Respiratory muscle training in children and adults with neuromuscular disease.
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ABSTRACT:
SUBMITTER: Silva IS
PROVIDER: S-EPMC6953358 | biostudies-literature |
REPOSITORIES: biostudies-literature
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| S-EPMC6486138 | biostudies-literature
Project description:Pompe disease is due to mutations in the gene encoding the lysosomal enzyme acid ?-glucosidase (GAA). Absence of functional GAA typically results in cardiorespiratory failure in the first year; reduced GAA activity is associated with progressive respiratory failure later in life. While skeletal muscle pathology contributes to respiratory insufficiency in Pompe disease, emerging evidence indicates that respiratory neuron dysfunction is also a significant part of dysfunction in motor units. Animal models show profound glycogen accumulation in spinal and medullary respiratory neurons and altered neural activity. Tissues from Pompe patients show central nervous system glycogen accumulation and motoneuron pathology. A neural mechanism raises considerations about the current clinical approach of enzyme replacement since the recombinant protein does not cross the blood-brain-barrier. Indeed, clinical data suggest that enzyme replacement therapy delays symptom progression, but many patients eventually require ventilatory assistance, especially during sleep. We propose that treatments which restore GAA activity to respiratory muscles, neurons and networks will be required to fully correct ventilatory insufficiency in Pompe disease.
| S-EPMC4083814 | biostudies-literature
Project description:BackgroundChildren with Intellectual disabilities who suffer from developmental coordination disorder represent insignificant physical fitness, strength, and balance. The prime objective of this research is to explore the impact of eight weeks of neuromuscular (combined physio-hemsball) training on postural control and balance of students with intellectual disabilities suffering from developmental coordination disorder.MethodsThe present study was a prospective randomized clinical trial with a pretest-posttest design. The statistical population consisted of boys with intellectual disabilities, suffering from developmental coordination disorder randomly divided into two groups: the experimental group (n = 15) and the control group (n = 15). informed consent was obtained from all participants' parents. Parents completed developmental coordination disorder questionnaires. Tests (Balance Error Scoring System, Y-Balance, timed Get Up & Go) were used to determine postural control, dynamic balance, and functional balance of subjects. The experimental group performed a combined physio-hemsball training for 8 weeks. Ethical considerations were observed according to the Helsinki Declaration and the CONSORT guidelines and regulations were followed to report this study.ResultsResults showed that combined physio-hemsball training for 8 weeks can greatly improve postural control and dynamic and postural balance among students with intellectual disabilities suffering from developmental coordination disorder.ConclusionsAccording to the results, instructors can use this type of training to improve postural control and balance in boys with intellectual disabilities enduring developmental coordination disorders.Trial registrationThis research was registered by the clinical trial centers of Iran (code IRCT20200125046254N1 , Date of registration: 24/04/2020).
| S-EPMC9250263 | biostudies-literature
Project description:ObjectiveTo investigate the efficacy of high-intensity respiratory muscle training (combined inspiratory and expiratory muscle training) in improving inspiratory and expiratory muscle strength, inspiratory muscle endurance, peak cough flow, dyspnea, fatigue, exercise capacity, and quality of life in this population.MethodsA randomized controlled trial, concealed allocation, blinded assessments, and intention-to-treat analysis will be carried out. Altogether, 34 individuals with PD (age ≥ 50 years old, with maximum inspiratory pressure (MIP) <80cmH2O or maximum expiratory pressure (MEP) <90cmH2O) will be recruited. Patients will be randomly assigned to either (1) high-intensity respiratory muscle training (experimental group, 60% of MIP and MEP) or (2) sham training (control group, 0cmH2O). Individuals will perform a home-based intervention, with indirect home supervision, consisting of two daily 20-min sessions (morning and afternoon), seven times a week, during eight weeks. Primary outcomes are MIP and MEP. Secondary outcomes are inspiratory muscle endurance, peak cough flow, dyspnea, fatigue, exercise capacity, and quality of life. The effects of the training will be analyzed from the collected data using intention-to-treat. Between-group differences will be measured using a two-way ANOVA with repeated measures (2*3), considering baseline, post-intervention, and 12-week follow-up.ImpactThe results of this trial will provide valuable new information on the efficacy of high-intensity respiratory muscle training in improving muscle strength, functional outcomes, and quality of life in individuals with PD. Performing combined inspiratory and expiratory muscle training using a single equipment is cheaper and feasible, takes less time and is easy to use. In addition, this intervention will be carried out in the home environment that increases accessibility, reduces time, and costs of transport, which increases the feasibility to reproduce their findings in clinical practice.Trial registrationNCT05608941. Registered on November 8, 2022.
| S-EPMC10490961 | biostudies-literature
Project description:BackgroundLittle is known about the response of the equine respiratory muscles to training.ObjectivesTo measure an index of inspiratory muscle strength (IMSi) before and after a period of conventional exercise training (phase 1) and inspiratory muscle training (IMT), comparing high-load (treatment) and low-load (control) groups (phase 2).Study designProspective randomised controlled trial.MethodsPhase 1: Twenty National Hunt Thoroughbred racehorses performed an inspiratory muscle strength test (IMST) twice on two occasions; when unfit at timepoint A (July), and when race fit at timepoint B (October). Phase 2: Thirty-five Thoroughbred racehorses at race fitness were randomly assigned into a high-load (treatment, n = 20) or low-load (control, n = 15) IMT group. The high-load group followed an IMT protocol that gradually increased the inspiratory pressure applied every 4 days. The low-load group underwent sham IMT with a low training load. The IMT was performed 5 days/week for 10 weeks. The IMST was performed twice on two occasions, timepoint B (October) and timepoint C (January). Conventional exercise training and racing continued during the study period. The peak IMSi values obtained from the different groups at timepoints A, B and C were compared using a Wilcoxon Signed Rank Test.ResultsPhase 1: There was a significant increase in IMSi from timepoint A: 22.5 cmH2 O (21-25) to timepoint B: 26 cmH2 O (24-30) (p = 0.015). Phase 2: From timepoint B to C there was a significant increase in IMSi for the high-load group 34 cmH2 O (28-36) (p = 0.001) but not the low-load group 26 cmH2 O (24-30) (p = 0.929). The peak IMSi at timepoint C was significantly higher for the high-load than low-load group (p = 0.019).Main limitationsSingle centre study with only National Hunt horses undergoing race-training included.ConclusionsIn horses undergoing race training there is a significant increase in IMSi in response to conventional exercise training and high-load IMT.
| S-EPMC10084158 | biostudies-literature
Project description:BACKGROUND:Adaptive balance control is often compromised in children with developmental coordination disorder (DCD). Neuromuscular training (NMT) is commonly used in clinical settings to improve neuromuscular control and hence balance performance in these children. However, its effectiveness has not been proven scientifically. This randomized controlled study aimed to explore the effectiveness of NMT for improving adaptive balance performance and the associated leg muscle activation times in children with DCD. METHODS:Eighty-eight children with DCD were randomly assigned to the NMT or control group (44 per group). The NMT group received two 40-minute NMT sessions/week for 3 months, whereas the control group received no intervention. The outcomes were measured at baseline and 3 and 6 months. The primary outcome was the sway energy score (SES) in both the toes-up and toes-down conditions as derived using the Adaptation Test (ADT). Secondary outcomes included the medial gastrocnemius, medial hamstring, tibialis anterior and rectus femoris muscle activation onset latencies during ADT, measured using surface electromyography and accelerometry. Data were analyzed using a repeated measures analysis of covariance based on the intention-to-treat principle. RESULTS:At 3 months, no significant within-group or between-group differences were noted in the SESs for either group. At 6 months, the toes-down SES decreased by 6.8% compared to the baseline value in exclusively the NMT group (P?=?.004). No significant time, group or group-by-time interaction effects were observed in any leg muscle activation outcomes. CONCLUSIONS:Short-term NMT failed to improve adaptive balance performance and leg muscle activation times in children with DCD. Further studies should explore the clinical applications of longer-term task-specific interventions intended to improve the adaptive balance performance of these children.
| S-EPMC6855479 | biostudies-literature
Project description:To address progressive respiratory muscle weakness in late-onset Pompe disease (LOPD), we developed a 12-week respiratory muscle training (RMT) program. In this exploratory, double-blind, randomized control trial, 22 adults with LOPD were randomized to RMT or sham-RMT. The primary outcome was maximum inspiratory pressure (MIP). Secondary and exploratory outcomes included maximum expiratory pressure (MEP), peak cough flow, diaphragm ultrasound, polysomnography, patient-reported outcomes, and measures of gross motor function. MIP increased 7.6 cmH2O (15.9) in the treatment group and 2.7 cmH2O (7.6) in the control group (P = 0.4670). MEP increased 14.0 cmH2O (25.9) in the treatment group and 0.0 cmH2O (12.0) in the control group (P = 0.1854). The only statistically significant differences in secondary/exploratory outcomes were improvements in time to climb 4 steps (P = 0.0346) and daytime sleepiness (P = 0.0160). The magnitude of changes in MIP and MEP in the treatment group were consistent with our pilot findings but did not achieve statistical significance in comparison to controls. Explanations for this include inadequate power and baseline differences in subject characteristics between groups. Additionally, control group subjects appeared to exhibit an active response to sham-RMT and therefore sham-RMT may not be an optimal control condition for RMT in LOPD.
| S-EPMC7680435 | biostudies-literature
Project description:ObjectiveTo examine the efficacy of combined inspiratory and expiratory respiratory muscle training (RMT) with respect to the swallowing function, pulmonary function, functional performance, and dysarthria in patients with stroke.DesignProspective, randomized controlled trial.SettingTertiary hospital.ParticipantsThe trial included 21 subjects (12 men, 9 women) aged 35 to 80 years presenting with 6 months history of unilateral stroke, respiratory muscle weakness (≥70% predicted maximal inspiratory pressure (MIP) and/or ≤70% maximal expiratory pressure (MEP)), dysphagia, or dysarthria. These subjects were randomly assigned to the control (n = 10, rehabilitation) and experimental (n = 11, rehabilitation with RMT) groups.InterventionInspiratory RMT starting from 30% to 60% of MIP and expiratory RMT starting from 15% to 75% of MEP for 5 days/week for 6 weeks.Main outcome measuresMIP, MEP, pulmonary function, peak cough flow, perception of dyspnea, Fatigue Assessment Scale, Modified Rankin Scale, Brunnstrom stage, Barthel index, Functional Oral Intake Scale (FOIS), and parameters of voice analysis.ResultsSignificant differences were observed between both groups in terms of MIP, forced vital capacity (FVC), and forced expiratory volume per second (FEV1) of the percentage predicted. Significant difference was found with respect to the change in fatigue, shimmer percent, amplitude perturbation quotient, and voice turbulence index (VTI) according to the acoustic analysis in the RMT group. The FEV1/FVC ratio was negatively correlated with jitter percent, relative average perturbation, pitch perturbation quotient, and VTI; the maximum mid-expiratory flow (MMEF) and MMEF% were also negatively correlated with VTI. Significant differences among participants of the same group were observed while comparing the Brunnstrom stage before and after training of the affected limbs and the Barthel scale and FOIS scores in both the groups.ConclusionsAltogether, 6-week combined inspiratory and expiratory RMT is feasible as adjuvant therapy for stroke patients to improve fatigue level, respiratory muscle strength, lung volume, respiratory flow, and dysarthria.Clinical trial registration number (Clinical Trial Identifier): NCT03491111.
| S-EPMC7478702 | biostudies-literature
Project description:Evidence from clinical trials and observational studies suggests that both progressive resistance training (PRT) and metformin delay a variety of age-related morbidities. Previously, we completed a clinical trial testing the effects of 14 weeks of PRT + metformin (metPRT) compared to PRT with placebo (plaPRT) on muscle hypertrophy in older adults. We found that metformin blunted PRT-induced muscle hypertrophic response. To understand potential mechanisms underlying the inhibitory effect of metformin on PRT, we analyzed the muscle transcriptome in 23 metPRT and 24 plaPRT participants. PRT significantly increased expression of genes involved in extracellular matrix remodeling pathways, and downregulated RNA processing pathways in both groups, however, metformin blunted the number of differentially expressed genes within these pathways compared to plaPRT. Pathway analysis showed that genes unique to metPRT modulated aging-relevant pathways, such as cellular senescence and autophagy. Differentially expressed genes from baseline biopsies in older adults compared to resting muscle from young volunteers were reduced following PRT in plaPRT and were further reduced in metPRT. We suggest that although metformin may blunt pathways induced by PRT to promote muscle hypertrophy, adjunctive metformin during PRT may have beneficial effects on aging-associated pathways in muscle from older adults.
2020-09-08 | GSE157585 | GEO