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Neuromuscular diseases ventilation

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https://www.readbyqxmd.com/read/28916122/myasthenia-gravis-and-crisis-evaluation-and-management-in-the-emergency-department
#1
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
#2
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
#3
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
#4
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...
September 4, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28842446/a-natural-history-study-of-x-linked-myotubular-myopathy
#5
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...
August 25, 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
#6
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
https://www.readbyqxmd.com/read/28834910/application-of-different-ventilator-modes-in-patients-with-amyotrophic-lateral-sclerosis-according-to-certain-clinical-situations-a-case-report
#7
Donghwi Park
RATIONALE: Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease that involves limb, axial, bulbar, and respiratory muscles. Fatigue and weakness of the respiratory muscles eventually induce respiratory insufficiency, which is one of the main causes of death in patients with ALS. In ALS patients with respiratory insufficiency, application of a ventilator is indispensable. Although there are various modes of ventilation, these modes are classified roughly into volume-controlled ventilation (VCV) and pressure-controlled ventilation (PCV)...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28834856/review-of-continuous-infusion-neuromuscular-blocking-agents-in-the-adult-intensive-care-unit
#8
Keaton S Smetana, Neil A Roe, Bruce A Doepker, G Morgan Jones
The use of continuous infusion neuromuscular blocking agents remains controversial. The clinical benefit of these medications may be overshadowed by concerns of propagating intensive care unit-acquired weakness, which may prolong mechanical ventilation and impair the inability to assess neurologic function or pain. Despite these risks, the use of neuromuscular blocking agents in the intensive care unit is indicated in numerous clinical situations. Understanding pharmacologic nuances and clinical roles of these agents will aid in facilitating safe use in a variety of acute disease processes...
October 2017: Critical Care Nursing Quarterly
https://www.readbyqxmd.com/read/28806453/neurologic-complications-associated-with-the-zika-virus-in-brazilian-adults
#9
Ivan Rocha Ferreira da Silva, Jennifer A Frontera, Ana Maria Bispo de Filippis, Osvaldo Jose Moreira do Nascimento
Importance: There are no prospective cohort studies assessing the incidence and spectrum of neurologic manifestations secondary to Zika virus (ZIKV) infection in adults. Objective: To evaluate the rates of acute ZIKV infection among patients hospitalized with Guillain-Barré syndrome (GBS), meningoencephalitis, or transverse myelitis. Design, Setting, and Participants: A prospective, observational cohort study was conducted at a tertiary referral center for neurological diseases in Rio de Janeiro, Brazil, between December 5, 2015, and May 10, 2016, among consecutive hospitalized adults (>18 years of age) with new-onset acute parainfectious or neuroinflammatory disease...
August 14, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28777179/comorbid-sleep-disturbances-in-neurologic-disorders
#10
Yo-El S Ju, Aleksandar Videnovic, Bradley V Vaughn
PURPOSE OF REVIEW: This article provides a review of disturbances of sleep comorbid with common neurologic disorders. RECENT FINDINGS: A wide variety of neurologic disorders are frequently complicated by comorbid sleep disturbances. In many cases, a bidirectional relationship appears to occur between sleep function and the neurologic disease, such that treatment of comorbid sleep disturbances may improve the symptoms of the neurologic disease. SUMMARY: Neurologic disorders are often associated with abnormalities of sleep...
August 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28758083/successful-extubation-after-weaning-failure-by-noninvasive-ventilation-in-patients-with-neuromuscular-disease-case-series
#11
Sun Mi Kim, Seong-Woong Kang, Young-Chul Choi, Yoon Ghil Park, Yu Hui Won
OBJECTIVE: To report successful cases of extubation from invasive mechanical ventilation at our institution using pulmonary rehabilitation consisting of noninvasive ventilation (NIV) in neuromuscular patients with experience of reintubation. METHODS: Patients who experienced extubation failure via the conventional weaning strategy but afterwards had extubation success via NIV were studied retrospectively. Continuous end-tidal CO2 (ETCO2) and pulse oxyhemoglobin saturation (SpO2) monitoring were performed...
June 2017: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/28743332/-clinical-significance-of-early-immunological-paralysis-in-patients-with-severe-h1n1-influenza-a
#12
Yongbing Qian, Hui Xie, Rui Tian, Jian Lu, Wei Jin, Ruilan Wang
OBJECTIVE: To analysis the immunological characteristics of patients with severe H1N1 influenza A, and to provide theoretical basis for predicting the prognosis of the disease. METHODS: A retrospective analysis was conducted. The clinical data of 15 patients diagnosed with severe H1N1 influenza A and admitted to Shanghai General Hospital of Nanjing Medical University from October 2015 to December 2016 were collected. All the patients were divided into survival and death groups according to 28-day survival...
July 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/28713509/mechanical-ventilation-practice-in-egyptian-pediatric-intensive-care-units
#13
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
#14
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
#15
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-noninvasive-ventilation-for-sleep-related-hypoventilation-disorders-advanced-modes-and-devices
#16
REVIEW
Bernardo J Selim, Lisa Wolfe, John M Coleman, Naresh A Dewan
Although noninvasive ventilation (NIV) has been used since the 1950s in the polio epidemic, the development of modern bilevel positive airway pressure (BPAP) devices did not become a reality until the 1990s. Over the past 25 years, BPAP technology options have increased exponentially. The number of patients receiving this treatment both in the acute setting and at home is growing steadily. However, a knowledge gap exists in the way the settings on these devices are adjusted to achieve synchrony and match the patient's unique physiology of respiratory failure...
July 8, 2017: Chest
https://www.readbyqxmd.com/read/28690393/effect-on-lung-function-of-mounthpiece-ventilation-in-steinert-disease-a-case-report
#17
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
#18
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
#19
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
#20
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...
August 2017: Lancet Respiratory Medicine
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