPD Patients Under Long-term Oxygen Therapy

BackgroundThis research investigates quadriceps muscle fatigability (MF) in chronic obstructive pulmonary disease (COPD) patients with chronic respiratory failure (CRF) at different levels of lung obstruction [severe obstruction (SO)=FEV1 <50% and >30% versus very severe obstruction (VSO)=FEV1 ≤30%]. It explores the relationships between quadriceps MF and lung function, respiratory muscles, and oxygenation status. MethodsA post hoc cross-over analysis in 45 COPD patients (20 SO and 25 VSO) undergoing long-term oxygen therapy was performed. Delta change in quadriceps maximum voluntary contraction (MVC) (absolute value and percentage) before and after a constant workload was calculated. Associations between quadriceps MF and lung function, respiratory muscles, and gas exchange were examined using Pearson's correlation and multivariate linear regression analysis. ResultsSO patients experience a more substantial reduction in MVC compared to VSO (−15.15±9.13% vs −9.29±8.90%, p=0.0357), despite comparable resting MVC. Dyspnea is more pronounced in VSO at the beginning and end of the exercise. Correlations were found between MF and maximal inspiratory pressure (MIP) (r=−0.4412, p=0.0056), maximal expiratory pressure (MEP) (r=−0.3561, p=0.0282), and a tendency for FEV1% (r=−0.2931, p=0.0507). The regression model (R2=0.4719) indicates that lower MIP and FEV1 and high total lung capacity are significant factors in reducing quadriceps muscle fatigability after a fatiguing task. ConclusionCOPD patients with more severe pulmonary obstruction and hyperinflation and lower respiratory muscle strength have lower quadriceps MF but higher dyspnea both at rest and during exercise.

0617 Efficacy of Nasal Expiratory Positive Airway Pressure in Veterans with Obstructive Sleep Apnea

0617 Efficacy of Nasal Expiratory Positive Airway Pressure in Veterans with Obstructive Sleep Apnea

0609 Improved Sleep Quality with Expiratory Positive Airway Pressure Enhanced Mandibular Advancement Device in OSA

0609 Improved Sleep Quality with Expiratory Positive Airway Pressure Enhanced Mandibular Advancement Device in OSA

Effect of inspiratory muscle training on pulmonary function, respiratory muscle strength, aerobic performance, sports skills, and quality of life in wheelchair rugby athletes

IntroductionInspiratory muscle training (IMT) has been used in sports to improve factors that can positively influence sports performance. However, the effects of IMT on athletes with disabilities are less well explored. This study aimed to investigate the effects of IMT on the pulmonary function, respiratory muscle strength, aerobic performance, sports skills, and quality of life (QoL) of wheelchair rugby (WCR) athletes. MethodsA pre–post-intervention case series study of IMT was carried out with six WCR athletes. Pulmonary function (spirometry), respiratory muscle strength (manovacuometry), aerobic (cardiopulmonary exercise test) and WCR skills (Beck Battery) performance and QoL (WHOQOL-DIS) were investigated. Participants were submitted to a six-week IMT with a linear load resistor involving 30 dynamic inspiratory maneuvers twice daily for five days/week. ResultsIncreases in maximum load (+14.6%; p = 0.03), maximum effort time (+19.4%; p = 0.03), absolute and percent of predicted maximal expiratory muscle pressure (both +13.2%; p = 0.04) and absolute and percent of predicted maximal inspiratory muscle pressure (both +28.6%; p = 0.04) were observed. No significant differences were observed for pulmonary function variables, WCR skills performance or QoL. ConclusionIMT seemed to be effective at improving the respiratory muscle strength and aerobic performance of WCR players but there was no impact on pulmonary function, sports skills or QoL.

Inspiratory Muscle Training in Phase 1 and 2 Postoperative Cardiac Rehabilitation Following Coronary Artery Bypass Graft Surgery: Systematic Review With Meta-Analysis.

This study aimed to determine the effects of inspiratory muscle training (IMT) on exercise capacity, respiratory muscle strength, length of hospital stay (LOS), and quality of life (QOL) following coronary artery bypass graft surgery. The search was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and the Cochrane Handbook and included the databases MEDLINE, EMBASE, CINAHL, Scopus, and CENTRAL. The review included randomized controlled trials utilizing IMT during phase 1 or 2 postoperative cardiac rehabilitation (PoCR) versus alternative treatment (active or passive control) in patients following coronary artery bypass graft surgery. Fifteen studies were included (11 phase 1 studies, 4 phase 2 studies) with no reported adverse events. In phase 1 PoCR, IMT reduced the LOS (-1.02days; 95% CI = -2.00 to -0.03) and increased exercise capacity (6-minute walk distance) (+75.46m; 95% CI = 52.34 to 98.57), and maximal inspiratory pressure (MIP) (10.46cm H2O; 95% CI = 2.83 to 18.10), but had no effect on maximal expiratory pressure. In phase 2 PoCR, IMT increased 6-minute walk distance (45.84m; 95% CI = 10.89 to 80.80), MIP (-23.19cm H2O; 95% CI = -31.31 to -15), maximal expiratory pressure (20.18cm H2O; 95% CI = 9.60 to 30.76), and QOL (-11.17; 95% CI = -17.98 to -4.36), with no effect on peak oxygen uptake. There was a high risk of bias for MIP (75% of the phase 1 studies) and 6MWT (1 of 4 phase 2 studies). The quality of the evidence ranged from very low to moderate. IMT significantly improves exercise capacity, respiratory muscle strength, LOS, and QOL in phase 1 and 2 PoCR. IMT may benefit patients during phase 1 and 2 of PoCR, considering the safety, low cost, and potential benefits.

Effect of different positive end expiratory pressure levels on optic nerve sheath diameter in patients with or without midline shift who are undergoing supratentorial craniotomy

PurposeIn general, high levels of PEEP application is avoided in patients undergoing craniotomy to prevent a rise in ICP. But that approach would increase the risk of secondary brain injury especially in hypoxemic patients. Because the optic nerve sheath is distensible, a rise in ICP is associated with an increase in the optic nerve sheath diameter (ONSD). The cutoff value for elevated ICP assessed by ONSD is between 5.6 and 6.3 mm. We aimed to evaluate the effect of different PEEP levels on ONSD and compare the effect of different PEEP levels in patients with and without intracranial midline shift.MethodsThis prospective observational study was performed in aged 18–70 years, ASA I–III, 80 patients who were undergoing supratentorial craniotomy. After the induction of general anesthesia, the ONSD’s were measured by the linear transducer from 3 mm below the globe at PEEP values of 0–5–10 cmH2O. The ONSD were compered between patients with (n = 7) and without midline shift (n = 73) at different PEEP values.ResultsThe increases in ONSD due to increase in PEEP level were determined (p < 0.001). No difference was found in the comparison of ONSD between patients with and without midline shift in different PEEP values (p = 0.329, 0.535, 0.410 respectively). But application of 10 cmH2O PEEP in patients with a midline shift increased the mean ONSD value to 5.73 mm. This value is roughly 0.1 mm higher than the lower limit of the ONSD cutoff value.ConclusionsThe ONSD in adults undergoing supratentorial tumor craniotomy, PEEP values up to 5 cmH2O, appears not to be associated with an ICP increase; however, the ONSD exceeded the cutoff for increased ICP when a PEEP of 10 cmH2O was applied in patients with midline shift.

Impact of pulmonary rehabilitation on patients with different chronic respiratory diseases during hospitalization.

The impact of pulmonary rehabilitation (PR) on patients with different chronic respiratory diseases (CRDs) during hospitalization has not been thoroughly evaluated before. The objectives of the current research were to assess the effect of comprehensive PR management on inpatients' self-management skills, exercise capacity, nutrition assessment and mental health issues and explore whether impacts of PR vary in different CRDs. This retrospective study analyzed the clinical data from 272 inpatients with CRDs receiving PR management during hospitalization between October 2020 and March 2022 in Beijing Chao-Yang Hospital. Significant improvements were found in the patients' ability of daily living (ADL), dyspnea (assessed by modified medical research council dyspnea scale (MMRC)), handgrip strength, maximal inspiratory and expiratory pressure, anxiety (using the 7-item generalized anxiety disorder scale (GAD-7)) and depression (the 9-item patient health questionnaire score (PHQ-9)). There was no significant change in nutrition assessment pre-post PR management during hospitalization. The subgroup analyses were conducted on hospitalized patients with chronic obstructive pulmonary disease (COPD), bronchiectasis, asthma, interstitial lung diseases (ILDs) and other CRDs (e.g., lung cancer, diaphragm hemiparesis, obesity, etc.). The results showed that ADL, MMRC score, MIP, MEP, PHQ-9 score improved in all subgroups with CRDs. Handgrip strength of left hand was increased in COPD inpatients and anxiety was improved in all subgroups except for ILDs. Comprehensive PR management was necessary and beneficial for patients with different CRDs during hospitalization.

Weaning strategies for children on home invasive mechanical ventilation.

Children who require home mechanical ventilation (HMV) with an artificial airway or invasive mechanical ventilation (HMV) have a possibility of successful weaning due to the potential of compensatory lung growth. Internationally accepted guidelines on how to wean from HMV in children is not available, we summarize the weaning strategies from the literature reviews combined with our 27-year experience in the Pediatric Home Respiratory Care program at the tertiary care center in Thailand. The readiness to wean is considered in patients with hemodynamic stability, having effective cough measured by maximal inspiratory pressure, requiring a fraction of inspired oxygen (FiO2) < 40%, positive end expiratory pressure <5 cmH2O, and acceptable arterial blood gases. The strategies of weaning is start weaning during the daytime while the child is awake and close monitoring is feasible. Disconnect time is gradually increased through naps and sleeping hours. Weaning from the conventional mechanical ventilator to Bilevel PAP or CPAP are optional. Factors affected the successful weaning are mainly the underlying diseases, complications, growth and development, caregivers, and resources. Weaning should be stopped during acute illness or increased work of breathing. The readiness for decannulation could be determined by using the speaking devices, tracheostomy capping, and measurement of end-expiratory pressure. Polysomnography and airway evaluation by bronchoscopy are recommended before decannulation.Weaning when the child is ready is crucial because living with HMV can be challenging and stressful. Failure to remove a tracheostomy when indicated can result in delayed speech, social problems as well as risk for infection.

Nusinersen in adults with type 3 spinal muscular atrophy: long-term outcomes on motor and respiratory function.

Nusinersen was approved for 5q spinal muscular atrophy (SMA), irrespective of age, SMA type or functional status. Nonetheless, long-term data on adults with milder phenotypes are scarce. We aimed to characterize evolution on motor and respiratory function in our cohort of adults with type 3 SMA. We conducted a longitudinal retrospective single-center study, including adults (≥18years) with type 3 SMA under nusinersen for > 22months. We reported on motor scores and spirometry parameters. Ten patients were included, with a median follow-up of 34months (range = 22-46). Four patients (40%) were walkers. None used non-invasive ventilation. In Revised Upper Limb Module (RULM) and Expanded Hammersmith Functional Motor Scale (HFMSE), difference of medians increased at 6, 22 and 46months comparing to baseline (-0.5 vs. + 1.5 vs. + 2.5 in RULM; + 4.0 vs. + 7.5 vs. + 6.0 in HFMSE). Two (50%) walkers presented a clinically meaningful improvement in 6-min walk distance. We did not report any clinically meaningful decrement in motor scores. Spirometry parameters showed an increasing difference of medians in maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) (-3 vs. + 13.4 vs. + 28.7 percentage points of predicted value for MIP; + 11.8 vs. + 13.1 vs. 13.3 percentage points of predicted value for MEP). Our cohort supports a sustained benefit of nusinersen in adults with type 3 SMA, in motor and respiratory function. Multicentric studies are still warranted.