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https://www.readbyqxmd.com/read/29160099/safety-of-intradiaphragmatic-delivery-of-adeno-associated-virus-mediated-alpha-glucosidase-raav1-cmv-hgaa-gene-therapy-in-children-affected-by-pompe-disease
#1
Manuela Corti, Cristina Liberati, Barbara K Smith, Lee Ann Lawson, Ibrahim S Tuna, Thomas J Conlon, Kirsten E Erger, Saleem Islam, Roland Herzog, David D Fuller, Shelley Collins, Barry J Byrne
We conducted a first in human trial of diaphragmatic gene therapy (AAV1-CMV-GAA) to treat respiratory and neural dysfunction in early-onset Pompe disease. The primary objective of this study was to assess the safety of rAAV1-CMV-hGAA vector delivered to the diaphragm muscle of Pompe disease subjects with ventilatory insufficiency. Safety was assessed by measurement of change in serum chemistries and hematology, urinalysis, and immune response to GAA and AAV as well as change in level of health. Our data demonstrate that the AAV treatment was safe and there were no adverse events related to the study agent...
November 21, 2017: Human Gene Therapy. Clinical Development
https://www.readbyqxmd.com/read/29155335/exercise-testing-in-patients-with-diaphragm-paresis
#2
Tristan Bonnevie, Francis-Edouard Gravier, Agathe Ducrocq, David Debeaumont, Catherine Viacroze, Antoine Cuvelier, Jean-François Muir, Catherine Tardif
PURPOSE: Diaphragm paresis (DP) is characterized by abnormalities of respiratory muscle function. However, the impact of DP on exercise capacity is not well known. This study was performed to assess exercise tolerance in patients with DP and to determine whether inspiratory muscle function was related to exercise capacity, ventilatory pattern and cardiovascular function during exercise. METHODS: This retrospective study included patients with DP who underwent both diaphragmatic force measurements, and cardiopulmonary exercise testing (CPET)...
November 16, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/29141527/decreases-in-mixed-venous-blood-o2-saturation-in-cardiac-surgery-patients-following-extubation
#3
Jeffrey Williams, Anna McLean, Jalil Ahari, Arun Jose, Georges Al-Helou, Italo Ibi, Farzad Najam Md, Guillermo Gutierrez
BACKGROUND: Decreases in mixed venous O2 saturation (SvO2) have been reported to occur in postcardiac surgery patients during weaning from mechanical ventilation. Our aim was to establish whether the physiological mechanism responsible for this phenomenon was a decrease in systemic O2 delivery (DO2) or an increase in global O2 consumption ([Formula: see text]O2). METHODS: We studied 21 mechanically ventilated, postoperative cardiac patients for 30 minutes before and 60 minutes after extubation...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/29129228/preoperative-low-muscle-mass-has-a-strong-negative-effect-on-pulmonary-function-in-patients-undergoing-living-donor-liver-transplantation
#4
Hisaya Shirai, Toshimi Kaido, Yuhei Hamaguchi, Siyuan Yao, Atsushi Kobayashi, Shinya Okumura, Naoko Kamo, Shintaro Yagi, Hideaki Okajima, Shinji Uemoto
OBJECTIVE: This study investigated the effect of preoperative sarcopenia on cardiopulmonary function in patients undergoing living donor liver transplantation (LDLT). METHODS: A retrospective analysis was performed of 207 patients who underwent LDLT between January 2008 and April 2015. The quantity and quality of skeletal muscle were evaluated by the psoas muscle mass index (PMI) and intramuscular adipose tissue content (IMAC), respectively, using preoperative computed tomography imaging...
January 2018: Nutrition
https://www.readbyqxmd.com/read/29124630/effectiveness-of-inspiratory-muscle-training-on-sleep-and-functional-capacity-to-exercise-in-obstructive-sleep-apnea-a-randomized-controlled-trial
#5
Adília Karoline Ferreira Souza, Armèle Dornelas de Andrade, Ana Irene Carlos de Medeiros, Maria Inês Remígio de Aguiar, Taciano Dias de Souza Rocha, Rodrigo Pinto Pedrosa, Anna Myrna Jaguaribe de Lima
PURPOSE: The aim of this study was to evaluate the effectiveness of inspiratory muscle training (IMT) on sleep and functional capacity to exercise in subjects with obstructive sleep apnea (OSA). METHODS: This is a controlled, randomized, double-blind study conducted in 16 OSA patients divided into two groups: training (IMT: n = 8) and placebo-IMT (P-IMT: n = 8). IMT was conducted during 12 weeks with a moderate load (50-60% of maximal inspiratory pressure-MIP), while P-IMT used a load < 20% of MPI...
November 9, 2017: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/29119278/development-of-endothermy-in-birds-patterns-and-mechanisms
#6
REVIEW
Edwin R Price, Edward M Dzialowski
Endothermy is a conspicuous and important adaptation in birds. Even though juvenile and adult birds are endothermic and maintain a constant, high body temperature by means of internal heat production, they begin life expressing an ectothermic phenotype. Depending on where a species falls along a continuum of maturity at hatching, from precocial to altricial, they begin to express endothermic traits either close to the time of hatching or as nestlings over a period of 1-3 weeks. Developing endothermy requires attaining a high basal metabolic rate and associated aerobic scope to produce sufficient internal heat, insulation to retain the internally produced heat, and a thermostat that "turns on" heat production in response to cooling ambient temperatures...
November 8, 2017: Journal of Comparative Physiology. B, Biochemical, Systemic, and Environmental Physiology
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
#7
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/29077984/neurally-adjusted-ventilatory-assist-compared-to-other-forms-of-triggered-ventilation-for-neonatal-respiratory-support
#8
REVIEW
Thomas E Rossor, Katie A Hunt, Sandeep Shetty, Anne Greenough
BACKGROUND: Effective synchronisation of infant respiratory effort with mechanical ventilation may allow adequate gas exchange to occur at lower peak airway pressures, potentially reducing barotrauma and volutrauma and development of air leaks and bronchopulmonary dysplasia. During neurally adjusted ventilatory assist ventilation (NAVA), respiratory support is initiated upon detection of an electrical signal from the diaphragm muscle, and pressure is provided in proportion to and synchronous with electrical activity of the diaphragm (EADi)...
October 27, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29077639/short-trail-running-race-beyond-the-classic-model-for-endurance-running-performance
#9
Sabine Ehrström, Marcus P Tartaruga, Christopher S Easthope, Jeanick Brisswalter, Jean-Benoit Morin, Fabrice Vercruyssen
PURPOSE: To examine the extent to which the classical physiological variables of endurance running performance [VO2max, %VO2max at ventilatory threshold (VT), and running economy (RE)], but also muscle strength factors contribute to short trail running (TR) performance. METHODS: A homogeneous group of nine highly-trained trail runners performed an official TR race (27-km) and laboratory-based sessions to determine VO2max, %VO2max at VT, level RE (RE0%) and RE on a +10% slope (RE+10%), voluntary concentric and eccentric knee extension torques (MVCCon and MVCEcc, respectively), local endurance assessed by a fatigue index (FI) and a time to exhaustion at 87...
October 26, 2017: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/29060280/an-ic-based-controllable-stimulator-for-respiratory-muscle-stimulation-investigations
#10
Jonathan Castelli, Florian Kolbl, Ricardo Siu, Gilles N'Kaoua, Yannick Bornat, Ashwin Mangalore, Brian Hillen, James J Abbas, Sylvie Renaud, Ranu Jung, Noelle Lewis
Functional Electrical Stimulation can be used to restore motor functions loss consecutive to spinal cord injury, such as respiratory deficiency due to paralysis of ventilatory muscles. This paper presents a fully configurable IC-centered stimulator designed to investigate muscle stimulation paradigms. It provides 8 current stimulation channels with high-voltage compliance and real-time operation capabilities, to enable a wide range of FES applications. The stimulator can be used in a standalone mode, or within a closed-loop setup...
July 2017: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/29040646/statins-affect-skeletal-muscle-performance-evidence-for-disturbances-in-energy-metabolism
#11
Neeltje A E Allard, Tom J J Schirris, Rebecca J Verheggen, Frans G M Russel, Richard J Rodenburg, Jan A M Smeitink, Paul D Thompson, Maria T E Hopman, Silvie Timmers
Context: Statin myopathy is linked to disturbances in mitochondrial function and exercise intolerance. Objectives: To determine whether differences exist in exercise performance, muscle function and mitochondrial oxidative capacity and content between symptomatic and asymptomatic statin users, and non-statin using controls. Design: Cross-sectional study. Setting: Department of Physiology of the Radboud University Medical Center...
October 9, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29038746/influence-of-exercise-duration-on-cardiorespiratory-responses-energy-cost-and-tissue-oxygenation-within-a-6-hour-treadmill-run
#12
Hugo A Kerhervé, Scott McLean, Karen Birkenhead, David Parr, Colin Solomon
PURPOSE: The physiological mechanisms for alterations in oxygen utilization ([Formula: see text]) and the energy cost of running (Cr ) during prolonged running are not completely understood, and could be linked with alterations in muscle and cerebral tissue oxygenation. METHODS: Eight trained ultramarathon runners (three women; mean ± SD; age 37 ± 7 yr; maximum [Formula: see text] 60 ± 15 mL min(-1) kg(-1)) completed a 6 hr treadmill run (6TR), which consisted of four modules, including periods of moderate (3 min at 10 km h(-1), 10-CR) and heavy exercise intensities (6 min at 70% of maximum [Formula: see text], HILL), separated by three, 100 min periods of self-paced running (SP)...
2017: PeerJ
https://www.readbyqxmd.com/read/29029284/the-influence-of-co2-on-genioglossus-muscle-after-discharge-following-arousal-from-sleep
#13
Jennifer M Cori, Peter D Rochford, Fergal J O'Donoghue, John Trinder, Amy S Jordan
Objectives: Ventilatory after-discharge (sustained elevation of ventilation following stimulus removal) occurs during sleep but not when hypocapnia is present. Genioglossus after-discharge also occurs during sleep, but CO2 effects have not been assessed. The relevance is that postarousal after-discharge may protect against upper airway collapse. This study aimed to determine whether arousal elicits genioglossus after-discharge that persists into sleep, and whether it is influenced by CO2...
November 1, 2017: Sleep
https://www.readbyqxmd.com/read/29027210/oedema-fibrosis-in-duchenne-muscular-dystrophy-role-of-cardiovascular-magnetic-resonance-imaging
#14
REVIEW
Sophie Mavrogeni, Antigoni Papavasiliou, Katerina Giannakopoulou, George Markousis-Mavrogenis, Maria Roser Pons, Evangelos Karanasios, Ioannis Nikas, George Papadopoulos, Genovefa Kolovou, George P Chrousos
Duchenne muscular dystrophy (DMD) is an X-linked muscle disorder characterized by progressive, irreversible loss of cardiac and skeletal muscular function. Muscular enlargement in DMD is attributed to oedema, due to the increased cytoplasmic Na+ concentration. The aim in this review is to present the current experience and emphasize the role of cardiovascular magnetic resonance (CMR) in the diagnosis of this condition. DMD patients' survival depends on ventilatory assistance, as respiratory muscle dysfunction was the most common cause of death in the past...
October 13, 2017: European Journal of Clinical Investigation
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/28977266/potential-clinical-application-of-surface-electromyography-as-indicator-of-neuromuscular-recovery-during-weaning-tests-after-organophosphate-poisoning
#16
Maria Bernarda Salazar Sánchez, Alher Mauricio Hernández Valdivieso, Miguel Ángel Mañanas Villanueva, Andrés Felipe Zuluaga Salazar
This study aimed to explore the usefulness of measuring respiratory muscle activity in mechanically ventilated patients suffering from acute organophosphate poisoning, with a view towards providing complementary information to determine the best time to suspend ventilatory support. Surface electromyography in respiratory muscles (diaphragm, external intercostal and sternocleidomastoid muscles) was recorded in a young man affected by self-poisoning with an unknown amount of parathion to determine the muscle activity level during several weaning attempts from mechanical ventilation...
April 2017: Revista Brasileira de Terapia Intensiva
https://www.readbyqxmd.com/read/28977261/assessment-of-weaning-indexes-based-on-diaphragm-activity-in-mechanically-ventilated-subjects-after-cardiovascular-surgery-a-pilot-study
#17
Isabel Cristina Muñoz Ortega, Alher Mauricio Hernández Valdivieso, Joan Francesc Alonso Lopez, Miguel Ángel Mañanas Villanueva, Luis Horacio Atehortúa Lopez
OBJECTIVE: The aim of this pilot study was to evaluate the feasibility of surface electromyographic signal derived indexes for the prediction of weaning outcomes among mechanically ventilated subjects after cardiac surgery. METHODS: A sample of 10 postsurgical adult subjects who received cardiovascular surgery that did not meet the criteria for early extubation were included. Surface electromyographic signals from diaphragm and ventilatory variables were recorded during the weaning process, with the moment determined by the medical staff according to their expertise...
April 2017: Revista Brasileira de Terapia Intensiva
https://www.readbyqxmd.com/read/28971036/noninvasive-respiratory-management-of-patients-with-neuromuscular-disease
#18
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/28970805/quadriceps-muscles-o2-extraction-and-emg-breakpoints-during-a-ramp-incremental-test
#19
Danilo Iannetta, Ahmad Qahtani, Guillaume Y Millet, Juan M Murias
Muscle deoxygenated breakpoint ([HHb]BP) has been found to be associated with other indices of exercise tolerance in the vastus lateralis (VL) muscle but not in the vastus medialis (VM) and rectus femoris (RF). Purpose: To investigate whether the [HHb]BP occurs also in the VM and RF muscles and whether or not it is associated with other physiological indices of exercise tolerance, such as the EMG threshold (EMGt) and the respiratory compensation point (RCP). Methods: Twelve young endurance trained participants performed maximal ramp incremental (RI) cycling tests (25-30 W·min(-1) increments)...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28953985/relationship-between-aerobic-capacity-and-pelvic-floor-muscles-function-a-cross-sectional-study
#20
S P Jürgensen, A Borghi-Silva, A M F G Bastos, G N Correia, V S Pereira-Baldon, R Cabiddu, A M Catai, P Driusso
The objective of this study was to evaluate the relationship between aerobic capacity and pelvic floor muscles (PFM) function in adult women. Women aged 18 or over and without urinary dysfunction or other chronic diseases were eligible to participate. They completed the habitual physical activity (HPA) questionnaire, underwent a PFM functional evaluation by palpation and perineometry, and performed a submaximal (between 75 and 85% of maximum heart rate) cardiopulmonary exercise (CPX) test to determine the ventilatory anaerobic threshold (VAT)...
September 21, 2017: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
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