Diaphragm pacing and motor neurone disease: lessons for all?
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ABSTRACT:
SUBMITTER: Elliott MW
PROVIDER: S-EPMC5005125 | biostudies-other | 2015 Oct
REPOSITORIES: biostudies-other
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Diaphragm pacing and motor neurone disease: lessons for all?
ERJ open research 20151001 2
<b>Respiratory physicians and neurologists should be cognisant of the respiratory complications of motor neurone disease</b> http://ow.ly/TIxe5. ...[more]
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Project description:PurposePatients with respiratory failure associated with neurological dysfunction often require mechanical ventilator support, which poses increased economic burden and ventilator-associated complications. A diaphragm pacing system (DPS) is an implanted device that provides respiratory support for such patients. In this systematic review, we reviewed the literature to assess the safety and efficacy of DPS for patients with respiratory failure resulting from amyotrophic lateral sclerosis (ALS) or cervical spinal cord injuries.Materials and methodsThe following databases were searched from July 10 to July 30, 2018: MEDLINE, EMBASE, Cochran library, KoreaMed, Research Information Sharing Service, Korean studies Information Service System, Korea Institute of Science and Technology Information, and Korean Medical database. The abstracts and full texts of the searched articles were reviewed by two reviewers.ResultsThe search keywords generated 197 articles: two randomized controlled trials, two case-control studies, and one case report involving patients with ALS; one cohort study, one case-control study, and two case reports involving patients with cervical spine injury; and one case report involving patients with both conditions were included. The primary outcome was safety profile (complications and adverse event) and efficacy (overall survival and sleep improvement). Complications and adverse events were more common in patients with ALS and spinal cord injury receiving DPS than in controls. Efficacy outcomes were inconsistent across ALS studies.ConclusionBased on safety and efficacy results, we do not support using DPS to manage respiratory failure in patients with ALS or cervical spine injury.
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Project description:Background and purposeCervical spinal cord injury (CSCI) can cause severe respiratory impairment. Although mechanical ventilation (MV) is a lifesaving standard of care for these patients, it is associated with diaphragm atrophy and dysfunction. Diaphragm pacing (DP) is a strategy now used acutely to promote MV weaning and to combat the associated negative effects. Initial reports indicate that DP also may promote neuromuscular plasticity and lead to improvements in spontaneous diaphragm activation and respiratory function. These outcomes suggest the need for reevaluation of respiratory rehabilitation for patients with CSCI using DP and consideration of new rehabilitation models for these patients and their unique care needs.Summary of key pointsThis article discusses the rationale for consideration of DP as a rehabilitative strategy, particularly when used in combination with established respiratory interventions. In addition, a model of respiratory rehabilitation and recovery (RRR) is presented, providing a framework for rehabilitation and consideration of DP as an adjuvant rehabilitation approach. The model promotes goals such as respiratory recovery and independence, and lifelong respiratory health, via interdisciplinary care, respiratory training, quantitative measurement, and use of adjuvant strategies such as DP. Application of the model is demonstrated through a description of an inpatient rehabilitation program that applies model components to patients with CSCI who require DP.Recommendations for clinical practiceAs DP use increases for patients with acute CSCI, so does the need and opportunity to advance rehabilitation approaches for these patients. This perspective article is a critical step in addressing this need and motivating the advancement of rehabilitation strategies for CSCI patients. (See Video Abstract, Supplemental Digital Content, available at: http://links.lww.com/JNPT/A348).
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Project description:RationaleTraumatic cervical spinal cord injury (SCI) is a devastating condition leading to respiratory failure that requires permanent mechanical ventilation, which is the main driver of increased medical costs. There is a great demand for establishing therapeutic interventions to treat respiratory dysfunction following severe cervical SCI.Patient concerns and diagnosisWe present a 24-year-old man who sustained a cervical displaced C2-C3 fracture with SCI due to a traffic accident. As the patient presented with tetraplegia and difficulty in spontaneous breathing following injury, he was immediately intubated and placed on a ventilator with cervical external fixation by halo orthosis. The patient then underwent open reduction and posterior fusion of the cervical spine 3 weeks after injury. Although the patient showed significant motor recovery of the upper and lower limbs over time, only a slight improvement in lung capacity was observed.Interventions and outcomesAt 1.5 years after injury, a diaphragmatic pacing stimulator was surgically implanted to support the patient's respiratory function. The mechanical ventilator support was successfully withdrawn from the patient 14 weeks after implantation. We observed that both the vital capacity and tidal volume of the patient were significantly promoted following implantation. The patient finally returned to daily life without any mechanical support.LessonsThe findings of this report suggest that diaphragmatic pacing implantation could be a promising treatment for improving respiratory function after severe cervical SCI. To our knowledge, this is the first SCI patient treated with a diaphragm pacing implantation covered by official medical insurance in Japan.
| S-EPMC9239610 | biostudies-literature
Project description:BackgroundMechanical ventilation (MV) is a life-saving technology that restores or assists breathing. Like any treatment, MV has side effects. In some patients it can cause diaphragmatic atrophy, injury, and dysfunction (ventilator-induced diaphragmatic dysfunction, VIDD). Accumulating evidence suggests that VIDD makes weaning from MV difficult, which involves increased morbidity and mortality.Methods and analysisThis paper describes the protocol of a randomized, controlled, open-label, multicenter trial that is designed to investigate the safety and effectiveness of a novel therapy, temporary transvenous diaphragm pacing (TTVDP), to improve weaning from MV in up to 88 mechanically ventilated adult patients who have failed at least two spontaneous breathing trials over at least 7 days. Patients will be randomized (1:1) to TTVDP (treatment) or standard of care (control) groups. The primary efficacy endpoint is time to successful extubation with no reintubation within 48 h. Secondary endpoints include maximal inspiratory pressure and ultrasound-measured changes in diaphragm thickness and diaphragm thickening fraction over time. In addition, observational data will be collected and analyzed, including 30-day mortality and time to discharge from the intensive care unit and from the hospital. The hypothesis to be tested postulates that more TTVDP patients than control patients will be successfully weaned from MV within the 30 days following randomization.DiscussionThis study is the first large-scale clinical trial of a novel technology (TTVDP) aimed at accelerating difficult weaning from MV. The technology tested provides the first therapy directed specifically at VIDD, an important cause of delayed weaning from MV. Its results will help delineate the place of this therapeutic approach in clinical practice and help design future studies aimed at defining the indications and benefits of TTVDP.Trial registrationClinicalTrials.gov, NCT03096639 . Registered on 30 March 2017.
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Project description:RationaleHypercapnic respiratory failure because of inspiratory muscle weakness is the most important cause of death in chronic obstructive pulmonary disease (COPD). However, the pathophysiology of failure of the diaphragm to generate force in COPD is in part unclear.ObjectivesThe present study investigated contractile function and myosin heavy chain content of diaphragm muscle single fibers from patients with COPD.MethodsSkinned muscle fibers were isolated from muscle biopsies from the diaphragm of eight patients with mild to moderate COPD and five patients without COPD (mean FEV(1) % predicted, 70 and 100%, respectively). Contractile function of single fibers was assessed, and afterwards, myosin heavy chain content was determined in these fibers. In diaphragm muscle homogenates, the level of ubiquitin-protein conjugation was determined.ResultsDiaphragm muscle fibers from patients with COPD showed reduced force generation per cross-sectional area, and reduced myosin heavy chain content per half sarcomere. In addition, these fibers had decreased Ca2+ sensitivity of force generation, and slower cross-bridge cycling kinetics. Our observations were present in fibers expressing slow and 2A isoforms of myosin heavy chain. Ubiquitin-protein conjugation was increased in diaphragm muscle homogenates of patients with mild to moderate COPD.ConclusionsEarly in the development of COPD, diaphragm fiber contractile function is impaired. Our data suggest that enhanced diaphragm protein degradation through the ubiquitin-proteasome pathway plays a role in loss of contractile protein and, consequently, failure of the diaphragm to generate force.
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Project description:The diaphragm muscle is essential for breathing in mammals. Its asymmetric elevation during contraction correlates with morphological features suggestive of inherent left-right (L/R) asymmetry. Whether this asymmetry is due to L versus R differences in the muscle or in the phrenic nerve activity is unknown. Here, we have combined the analysis of genetically modified mouse models with transcriptomic analysis to show that both the diaphragm muscle and phrenic nerves have asymmetries, which can be established independently of each other during early embryogenesis in pathway instructed by Nodal, a morphogen that also conveys asymmetry in other organs. We further found that phrenic motoneurons receive an early L/R genetic imprint, with L versus R differences both in Slit/Robo signaling and MMP2 activity and in the contribution of both pathways to establish phrenic nerve asymmetry. Our study therefore demonstrates L-R imprinting of spinal motoneurons and describes how L/R modulation of axon guidance signaling helps to match neural circuit formation to organ asymmetry.
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Project description:Rostro-caudal patterning of vertebrates depends on the temporally progressive activation of HOX genes within axial stem cells that fuel axial embryo elongation. Whether HOX genes sequential activation, the “HOX clock”, is paced by intrinsic chromatin-based timing mechanisms or by temporal changes in extrinsic cues remains unclear. Here, we studied HOX clock pacing in human pluripotent stem cells differentiating into spinal cord motor neuron subtypes which are progenies of axial progenitors. We show that the progressive activation of caudal HOX genes is controlled by a dynamic increase in FGF signaling. Blocking FGF pathway stalled induction of HOX genes, while precocious increase in FGF alone, or with GDF11 ligand, accelerated the HOX clock. Cells differentiated under accelerated HOX induction generated appropriate posterior motor neuron subtypes found along the human embryonic spinal cord. The HOX clock is thus dynamically paced by exposure parameters to secreted cues. Its manipulation by extrinsic factors alleviates temporal requirements to provide unprecedented synchronized access to human cells of multiple, defined, rostro-caudal identities for basic and translational applications.
2021-03-02 | GSE153519 | GEO
Project description:Small bowel diaphragm disease is a rare condition usually associated with the prolonged use of non-steroidal anti-inflammatory drugs (NSAID) and that can be mistaken and treated as other pathologies. We describe a case of a 64-year-old man with a prolonged course of pain and subacute bowel obstructions, without any history of NSAID usage, found to have a multiple diaphragmatic stricture in the small bowel.
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Project description: Not available
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Project description:Purpose: This article describes the content of and delivery methods for motor learning (ML) education and the attitudes and beliefs of instructors with regard to how ML is taught in Canadian physical therapy (PT) programmes. Method: A qualitative descriptive design was employed, using an online questionnaire and semi-structured telephone interviews. A descriptive content analysis was used to develop codes and themes. An online search of PT programme Web sites was conducted to supplement missing data and collect information from schools that did not participate in the online questionnaire or interview. Results: Eight individuals representing seven schools completed the questionnaire; six of the eight also completed the interview. Responses conveyed the fact that ML content was fairly consistent across schools and was predominantly situated in the neurological curriculum. Schools differed in the delivery methods used for clinical application of ML content. Respondents believed that ML underlies PT practice and should be integrated throughout the programme. Conclusion: Current instruction may deliver adequate ML content but may not provide optimal opportunities to apply ML principles in a clinical context. Continuing education emerged as one suggestion for remediating clinicians' knowledge-practice gap and facilitating student learning on placement. Only half the eligible PT schools participated, and all were English-language programmes; thus, the findings may not be generalizable to all Canadian programmes. Future work should explore how ML can be integrated into the PT curriculum to promote the application of ML principles across different fields. Students' perspectives on their understanding of ML and ML principles and self-efficacy for entry to practice should also be explored.
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