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Neuromuscular diseases ventilation

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https://www.readbyqxmd.com/read/28713509/mechanical-ventilation-practice-in-egyptian-pediatric-intensive-care-units
#1
Bassant Salah Meligy, Sally Kamal, Seham Awad El Sherbini
INTRODUCTION: Mechanical ventilation is one of the indispensable tools in pediatric intensive care units. Few studies addressed the epidemiology of pediatric patients on mechanical ventilation and the frequently used modes of ventilation. This is the first study to describe the practice of mechanical ventilation (MV) in Egyptian pediatric intensive care units (PICUs). METHODS: This prospective observational study was conducted from January 2014 to December 2014 in two pediatric intensive care units at Cairo University Pediatric Hospital...
May 2017: Electronic Physician
https://www.readbyqxmd.com/read/28705229/brugada-syndrome-in-a-patient-with-amyotrophic-lateral-sclerosis-a-case-report
#2
Anusha Battineni, Rohit Gummi, Naresh Mullaguri, Raghav Govindarajan
BACKGROUND: Amyotrophic lateral sclerosis is a fatal neuromuscular disorder characterized by progressive death of the upper and lower motor neurons in the central nervous system. Patients with this disease die mostly as a result of respiratory failure; however, owing to prolonged survival through assisted ventilation, cardiovascular causes are increasingly responsible for mortality. We report what is to the best of our knowledge the first case of type 2 Brugada syndrome causing ventricular tachyarrhythmia and cardiac arrest in a patient with upper limb onset amyotrophic lateral sclerosis...
July 14, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28702830/sleep-disordered-breathing-in-patients-with-neuromuscular-disease
#3
REVIEW
Mohamad Ammar Albdewi, Giuseppe Liistro, Riëm El Tahry
Sleep-disordered breathing (SDB) is relatively common in general population as well as in patients with neuromuscular disease. SDB comprises a wide spectrum of disorders varying from simple snoring to complete closure of the upper airway as seen in obstructive sleep apnoea (OSA). It includes also other disorders like prolonged hypoxemia, hypoventilation, and central sleep apnoea (CSA). Neuromuscular diseases (NMD) form a group of disorders that can cause significant reduction in the quality and span of life...
July 12, 2017: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/28694199/initiation-of-non-invasive-ventilation-for-sleep-related-hypoventilation-disorders-advanced-modes-and-devices
#4
REVIEW
Bernardo J Selim, Lisa Wolfe, John M Coleman, Naresh Dewan
Although non-invasive ventilation (NIV) has been used since the 1960's in the polio epidemic, the development of modern bilevel PAP devices did not become a reality until the 1990's. Over the last 25 years bilevel PAP technology options have exponentially increased. The number of patients receiving this treatment both in the acute setting as well as at home is steadily growing. However, a knowledge gap exists in the way these device settings are adjusted to achieve synchrony and match patient's unique respiratory failure physiology...
July 7, 2017: Chest
https://www.readbyqxmd.com/read/28690393/effect-on-lung-function-of-mounthpiece-ventilation-in-steinert-disease-a-case-report
#5
Anna Annunziata, Giuseppe Fiorentino, Antonio Esquinas
In patients with muscular dystrophies both muscle length tension relationship changes and muscle elasticity and plasticity are decreased, resulting in impaired inspiratory muscle function and decreased vital capacity. Furthermore, the loss of deep breathing further increases the risk of alveolar collapse, hypoventilation and atelectasias. In this case report, a stable improvement of vital capacity after treatment with mounthpiece ventilation (MPV), was observed, suggesting that not invasive ventilation (NIV) might help to maintai lung and chest wall compliance, prevent hypoventilation and atelectasias which in turn may slow down the development of the restrictive respiratory pattern...
March 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/28668199/chronic-respiratory-failure-utilization-of-a-pediatric-specialty-integrated-care-program
#6
Robert J Graham, Michael L McManus, Angie Mae Rodday, Ruth Ann Weidner, Susan K Parsons
OBJECTIVE: Describe utilization and satisfaction in a specialty integrated care program for children with severe, chronic respiratory insufficiency (CRI). SUBJECTS: Enrollees of the Critical Care, Anesthesia, Perioperative Extension (CAPE) and Home Ventilation Program. METHODS: Children with CRI received home visits, care coordination, and "on-demand" 24/7 access to physicians. Program activity and outcomes were recorded for 3 years using an adapted Care Coordination Measurement Tool© version...
March 2017: Healthcare
https://www.readbyqxmd.com/read/28660206/attitudes-and-preferences-of-home-mechanical-ventilation-users-from-four-european-countries-an-ers-elf-survey
#7
Sarah Masefield, Michele Vitacca, Michael Dreher, Michael Kampelmacher, Joan Escarrabill, Mara Paneroni, Pippa Powell, Nicolino Ambrosino
Home mechanical ventilation is increasingly used by people with chronic respiratory failure. However, there are few reports on attitudes towards treatment. A web-based survey in eight languages was disseminated across 11 European countries to evaluate the perception of home mechanical ventilation provision in ventilator-assisted individuals and caregivers. Out of 787 responders from 11 European countries, 687 were patients and 100 were caregivers. 95% of patients and 94% of caregivers were from only 4 countries (Germany, the Netherlands, Italy, Spain)...
April 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28624388/geo-economic-variations-in-epidemiology-patterns-of-care-and-outcomes-in-patients-with-acute-respiratory-distress-syndrome-insights-from-the-lung-safe-prospective-cohort-study
#8
John G Laffey, Fabiana Madotto, Giacomo Bellani, Tài Pham, Eddy Fan, Laurent Brochard, Pravin Amin, Yaseen Arabi, Ednan K Bajwa, Alejandro Bruhn, Vladimir Cerny, Kevin Clarkson, Leo Heunks, Kiyoyasu Kurahashi, Jon Henrik Laake, Jose A Lorente, Lia McNamee, Nicolas Nin, Jose Emmanuel Palo, Lise Piquilloud, Haibo Qiu, Juan Ignacio Silesky Jiménez, Andres Esteban, Daniel F McAuley, Frank van Haren, Marco Ranieri, Gordon Rubenfeld, Hermann Wrigge, Arthur S Slutsky, Antonio Pesenti
BACKGROUND: Little information is available about the geo-economic variations in demographics, management, and outcomes of patients with acute respiratory distress syndrome (ARDS). We aimed to characterise the effect of these geo-economic variations in patients enrolled in the Large Observational Study to Understand the Global Impact of Severe Acute Respiratory Failure (LUNG SAFE). METHODS: LUNG SAFE was done during 4 consecutive weeks in winter, 2014, in a convenience sample of 459 intensive-care units in 50 countries across six continents...
June 14, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28546380/respiratory-implications-of-pediatric-neuromuscular-disease
#9
Howard B Panitch
Children with progressive neuromuscular weakness undergo a stereotypical progression of respiratory involvement, beginning with impaired airway clearance and progressing to nocturnal and then diurnal ventilatory failure. This review examines issues related to airway clearance and mucus mobilization, sleep problems, and use of assisted ventilation in children with neuromuscular diseases. Interventions for each of these problems have been created or adapted for the pediatric population. The use of airway clearance therapies and assisted ventilation have improved survival of children with neuromuscular weakness...
June 2017: Respiratory Care
https://www.readbyqxmd.com/read/28503469/motor-neuron-disease-presenting-with-acute-respiratory-failure-a-case-study
#10
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
#11
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...
June 14, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28462858/neuromuscular-disorders-and-chronic-ventilation
#12
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
#13
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
#14
REVIEW
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
#15
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
#16
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
#17
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
#18
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...
August 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28435672/tele-medicine-in-respiratory-diseases
#19
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
#20
Trevor T Nicholson, Sean B Smith, Teepu Siddique, Robert Sufit, Senda Ajroud-Driss, John M Coleman, Lisa F Wolfe
RATIONALE: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease resulting in respiratory failure and death. Use of noninvasive ventilation (NIV) improves survival. However, use of volume-assured pressure support (VAPS) has not been extensively studied in ALS. OBJECTIVES: To explore the clinical usefulness of a detailed evaluation of device-recorded NIV data in the management of chronic respiratory failure in ALS, and to determine whether there are differences in efficacy between patients using VAPS or PS...
July 2017: Annals of the American Thoracic Society
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