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Neuromuscular diseases ventilation

P Gouda, J Chua, D Langan, T Hannon, A Scott, A O'Regan
BACKGROUND: While domiciliary non-invasive ventilation (NIV) was initially used in the treatment of respiratory failure resulting from chest wall deformity, the main indication is now chronic obstructive pulmonary disease (COPD) with recurrent exacerbations associated with type 2 respiratory failure. A longitudinal study of domiciliary NIV provides insights into the evolution of this treatment in the west of Ireland. METHODS: The cohort of patients receiving new prescriptions for domiciliary NIV from Galway University Hospital from 2000 to 2012 was reviewed using study coordinator chart reviews and telephone follow-ups...
October 15, 2016: Irish Journal of Medical Science
Eun Ji Ahn, Mi Sun Yum, Eun Hee Kim, Han Wook Yoo, Beom Hee Lee, Gu Hwan Kim, Tae Sung Ko
BACKGROUND AND PURPOSE: Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by progressive muscle weakness and atrophy. Most SMA patients have a homozygous deletion in survival of motor neuron 1 (SMN1) gene, and neuronal apoptosis inhibitory protein (NAIP) gene is considered a phenotype modifier. We investigated the genotype-phenotype correlation of SMN1 and NAIP deletions in Korean SMA patients. METHODS: Thirty-three patients (12 males and 21 females) treated at the Asan Medical Center between 1999 and 2013 were analyzed retrospectively...
October 7, 2016: Journal of Clinical Neurology
Anna Marie Nathan, Hui Yan Loo, Jessie Anne de Bruyne, Kah Peng Eg, Sze Ying Kee, Surendran Thavagnanam, Marilyn Bouniu, Jiat Earn Wong, Chin Seng Gan, Lucy Chai See Lum
INTRODUCTION: Home ventilation (HV) for children is growing rapidly worldwide. The aim was to describe (1) the sociodemographic characteristics of children on HV and (2) the indications for, means and outcome of initiating HV in children from a developing country. METHODOLOGY: This retrospective study included patients sent home on noninvasive or invasive ventilation, over 13 years, by the pediatric respiratory unit in a single center. Children who declined treatment were excluded...
October 6, 2016: Pediatric Pulmonology
Adam Ogna, Julie Nardi, Helene Prigent, Maria-Antonia Quera Salva, Cendrine Chaffaut, Laure Lamothe, Sylvie Chevret, Djillali Annane, David Orlikowski, Frederic Lofaso
BACKGROUND: Restrictive respiratory failure is a major cause of morbidity and mortality in neuromuscular diseases (NMD). Home mechanical ventilation (HMV) is used to treat hypoventilation, and its efficiency is mostly assessed by daytime blood gases or nocturnal oxygen saturation monitoring (SpO2). Non-invasive transcutaneous measure of CO2 (TcCO2) allows to directly assess nocturnal hypercapnia and to detect residual hypoventilation with a higher sensitivity than SpO2. We aimed to compare the prognostic value of nocturnal SpO2 and TcCO2 in ventilated adult NMD patients...
2016: Frontiers in Medicine
Louise Rose, Neill K Adhikari, Joseph Poon, David Leasa, Douglas A McKim
BACKGROUND: Critically ill mechanically ventilated patients experience impaired airway clearance due to ineffective cough and impaired secretion mobilization. Cough augmentation techniques, including mechanical insufflation-exsufflation (MI-E), manually assisted cough, and lung volume recruitment, improve cough efficiency. Our objective was to describe use, indications, contraindications, interfaces, settings, complications, and barriers to use across Canada. METHODS: An e-mail survey was sent to nominated local survey champions in eligible Canadian units (ICUs, weaning centers, and intermediate care units) with 4 telephone/e-mail reminders...
October 2016: Respiratory Care
Sun Mi Kim, Won Ah Choi, Yu Hui Won, Seong Woong Kang
PURPOSE: To assess the ability of a mechanical in-exsufflator (MI-E), either alone or in combination with manual thrust, to augment cough in patients with neuromuscular disease (NMD) and respiratory muscle dysfunction. MATERIALS AND METHODS: For this randomized crossover single-center controlled trial, patients with noninvasive ventilator-dependent NMD were recruited. The primary outcome was peak cough flow (PCF), which was measured in each patient after a cough that was unassisted, manually assisted following a maximum insufflation capacity (MIC) maneuver, assisted by MI-E, or assisted by manual thrust plus MI-E...
November 2016: Yonsei Medical Journal
Elisa Estenssoro, Arnaldo Dubin
Acute respiratory distress syndrome (ARDS) is an acute respiratory failure produced by an inflammatory edema secondary to increased lung capillary permeability. This causes alveolar flooding and subsequently deep hypoxemia, with intrapulmonary shunt as its most important underlying mechanism. Characteristically, this alteration is unresponsive to high FIO2 and only reverses with end-expiratory positive pressure (PEEP). Pulmonary infiltrates on CXR and CT are the hallmark, together with decreased lung compliance...
2016: Medicina
Matthias Boentert, Bianca Dräger, Christian Glatz, Peter Young
STUDY OBJECTIVES: In neuromuscular disease, non-invasive ventilation (NIV) is indicated if sleep-disordered breathing (SDB) or significant respiratory muscle weakness (RMW) is present. We investigated immediate and long-term effects of NIV on sleep and nocturnal ventilation in patients with late-onset Pompe disease (LOPD). METHODS: Polysomnography and transcutaneous capnometry were performed in 22 adult patients. If indicated, NIV was initiated the subsequent night and follow-up sleep studies were scheduled...
August 22, 2016: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
Paola Ortiz-Salas, Alberto Velez-Van-Meerbeke, Camilo Alberto Galvis-Gomez, Jesús H Rodriguez Q
OBJECTIVES: To evaluate the efficacy and safety over a short time period of human intravenous immunoglobulin versus plasma exchange (PE) in the management of some autoimmune neurologic diseases. In addition, length of hospital stay and duration of ventilator support were compared. METHODS: Randomized controlled trials and analytical observational studies of more than 10 cases were reviewed. Cochrane Neuromuscular Disease Group trials, MEDLINE, EMBASE, HINARI, and Ovid, were searched as data source...
September 2016: Journal of Clinical Neuromuscular Disease
Ghilas Boussaïd, Frédéric Lofaso, Dante Brasil Santos, Isabelle Vaugier, Sandra Pottier, Hélène Prigent, David Orlikowski, Stéphane Bahrami
This study evaluated compliance with non-invasive ventilation in patients with myotonic dystrophy type 1 and identified predictors of cessation at 5 years in a cohort of patients followed in a specialist center for Neuromuscular Diseases in France. Mechanical ventilation in these patients poses a very strong challenge to caregivers. Factors predicting relative compliance were identified using multivariate linear regressions. Cox proportional-hazards regression was used to estimate hazard ratios associated with risk of cessation...
October 2016: Neuromuscular Disorders: NMD
Caitlin S Jackson-Tarlton, Timothy J Benstead, Steve Doucette
Amyotrophic lateral sclerosis (ALS) is associated with nutritional deficits. Gastrostomy tubes are often inserted in patients with ALS to supplement or replace oral intake. The aim of this study was to better understand the practices of gastrostomy tube insertion in patients with ALS. Pre-collected de-identified data were obtained from the Canadian Neuromuscular Disease Registry (CNDR). Feeding tube status was compared with markers of dysphagia, respiratory compromise, and weight status in both univariate and multivariate analysis by employing odds ratios...
August 18, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
David Orlikowski, Helene Prigent, Xavier Ambrosi, Isabelle Vaugier, Sandra Pottier, Djillali Annane, Frederic Lofaso, Adam Ogna
BACKGROUND: Non-invasive transcutaneous capnometry (TcCO2) is used to assess the home ventilation's efficiency. Recently, end-tidal CO2 (ETCO2) sensors have been integrated in life-support home ventilators. The purpose of this study was to compare the ventilator-integrated ETCO2 with TcCO2, in home-ventilated neuromuscular disease patients. METHODS: ETCO2 and TcCO2 were simultaneously measured during one night in 28 patients. Daytime blood gases were drawn on the following morning to measure arterial PCO2 (PaCO2)...
August 2016: Respiratory Medicine
Fernanda F Cruz, Daniel J Weiss, Patricia R M Rocco
INTRODUCTION: Despite progress in understanding the pathophysiology of the acute respiratory distress syndrome (ARDS), it remains a devastating clinical disorder associated with high mortality rates, and those who survive can experience significant long-term morbidity. Recent advances in the management of ARDS have mostly been achieved in supportive care, including the use of protective mechanical ventilation, neuromuscular blocking agents, prone positioning, and conservative fluid strategies...
August 4, 2016: Expert Opinion on Biological Therapy
Barbara K Smith, A Daniel Martin, Lee Ann Lawson, Valerie Vernot, Jordan Marcus, Saleem Islam, Nadeem Shafi, Manuela Corti, Shelley W Collins, Barry J Byrne
Pompe disease is an inherited disorder due to a mutation in the gene that encodes acid α-glucosidase (GAA). Children with infantile-onset Pompe disease develop progressive hypotonic weakness and cardiopulmonary insufficiency that may eventually require mechanical ventilation (MV). Our team conducted a first in human trial of diaphragmatic gene therapy (AAV1-CMV-GAA) to treat respiratory neural dysfunction in infantile-onset Pompe. Subjects (aged 2-15years, full-time MV: n=5, partial/no MV: n=4) underwent a period of preoperative inspiratory muscle conditioning exercise...
July 21, 2016: Experimental Neurology
Erika J MacIntyre, Leyla Asadi, Doug A Mckim, Sean M Bagshaw
Background. The prevalence of patients supported with home mechanical ventilation (HMV) for chronic respiratory failure has increased. However, the clinical outcomes associated with HMV are largely unknown. Methods. We performed a systematic review of studies evaluating patients receiving HMV for indications other than obstructive lung disease, reporting at least one clinically relevant outcome including health-related quality of life (HRQL) measured by validated tools; hospitalization requirements; caregiver burden; and health service utilization...
2016: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
Arsalan Rafiq, Mohsin Ijaz, Hassan Tariq, Trupti Vakde, Richard Duncalf
INTRODUCTION: Idiopathic phrenic nerve palsy is a rare cause of exertional dyspnea. We present a case of a patient presenting with worsening dyspnea of an unknown etiology found to be related to bilateral phrenic nerve palsy. DISCUSSION: Forty-two-year-old man presented to our emergency department with exertional dyspnea, orthopnea, and a left lower lobe consolidation treated initially as bronchitis by his primary physician as an outpatient, then subsequently as pneumonia at another institution, with no improvement in symptomatology...
July 2016: Medicine (Baltimore)
Robin S Howard
PURPOSE OF REVIEW: Understanding the mechanisms and abnormalities of respiratory function in neuromuscular disease is critical to supporting the patient and maintaining ventilation in the face of acute or chronic progressive impairment. RECENT FINDINGS: Retrospective clinical studies reviewing the care of patients with Guillain-Barré syndrome and myasthenia have shown a disturbingly high mortality following step-down from intensive care. This implies high dependency and rehabilitation management is failing despite evidence that delayed improvement can occur with long-term care...
October 2016: Current Opinion in Neurology
W Windisch, B Schönhofer, F S Magnet, E Stoelben, H-J Kabitz
There are many reasons for an impairment of the diaphragmatic function potentially affecting all components of the respiratory pump. Particularly, diagnosis and treatment of unilateral and bilateral phrenic nerve paralysis are challenging. Neuromuscular disorders, trauma, iatrogenic conditions, tumor compression, but also infectious and inflammatory conditions in addition to neuralgic amyotrophy and idiopathic phrenic nerve paralysis are reasons for phrenic nerve paralysis. Primarily, diagnostic procedures include the anamnesis, physical examination, blood gas analysis, lung function testing and the diagnosis of the underlying disease...
July 2016: Pneumologie
J Raignoux, U Walther-Louvier, C Espil, L Berthomieu, E Uro-Coste, F Rivier, C Cances
UNLABELLED: Apart from spinal muscular atrophy (SMA) and myotonic dystrophy type 1 (DM1), congenital neuromuscular diseases with early neonatal symptoms mean diagnostic and prognostic challenges mainly when infants require ventilatory support. OBJECTIVES: Consider a standardized strategy for infants suspected of congenital neuromuscular disease from analysis of the literature and retrospective experience with floppy and ventilatory support-dependent infants, after exclusion of well-known diseases (DM1, SMA)...
September 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Eduardo Barbin Zuccolotto, Guilherme Coelho Machado Nunes, Rafael Soares Lopes Nogueira, Eugenio Pagnussatt Neto, José Roberto Nociti
BACKGROUND AND OBJECTIVES: Multiple sclerosis is a demyelinating disease of the brain and spinal cord, characterized by muscle weakness, cognitive dysfunction, memory loss, and personality disorders. Factors that promote disease exacerbation are stress, physical trauma, infection, surgery, and hyperthermia. The objective is to describe the anesthetic management of a case referred to urological surgery. CASE REPORT: A female patient, 44 years of age, with multiple sclerosis, diagnosed with nephrolithiasis, referred for endoscopic ureterolythotripsy...
July 2016: Brazilian Journal of Anesthesiology
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