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Neuromuscular diseases ventilation

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https://www.readbyqxmd.com/read/29157220/combined-effects-of-leaks-respiratory-system-properties-and-upper-airway-patency-on-the-performance-of-home-ventilators-a-bench-study
#1
Kaixian Zhu, Claudio Rabec, Jésus Gonzalez-Bermejo, Sébastien Hardy, Sami Aouf, Pierre Escourrou, Gabriel Roisman
BACKGROUND: Combined effects of leaks, mechanical property of respiratory system and upper airway (UA) patency on patient-ventilator synchrony (PVA) and the level of clinically "tolerable" leaks are not well established in home ventilators. METHODS: We comparatively assessed on a bench model, the highest leak level tolerated without inducing significant asynchrony ("critical leak") in three home ventilators (Astral 150, Trilogy 100 and Vivo 60; noted as A150, T100 and V60 respectively) subjected to three simulated diseased respiratory conditions: chronic obstructive pulmonary disease (COPD), obesity hypoventilation (OHS) and neuromuscular disorders (NMD), with both open and closed UA...
November 21, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29125503/patient-preferences-for-treatments-of%C3%A2-neuromuscular-diseases-a-systematic-literature-review
#2
Erik Landfeldt, Josefin Edström, Peter Lindgren, Hanns Lochmüller
BACKGROUND: Treatment decisions of neuromuscular diseases involve weighing clinical benefits and risks, as well as impact on patient social life, work status, other activities of daily living, and health-related quality of life. OBJECTIVE: To conduct a systemic literature review of patient preferences for treatments of neuromuscular diseases. METHODS: We searched Embase, Web of Science, and PubMed for full-text articles reporting results from studies of patient preferences for treatments of neuromuscular diseases...
November 8, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/29095316/genome-wide-association-study-for-genetic-variants-related-with-maximal-voluntary-ventilation-reveals-two-novel-genomic-signals-associated-with-lung-function
#3
Yujin Suh, Chaeyoung Lee
Genome-wide association studies (GWAS) for spirometry parameters have been limited to forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), and their ratio. This study examined to identify genetic variants associated with maximal voluntary ventilation (MVV), an important spirometry parameter presenting inspiratory muscle strength.A total of 8842 Korean subjects participated in the Korean Association REsource Consortium were used to identify nucleotide variants associated with MVV and other spirometry parameters through a GWAS...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29091570/nusinersen-versus-sham-control-in-infantile-onset-spinal-muscular-atrophy
#4
RANDOMIZED CONTROLLED TRIAL
Richard S Finkel, Eugenio Mercuri, Basil T Darras, Anne M Connolly, Nancy L Kuntz, Janbernd Kirschner, Claudia A Chiriboga, Kayoko Saito, Laurent Servais, Eduardo Tizzano, Haluk Topaloglu, Már Tulinius, Jacqueline Montes, Allan M Glanzman, Kathie Bishop, Z John Zhong, Sarah Gheuens, C Frank Bennett, Eugene Schneider, Wildon Farwell, Darryl C De Vivo
BACKGROUND: Spinal muscular atrophy is an autosomal recessive neuromuscular disorder that is caused by an insufficient level of survival motor neuron (SMN) protein. Nusinersen is an antisense oligonucleotide drug that modifies pre-messenger RNA splicing of the SMN2 gene and thus promotes increased production of full-length SMN protein. METHODS: We conducted a randomized, double-blind, sham-controlled, phase 3 efficacy and safety trial of nusinersen in infants with spinal muscular atrophy...
November 2, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29091557/single-dose-gene-replacement-therapy-for-spinal-muscular-atrophy
#5
Jerry R Mendell, Samiah Al-Zaidy, Richard Shell, W Dave Arnold, Louise R Rodino-Klapac, Thomas W Prior, Linda Lowes, Lindsay Alfano, Katherine Berry, Kathleen Church, John T Kissel, Sukumar Nagendran, James L'Italien, Douglas M Sproule, Courtney Wells, Jessica A Cardenas, Marjet D Heitzer, Allan Kaspar, Sarah Corcoran, Lyndsey Braun, Shibi Likhite, Carlos Miranda, Kathrin Meyer, K D Foust, Arthur H M Burghes, Brian K Kaspar
BACKGROUND: Spinal muscular atrophy type 1 (SMA1) is a progressive, monogenic motor neuron disease with an onset during infancy that results in failure to achieve motor milestones and in death or the need for mechanical ventilation by 2 years of age. We studied functional replacement of the mutated gene encoding survival motor neuron 1 (SMN1) in this disease. METHODS: Fifteen patients with SMA1 received a single dose of intravenous adeno-associated virus serotype 9 carrying SMN complementary DNA encoding the missing SMN protein...
November 2, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29064259/the-cardiovascular-implications-of-sedatives-in-the-cardiac-intensive-care-unit
#6
Sammy Zakaria, Helaine J Kwong, Jonathan E Sevransky, Marlene S Williams, Nisha Chandra-Strobos
Patients admitted to the cardiac intensive care unit frequently develop multi-organ system dysfunction associated with their cardiac disease. In many cases, invasive mechanical ventilation is required, which often necessitates sedation for patient-ventilator synchrony, reduction of work of breathing, and patient comfort. In this paper, we describe the use of common sedatives available in the endotracheally intubated critically ill patient and emphasize the clinical and cardiovascular effects. We review γ-aminobutyric acid agonists such as etomidate, benzodiazepines, and propofol, the centrally acting α2-agonist dexmedetomidine, and the N-methyl-D-aspartate receptor antagonist ketamine...
February 1, 2017: European Heart Journal. Acute Cardiovascular Care
https://www.readbyqxmd.com/read/28992936/-short-term-exercise-based-cardiac-rehabilitation-induced-changes-in-cardiorespiratory-mechanical-and-neuromuscular-responses-to-progressive-exercise-testing
#7
P-M Leprêtre, M Ghannem, S Delanaud, N Jaunet, L Gaillard, A Barnabé, T Porcher, T Weissland
Previous studies showed that changes in peak of oxygen uptake value (VO2peak) with training were poorly related to changes in Maximal Tolerated Power output (MTP) among patients with cardiovascular disease. This result could be due to a difference between cardiopulmonary adaptation to training and the skeletal muscle conditioning. OBJECTIVE: The aim of the study was to compare the responses to exercise training of electromyographic activities of vastus lateralis (rms-EMG) and respiratory parameters...
November 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/28985727/respiratory-mechanics-in-infants-with-severe-bronchiolitis-on-controlled-mechanical-ventilation
#8
Pablo Cruces, Sebastián González-Dambrauskas, Julio Quilodrán, Jorge Valenzuela, Javier Martínez, Natalia Rivero, Pablo Arias, Franco Díaz
BACKGROUND: Analysis of respiratory mechanics during mechanical ventilation (MV) is able to estimate resistive, elastic and inertial components of the working pressure of the respiratory system. Our aim was to discriminate the components of the working pressure of the respiratory system in infants on MV with severe bronchiolitis admitted to two PICU's. METHODS: Infants younger than 1 year old with acute respiratory failure caused by severe bronchiolitis underwent neuromuscular blockade, tracheal intubation and volume controlled MV...
October 6, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28983937/factors-associated-with-change-in-health-related-quality-of-life-among-individuals-treated-with-long-term-mechanical-ventilation-a-six-year-follow-up-study
#9
Heidi Markussen, Sverre Lehmann, Roy M Nilsen, Gerd K Natvig
AIMS: To examine changes and explanatory variables for changes in health-related quality of life in patients treated with long-term mechanical ventilation over a six-year period. BACKGROUND: Long-term mechanical ventilation is a treatment for individuals with chronic hypercapnic respiratory failure, primarily caused by neuromuscular diseases, obesity hypoventilation syndrome, chronic obstructive pulmonary and restrictive thoracic diseases. Studies on long-term outcome on health-related quality of life and factors influencing it are lacking...
October 6, 2017: Journal of Advanced Nursing
https://www.readbyqxmd.com/read/28982219/mechanical-ventilation-for-amyotrophic-lateral-sclerosis-motor-neuron-disease
#10
REVIEW
Aleksandar Radunovic, Djillali Annane, Muhammad K Rafiq, Ruth Brassington, Naveed Mustfa
BACKGROUND: Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is a fatal neurodegenerative disease. Neuromuscular respiratory failure is the most common cause of death, which usually occurs within two to five years of the disease onset. Supporting respiratory function with mechanical ventilation may improve survival and quality of life. This is the second update of a review first published in 2009. OBJECTIVES: To assess the effects of mechanical ventilation (tracheostomy-assisted ventilation and non-invasive ventilation (NIV)) on survival, functional measures of disease progression, and quality of life in ALS, and to evaluate adverse events related to the intervention...
October 6, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28971036/noninvasive-respiratory-management-of-patients-with-neuromuscular-disease
#11
REVIEW
John R Bach
This review article describes definitive noninvasive respiratory management of respiratory muscle dysfunction to eliminate need to resort to tracheotomy. In 2010 clinicians from 22 centers in 18 countries reported 1,623 spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis users of noninvasive ventilatory support (NVS) of whom 760 required it continuously (CNVS). The CNVS sustained their lives by over 3,000 patient-years without resort to indwelling tracheostomy tubes...
August 2017: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/28967584/telemedicine-for-facio-scapulo-humeral-muscular-dystrophy-a-multidisciplinary-approach-to-improve-quality-of-life-and-reduce-hospitalization-rate
#12
Simona Portaro, Rocco Salvatore Calabrò, Placido Bramanti, Giuseppe Silvestri, Michele Torrisi, Valeria Conti-Nibali, Santina Caliri, Christian Lunetta, Bernardo Alagna, Antonino Naro, Alessia Bramanti
BACKGROUND: Facio-Scapulo-Humeral Muscular Dystrophy (FSHD) is an autosomal dominant inherited disorder characterized by a variable and asymmetric involvement of facial, trunk, upper and lower extremity muscles. Although respiratory weakness is a relatively unknown feature of FSHD, it is not rare. Telemedicine has been used in a variety of health care fields, but only recently, with the advent of sophisticated technology, its interest among health professionals became evident, even in such diseases...
September 21, 2017: Disability and Health Journal
https://www.readbyqxmd.com/read/28958052/treating-chronic-hypoventilation-with-automatic-adjustable-versus-fixed-epap-intelligent-volume-assured-positive-airway-pressure-support-ivaps-a-randomized-controlled-trial
#13
Nigel McArdle, Clare Rea, Stuart King, Kathleen Maddison, Dinesh Ramanan, Sahisha Ketheeswaran, Lisa Erikli, Vanessa Baker, Jeff Armitstead, Glenn Richards, Bhajan Singh, David Hillman, Peter Eastwood
Objectives: New noninvasive ventilation (NIV) modes can automatically adjust pressure support settings to deliver effective ventilation in response to varying ventilation demands. It is recommended that fixed expiratory positive airway pressure (FixedEPAP) is determined by attended laboratory polysomnographic (PSG) titration. This study investigated whether automatically determined EPAP (AutoEPAP) was noninferior to FixedEPAP for the control of obstructive sleep apnea (OSA) during intelligent volume-assured pressure support (iVAPS) treatment of chronic hypoventilation...
October 1, 2017: Sleep
https://www.readbyqxmd.com/read/28942692/the-role-of-tele-medicine-in-patients-with-respiratory-diseases
#14
Nicolino Ambrosino, Claudio Fracchia
Tele-medicine is a clinical application connecting a patient with specialized care consultants by means of electronic platforms, potentially able to improve patients' self-management and allow for the care of patients with limited access to health services. This article summarizes the use of tele-medicine as a tool in managing patients suffering from some pathological respiratory conditions. Areas covered: We searched papers published between 1990 and 2017 dealing with tele-medicine and respiratory diseases, chronic obstructive pulmonary disease, asthma, interstitial lung disease, chronic respiratory failure, neuromuscular diseases, critical illness, home mechanical ventilation, and also legal and economic issues...
November 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28916122/myasthenia-gravis-and-crisis-evaluation-and-management-in-the-emergency-department
#15
Jamie Roper, M Emily Fleming, Brit Long, Alex Koyfman
BACKGROUND: Myasthenia gravis (MG) is an uncommon autoimmune disorder affecting the neuromuscular junction and manifesting as muscle weakness. A multitude of stressors can exacerbate MG. When symptoms are exacerbated, muscle weakness can be severe enough to result in respiratory failure, a condition known as myasthenic crisis (MC). OBJECTIVE: This review discusses risk factors, diagnosis, management, and iatrogenic avoidance of MC. DISCUSSION: MC can affect any age, ethnicity, or sex and can be precipitated with any stressor, infection being the most common...
September 12, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28910910/-respiratory-function-measurement-and-noninvasive-ventilation-therapy-for-neuromuscular-diseases
#16
Y Wang, Y M Luo
No abstract text is available yet for this article.
September 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28910906/-noninvasive-ventilation-in-neuromuscular-diseases-respiratory-support-and-airway-management
#17
L P Wang
No abstract text is available yet for this article.
September 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28877087/percutaneous-endoscopic-gastrostomy-under-conscious-sedation-in-patients-with-amyotrophic-lateral-sclerosis-is-safe-an-observational-study
#18
Denise Strijbos, José Hofstede, Daniel Keszthelyi, Ad A M Masclee, Lennard P L Gilissen
OBJECTIVES: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease that causes muscle weakness with respiratory and swallowing dysfunction, eventually leading to death. Permanent enteral feeding is indicated in almost all patients. A percutaneous endoscopic gastrostomy (PEG) tube is considered the first choice, usually performed under conscious sedation (intravenous midazolam). Guidelines are very cautious with respect to sedation in ALS because of the risk for respiratory complications...
November 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28842446/a-natural-history-study-of-x-linked-myotubular-myopathy
#19
Kimberly Amburgey, Etsuko Tsuchiya, Sabine de Chastonay, Michael Glueck, Rachel Alverez, Cam-Tu Nguyen, Anne Rutkowski, Joseph Hornyak, Alan H Beggs, James J Dowling
OBJECTIVE: To define the natural history of X-linked myotubular myopathy (MTM). METHODS: We performed a cross-sectional study that included an online survey (n = 35) and a prospective, 1-year longitudinal investigation using a phone survey (n = 33). RESULTS: We ascertained data from 50 male patients with MTM and performed longitudinal assessments on 33 affected individuals. Consistent with existing knowledge, we found that MTM is a disorder associated with extensive morbidities, including wheelchair (86...
September 26, 2017: Neurology
https://www.readbyqxmd.com/read/28838325/a-study-on-the-safety-and-efficacy-of-reveglucosidase-alfa-in-patients-with-late-onset-pompe-disease
#20
Barry J Byrne, Tarekegn Geberhiwot, Bruce A Barshop, Richard Barohn, Derralynn Hughes, Drago Bratkovic, Claude Desnuelle, Pascal Laforet, Eugen Mengel, Mark Roberts, Peter Haroldsen, Kristin Reilley, Kala Jayaram, Ke Yang, Liron Walsh
BACKGROUND: Late-onset Pompe disease is a rare genetic neuromuscular disorder caused by lysosomal acid alpha-glucosidase (GAA) deficiency that ultimately results in mobility loss and respiratory failure. Current enzyme replacement therapy with recombinant human (rh)GAA has demonstrated efficacy in subjects with late-onset Pompe disease. However, long-term effects of rhGAA on pulmonary function have not been observed, likely related to inefficient delivery of rhGAA to skeletal muscle lysosomes and associated deficits in the central nervous system...
August 24, 2017: Orphanet Journal of Rare Diseases
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