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Neuromuscular diseases ventilation

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https://www.readbyqxmd.com/read/28503469/motor-neuron-disease-presenting-with-acute-respiratory-failure-a-case-study
#1
Hyeonjun Oh, Seong Woong Kang, Won Ah Choi, Jang Woo Lee, Miri Suh, Eun Young Kim
Motor neuron diseases (MNDs) refer to a heterogeneous group of progressive neurologic disorders caused by degeneration of motor neurons. The diseases affect either the upper motor neurons, lower motor neurons, or both, and are characterized by weakness, atrophy, fasciculation, spasticity, and respiratory failure. We report a case of a 61-year-old male patient with no past history of cardiovascular or pulmonary disease, who presented with only dyspnea, and no indication of any other symptom such as muscle weakness, atrophy, or bulbar dysfunction...
April 2017: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/28500219/mechanisms-of-enhanced-phrenic-long-term-facilitation-in-sod1-g93a-rats
#2
Nicole L Nichols, Irawan Satriotomo, Latoya L Allen, Ashley M Grebe, Gordon S Mitchell
Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease, causing muscle paralysis and death from respiratory failure. Effective means to preserve/restore ventilation are necessary to increase the quality and duration of life in ALS patients. At disease end-stage in a rat ALS model (SOD1(G93A)), acute intermittent hypoxia (AIH) restores phrenic nerve activity to normal levels via enhanced phrenic long-term facilitation (pLTF). Mechanisms enhancing pLTF in end-stage SOD1(G93A) rats are not known...
May 12, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28462858/neuromuscular-disorders-and-chronic-ventilation
#3
REVIEW
Stamatia Alexiou, Joseph Piccione
Morbidity and mortality have decreased in patients with neuromuscular disease due to implementation of therapies to augment cough and improve ventilation. Infants with progressive neuromuscular disease will eventually develop respiratory complications as a result of muscle weakness and their inability to compensate during periods of increased respiratory loads. The finding of nocturnal hypercapnia is often the trigger for initiating non-invasive ventilation and studies have shown that its use not only may improve sleep-disordered breathing, but also that it may have an effect on daytime function, symptoms related to hypercapnia, and partial pressure of CO2...
April 24, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28461884/risk-of-unsuccessful-noninvasive-ventilation-for-acute-respiratory-failure-in-heterogeneous-neuromuscular-diseases-a-retrospective-study
#4
Hiroshi Kataoka, Hitoki Nanaura, Kaoru Kinugawa, Yuto Uchihara, Hiroya Ohara, Nobuyuki Eura, Ryogo Syobatake, Nobuhiro Sawa, Kiriyama Takao, Kazuma Sugie, Satoshi Ueno
If invasive ventilation can be avoided by performing noninvasive mechanical ventilation (NIV) in patients with acute respiratory failure (ARF), the disease can be effectively managed. It is important to clarify the characteristics of patients with neuromuscular diseases in whom initial NIV is likely to be unsuccessful. We studied 27 patients in stable neuromuscular condition who initially received NIV to manage fatal ARF to identify differences in factors immediately before the onset of ARF among patients who receive continuous NIV support, patients who are switched from NIV to invasive ventilation, and patients in whom NIV is discontinued...
February 20, 2017: Neurology International
https://www.readbyqxmd.com/read/28459325/ajrccm-100-year-anniversary-homeward-bound-a-centenary-of-home-mechanical-ventilation
#5
Matthew Hind, Michael I Polkey, Anita K Simonds
The evolution of home mechanical ventilation is an intertwined chronicle of negative and positive pressure modes and their role in managing ventilatory failure in neuromuscular diseases and other chronic disorders. The uptake of noninvasive positive pressure ventilation has resulted in widespread growth in home ventilation internationally and fewer patients being ventilated invasively. As with many applications of domiciliary medical technology, home ventilatory support has either led or run in parallel with acute hospital applications and has been influenced by medical and societal shifts in the approach to chronic care, the creation of community support teams, a preference of recipients to be treated at home, and economic imperatives...
May 1, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28448298/anesthetic-consideration-for-neuromuscular-diseases
#6
Jeffery A Katz, Glenn S Murphy
PURPOSE OF REVIEW: The aim of this review is to examine data relating to perioperative management of the patient with neuromuscular disorders RECENT FINDINGS: Patients with pre-existing neuromuscular disorders are at risk for a number of postoperative complications that are related to anesthetic drugs that are administered intraoperatively. Careful preoperative assessment is necessary to reduce morbidity and mortality. In particular, the risk of postoperative respiratory failure and need for long-term ventilation should be reviewed with patients...
June 2017: Current Opinion in Anaesthesiology
https://www.readbyqxmd.com/read/28448001/repeated-measurement-of-respiratory-muscle-activity-and-ventilation-in-mouse-models-of-neuromuscular-disease
#7
Victoria N Jensen, Shannon H Romer, Sarah M Turner, Steven A Crone
Accessory respiratory muscles help to maintain ventilation when diaphragm function is impaired. The following protocol describes a method for repeated measurements over weeks or months of accessory respiratory muscle activity while simultaneously measuring ventilation in a non-anesthetized, freely behaving mouse. The technique includes the surgical implantation of a radio transmitter and the insertion of electrode leads into the scalene and trapezius muscles to measure the electromyogram activity of these inspiratory muscles...
April 17, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28446219/the-burden-epidemiology-costs-and-treatment-for-duchenne-muscular-dystrophy-an-evidence-review
#8
REVIEW
S Ryder, R M Leadley, N Armstrong, M Westwood, S de Kock, T Butt, M Jain, J Kleijnen
BACKGROUND: Duchenne Muscular Dystrophy (DMD) is a rapidly progressive, lethal neuromuscular disorder, present from birth, which occurs almost exclusively in males. We have reviewed contemporary evidence of burden, epidemiology, illness costs and treatment patterns of DMD. This systematic review adhered to published methods with information also sought from the web and contacting registries. Searches were carried out from 2005 to June 2015. The population of interest was individuals with clearly defined DMD or their carers...
April 26, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28437378/risk-factors-for-pediatric-extubation-failure-the-importance-of-respiratory-muscle-strength
#9
Robinder G Khemani, Tro Sekayan, Justin Hotz, Rutger C Flink, Gerrard F Rafferty, Narayan Iyer, Christopher J L Newth
OBJECTIVE: Respiratory muscle weakness frequently develops during mechanical ventilation, although in children there are limited data about its prevalence and whether it is associated with extubation outcomes. We sought to identify risk factors for pediatric extubation failure, with specific attention to respiratory muscle strength. DESIGN: Secondary analysis of prospectively collected data. SETTING: Tertiary care PICU. PATIENTS: Four hundred nine mechanically ventilated children...
April 21, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28435672/tele-medicine-in-respiratory-diseases
#10
REVIEW
Nicolino Ambrosino, Dewi Nurul Makhabah, Yusup Subagio Sutanto
Information and Communication Technologies applied to health care and advances in sensor and data transmission technology allowed tele-medicine based programs of care also for patients with respiratory diseases. Different sensors, transmission devices and interventions are used in tele-medicine for some indications. Patients suffering from Chronic Obstructive Pulmonary Disease, asthma, neuromuscular diseases, ventilator assisted individuals and those undergoing pulmonary rehabilitation programs may benefit from this approach...
2017: Multidisciplinary Respiratory Medicine
https://www.readbyqxmd.com/read/28410001/respiratory-pattern-and-tidal-volumes-differ-for-pressure-support-and-volume-assured-pressure-support-in-amyotrophic-lateral-sclerosis
#11
Trevor T Nicholson, Sean B Smith, Teepu Siddique, Robert Sufit, Senda Ajroud-Driss, John M Coleman, Lisa F Wolfe
RATIONALE: Amyotrophic Lateral Sclerosis is a progressive neuromuscular disease resulting in respiratory failure and death. Use of non-invasive ventilation (NIV) improves survival. However, use of Volume-Assured Pressure Support (VAPS) has not been extensively studied in ALS. OBJECTIVES: To explore the clinical utility of a detailed evaluation of device-recorded NIV data in the management of chronic respiratory failure in ALS. To determine if there are differences in efficacy between patients using VAPS or PS...
April 14, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28382599/diagnostic-accuracy-of-quantitative-neuromuscular-ultrasound-for-the-diagnosis-of-intensive-care-unit-acquired-weakness-a-cross-sectional-observational-study
#12
Esther Witteveen, Juultje Sommers, Luuk Wieske, Jonne Doorduin, Nens van Alfen, Marcus J Schultz, Ivo N van Schaik, Janneke Horn, Camiel Verhamme
BACKGROUND: Neuromuscular ultrasound is a noninvasive investigation, which can be easily performed at the bedside on the ICU. A reduction in muscle thickness and increase in echo intensity over time have been described in ICU patients, but the relation to ICU-acquired weakness (ICU-AW) is unknown. We hypothesized that quantitative assessment of muscle and nerve parameters with ultrasound can differentiate between patients with and without ICU-AW. The aim of this cross-sectional study was to investigate the diagnostic accuracy of neuromuscular ultrasound for diagnosing ICU-AW...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28372949/sleep-disordered-breathing-in-neuromuscular-disease-diagnostic-and-therapeutic-challenges
#13
REVIEW
Loutfi S Aboussouan, Eduardo Mireles-Cabodevila
Normal sleep related rapid eye movement sleep atonia, reduced lung volumes, reduced chemosensitivity, and impaired airway dilator activity, become significant vulnerabilities in the setting of neuromuscular disease. In that context, the compounding effects of respiratory muscle weakness and disease-specific features that promote upper airway collapse or cause dilated cardiomyopathy, contribute to various sleep-disordered breathing events. The reduction in lung volumes with neuromuscular disease is further compromised by sleep and the supine position, exaggerating the tendency for upper airway collapse and desaturation with sleep-disordered breathing events...
March 31, 2017: Chest
https://www.readbyqxmd.com/read/28357836/safety-of-hyperbaric-oxygen-therapy-in-mechanically-ventilated-patients
#14
Jacques Bessereau, Jérôme Aboab, Thomas Hullin, Anne Huon-Bessereau, Jean-Luc Bourgeois, Pierre-Marie Brun, Sylvie Chevret, Djillali Annane
BACKGROUND: To evaluate the epidemiology of patients who require mechanical ventilation during hyperbaric oxygen therapy. MATERIALS AND METHODS: One-hundred-fifty patients who required mechanical ventilation during hyperbaric oxygen therapy were prospectively studied during a 6-year period in a French university hyperbaric centre. We analysed the indication of hyperbaric oxygen therapy, agent used for sedation, presence of a chest tube, need for vasopressor agents and tolerance and appearance of side effects...
2017: International Maritime Health
https://www.readbyqxmd.com/read/28338488/respiratory-complications-management-and-treatments-for-neuromuscular-disease-in-children
#15
MyMy C Buu
PURPOSE OF REVIEW: To summarize current literature describing the respiratory complications of neuromuscular disease (NMD) and the effect of respiratory interventions and to explore new gene therapies for patients with NMD. RECENT FINDINGS: Measurements of respiratory function focus on vital capacity and maximal inspiratory and expiratory pressure and show decline over time. Management of respiratory complications includes lung volume recruitment, mechanical insufflation-exsufflation, chest physiotherapy and assisted ventilation...
June 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28256693/-communication-and-language-problems-in-children-with-nemaline-myopathy
#16
J F Cervera-Merida, I Villa-Garcia, A Ygual-Fernandez
INTRODUCTION: Nemaline myopathy is a rare disease with an incidence of 1 in every 50,000 live births. It is the most prevalent of the congenital myopathies, a heterogeneous set of neuromuscular disorders present at birth or manifesting at a very early age, which affect the skeletal muscles and give rise to weakness, hypotonia and psychomotor retardation, although cognitive development remains normal. AIM: To review the studies conducted to date on the communication difficulties and dysphagia of children with nemaline myopathy and their possible management based on speech therapy...
February 24, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28241090/plasma-exchange-for-guillain-barr%C3%A3-syndrome
#17
REVIEW
Sylvie Chevret, Richard Ac Hughes, Djillali Annane
BACKGROUND: Guillain-Barré syndrome (GBS) is an acute paralysing disease caused by peripheral nerve inflammation. This is an update of a review first published in 2001 and last updated in 2012. OBJECTIVES: To assess the effects of plasma exchange for treating GBS. SEARCH METHODS: On 18 January 2016 we searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, and Embase. We also searched clinical trials registries. SELECTION CRITERIA: Randomised and quasi-randomised trials of plasma exchange versus sham exchange or supportive treatment, or comparing different regimens or techniques of plasma exchange...
February 27, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28159100/social-and-economic-impacts-of-managing-sleep-hypoventilation-syndromes
#18
REVIEW
Dianne M Augelli, Ana C Krieger
Hypoventilation during sleep is often an early indicator of the development of respiratory failure. Alterations in ventilation are more pronounced during sleep and often present before the onset of daytime symptoms. This article discusses the most common sleep-related hypoventilatory disorders and recommended treatment approaches for obesity hypoventilation, chronic obstructive pulmonary disease, and neuromuscular disorders. Accurate diagnosis and appropriate treatment is of paramount importance because of the impact on individual health outcomes and overall cost of health care delivery...
March 2017: Sleep Medicine Clinics
https://www.readbyqxmd.com/read/28153460/prognostic-value-of-nocturnal-hypoventilation-in-neuromuscular-patients
#19
David Orlikowski, Helene Prigent, Maria-Antonia Quera Salva, Nicholas Heming, Cendrine Chaffaut, Sylvie Chevret, Djillali Annane, Frederic Lofaso, Adam Ogna
In neuromuscular disease (NMD) patients, current guidelines recommend the initiation of home mechanical ventilation (HMV) in case of daytime hypercapnia or nocturnal desaturation as an indirect sign of hypoventilation. Transcutaneous capno-oximetry (TcCO2) enables the direct assessment of nocturnal hypercapnia; however the best cut-off value remains to be defined. We aimed to compare the prognostic value of several published definitions of nocturnal hypercapnia in a cohort of NMD patients. All consecutive TcCO2 recordings performed between 2010 and 2014 in unventilated adult NMD patients in a tertiary reference centre were retrospectively collected...
December 21, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28151766/the-use-of-sugammadex-in-a-patient-with-guillain-barre-syndrome-a-case-report
#20
Büşra Tezcan, Demet Bölükbaşi, Dilek Kazanci, Sema Turan, Gülseren Suer Kaya, Ayşegül Özgök
Sugammadex encapsulates and inactivates rocuronium and vecuronium. It is used to reverse neuromuscular blockade from these nondepolarizing agents. The safety of sugammadex in patients with neuromuscular disease has not been established. Guillain-Barre Syndrome (GBS) is a neuromuscular disease characterized by acute inflammatory polyneuropathy. Patients with GBS may exhibit autonomic dysfunction, chronic pain, abnormal reactions to neuromuscular blocking agents, and may require postoperative mechanical ventilation...
April 15, 2017: A & A Case Reports
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