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Neuromuscular diseases ventilation

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https://www.readbyqxmd.com/read/29323413/extracorporeal-membrane-oxygenation-for-refractory-severe-respiratory-failure-in-acute-interstitial-pneumonia
#1
Gabriela Gonçalves-Venade, Nuno Lacerda-Príncipe, Roberto Roncon-Albuquerque, José Artur Paiva
Acute interstitial pneumonia (AIP) is a rare idiopathic interstitial lung disease with rapid progressive respiratory failure and high mortality. In the present report, three cases of AIP complicated by refractory respiratory failure supported with extracorporeal membrane oxygenation (ECMO) are presented. One male and two female patients (ages 27-59) were included. Venovenous ECMO support was provided using miniaturized systems, with two-site femoro-jugular circuit configuration. Despite lung protective ventilation, prone position and neuromuscular blockade, refractory respiratory failure of unknown etiology supervened (ratio of arterial oxygen partial pressure to fractional inspired oxygen 46-130) and ECMO was initiated after 3-7 days of mechanical ventilation...
January 11, 2018: Artificial Organs
https://www.readbyqxmd.com/read/29298901/five-year-follow-up-and-outcomes-of-noninvasive-ventilation-in-subjects-with-neuromuscular-diseases
#2
Mi Ri Suh, Won Ah Choi, Dong Hyun Kim, Jang Woo Lee, Eun Young Kim, Seong-Woong Kang
INTRODUCTION: The purpose of this study was to investigate the 5-year outcomes of noninvasive ventilation (NIV) application in different neuromuscular disease (NMD) groups. METHODS: We categorized 180 subjects who had initiated NIV between March 2001 and August 2009 into 4 groups and followed them for > 5 y. The NIV maintenance rate and average duration, applying time, and forced vital capacity (FVC) were investigated at the time NIV was initiated and 5 y after NIV initiation in each group...
January 3, 2018: Respiratory Care
https://www.readbyqxmd.com/read/29243458/prevalence-of-life-supporting-prolonged-invasive-ventilation-support-in-finland
#3
Waltteri Siirala, Aki Vainionpää, Annette Kainu, Jaana Korpela, Klaus Olkkola, Riku Aantaa
BACKGROUND: There is no comprehensive data in our country on the prevalence of life-supporting prolonged invasive ventilation support. The objective of the survey was to clarify in all hospital districts of continental Finland the prevalence of patients who were dependent on invasive ventilation support, and the disease leading to the treatment. PATIENTS AND METHODS: The KOTIVEHNO 2015 survey was carried out as population-based cross-sectional study by sending a questionnaire to all doctors in charge of prolonged invasive ventilation support...
2017: Duodecim; Lääketieteellinen Aikakauskirja
https://www.readbyqxmd.com/read/29208755/home-mechanical-ventilation-a-12-year-population-based-retrospective-cohort-study
#4
Marcus Povitz, Louise Rose, Salimah Z Shariff, Sean Leonard, Blayne Welk, Krista Bray Jenkyn, David J Leasa, Andrea S Gershon
BACKGROUND: Increasing numbers of individuals are being initiated on home mechanical ventilation, including noninvasive (bi-level) and invasive mechanical ventilation delivered via tracheostomy due to chronic respiratory failure to enable symptom management and promote quality of life. Given the high care needs of these individuals, a better understanding of the indications for home mechanical ventilation, and health-care utilization is needed. METHODS: We performed a retrospective cohort study using provincial health administrative data from Ontario, Canada (population ∼13,000,000)...
December 5, 2017: Respiratory Care
https://www.readbyqxmd.com/read/29208343/single-center-experience-with-intrathecal-administration-of-nusinersen-in-children-with-spinal-muscular-atrophy-type-1
#5
Astrid Pechmann, Thorsten Langer, Sabine Wider, Janbernd Kirschner
BACKGROUND: Spinal muscular atrophy (SMA) is a neuromuscular disorder mainly characterized by proximal muscle weakness. There have been enormous advances in therapeutic development with the possibility to influence the clinical course of the disease. Nusinersen is the first approved drug to treat SMA. It is administered intrathecally and acts as splicing modifier of the SMN2 gene. METHODS: Lumbar punctures were performed using a standardized protocol. To evaluate safety and feasibility of the intrathecal treatment, vital signs and the need for sedation, analgesia or mechanical ventilation during the procedure were monitored...
November 21, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29201832/letter-to-the-editor-successful-extubation-after-weaning-failure-by-noninvasive-ventilation-in-patients-with-neuromuscular-disease-do-we-appreciate-the-bigger-picture
#6
COMMENT
Alastair J Glossop, Antonio M Esquina
No abstract text is available yet for this article.
October 2017: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/29199768/invasive-versus-non-invasive-ventilation-for-acute-respiratory-failure-in-neuromuscular-disease-and-chest-wall-disorders
#7
REVIEW
Fang Luo, Djillali Annane, David Orlikowski, Li He, Mi Yang, Muke Zhou, Guan J Liu
BACKGROUND: Acute respiratory failure is a common life-threatening complication of acute onset neuromuscular diseases, and may exacerbate chronic hypoventilation in patients with neuromuscular disease or chest wall disorders. Standard management includes oxygen supplementation, physiotherapy, cough assistance, and, whenever needed, antibiotics and intermittent positive pressure ventilation. Non-invasive mechanical ventilation (NIV) via nasal, buccal or full-face devices has become routine practice in many centres...
December 4, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29174288/-update-on-the-respiratory-management-of-patients-with-chronic-neuromuscular-disease
#8
REVIEW
P Priou, W Trzepizur, N Meslier, F Gagnadoux
BACKGROUND: Neuromuscular diseases include a wide range of conditions that may involve potentially life-threatening respiratory complications (infection, respiratory failure). SURVEILLANCE AND PULMONARY FUNCTION TESTS: For patients with neuromuscular diseases, clinical assessment of respiratory function and regular pulmonary function tests are needed to screen for nocturnal respiratory disorders, weakness of the diaphragm and potential restrictive disorders and/or chronic hypercapnic respiratory insufficiency, possibly with couch deficiency...
November 21, 2017: Revue de Pneumologie Clinique
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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-6-year-follow-up-study
#17
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 6-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
#18
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
#19
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
#20
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
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