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Neuromuscular diseases ventilation

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https://www.readbyqxmd.com/read/29773292/health-related-quality-of-life-measurement-in-patients-with-chronic-respiratory-failure
#1
REVIEW
Toru Oga, Wolfram Windisch, Tomohiro Handa, Toyohiro Hirai, Kazuo Chin
The improvement of health-related quality of life (HRQL) is an important goal in managing patients with chronic respiratory failure (CRF) receiving long-term oxygen therapy (LTOT) and/or domiciliary noninvasive ventilation (NIV). Two condition-specific HRQL questionnaires have been developed to specifically assess these patients: the Maugeri Respiratory Failure Questionnaire (MRF) and the Severe Respiratory Insufficiency Questionnaire (SRI). The MRF is more advantageous in its ease of completion; conversely, the SRI measures diversified health impairments more multi-dimensionally and discriminatively with greater balance, especially in patients receiving NIV...
May 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29724584/stereotactic-body-radiation-therapy-for-heart-base-tumors-in-six-dogs
#2
L M Magestro, T L Gieger, M W Nolan
INTRODUCTION: Heart-base tumors are increasingly treated with radiotherapy, yet safety and efficacy are incompletely understood. This case series describes outcomes after stereotactic body radiation therapy (SBRT) for presumed chemodectoma. ANIMALS: Six pet dogs. METHODS: A retrospective study was performed, including dogs with a clinical diagnosis of chemodectoma and treatment with three-fraction SBRT (30 Gy total). RESULTS: Heart-base tumors, presumed or confirmed to be chemodectomas, were diagnosed via histopathology or imaging...
April 30, 2018: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/29701428/anaesthetic-management-in-a-duchennne-muscle-dystrophy-patient-for-treatment-of-recurrent-pneumothorax
#3
Sara Mota, Liuba Germanova, Joana Cortesão, Teresa Paiva
INTRODUCTION: Duchenne muscular dystrophy (DMD) is an x linked recessive disorder. Long term prognosis is ominous, with development of respiratory distress and cardiomyopathy in advanced stage of the disease and expected death in the teens-to-mid 20s due to respiratory or cardiac failure. Peri-operative management of this patients is challenging due to difficult airway anatomy (macroglossia, limited neck and mandibular mobility). Additionally, they are at risk of developing malignant hyperthermia, rhabdomyolysis and hyperkalemic cardiac arrest when exposed to halogenated inhalational anaesthetics and depolarizing muscle relaxants...
July 2017: Revista Portuguesa de Cirurgia Cardio-torácica e Vascular
https://www.readbyqxmd.com/read/29692352/respiratory-care-in-neuromuscular-diseases
#4
REVIEW
Ashima S Sahni, Lisa Wolfe
Caring for patients with neuromuscular disease (NMD) is challenging. Respiratory care is of the utmost importance because it is a major determinant of quality of life and survival. Noninvasive ventilation (NIV) is one of the few modalities that has shown survival benefit in the NMD patient population. Newer modes with smart technologies are being developed to assist in better ventilation. Some noninvasive methods have shown success in the management of sialorrhea, which is of paramount importance in the initiation of NIV...
April 24, 2018: Respiratory Care
https://www.readbyqxmd.com/read/29660267/recovery-process-and-determinants-of-adverse-event-occurrence-in-bronchoscopic-procedures-performed-under-general-anesthesia
#5
Derya Özden Omaygenç, Nermin Ünal, Saadet İpek Edipoğlu, Tuğçe Barca Şeker, Mehmet Akif Özgül, Demet Turan, Cengiz Özdemir, İbrahim Oğuz Karaca, Erdoğan Çetinkaya
OBJECTIVE: Regarding the fact that rigid bronchoscopy is generally performed under general anesthesia and this patient subgroup is remarkably morbid, encountering procedure and/or anesthesia related complications are highly likely. Here, we aimed to assess factors influencing recovery and detect possible determinants of adverse event occurence during these operations performed in a tertiary referral centre. METHODS: 81 consecutive ASA I-IV patients were recruited for this investigation...
April 16, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29614690/characteristics-of-japanese-patients-with-becker-muscular-dystrophy-and-intermediate-muscular-dystrophy-in-a-japanese-national-registry-of-muscular-dystrophy-remudy-heterogeneity-and-clinical-variation
#6
Madoka Mori-Yoshimura, Satomi Mitsuhashi, Harumasa Nakamura, Hirofumi Komaki, Kanako Goto, Naohiro Yonemoto, Fumi Takeuchi, Yukiko K Hayashi, Miho Murata, Yuji Takahashi, Ichizo Nishino, Shin'ichi Takeda, En Kimura
BACKGROUND: Obtaining an adequate number of patients to conduct a natural history study for rare diseases such as Becker muscular dystrophy (BMD) is difficult. OBJECTIVES: The present study used data from Remudy, a national registry for neuromuscular diseases in Japan, to conduct a phenotypic analysis of BMD. METHODS: We analyzed Remudy data of participants with dystrophinopathy. All participants who were aged 17 and older and were ambulant at age 13 were included in this study...
March 26, 2018: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/29566571/respiratory-measures-in-amyotrophic-lateral-sclerosis
#7
Noah Lechtzin, Merit E Cudkowicz, Mamede de Carvalho, Angela Genge, Orla Hardiman, Hiroshi Mitsumoto, Jesus S Mora, Jeremy Shefner, Leonard H Van den Berg, Jinsy A Andrews
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease that causes skeletal muscle weakness, including muscles involved with respiration. Death often results from respiratory failure within 3-5 years. Monitoring respiratory status is therefore critical to ALS management, as respiratory/pulmonary function tests (PFTs) are used to make decisions including when to initiate noninvasive ventilation. Understanding the different respiratory and PFTs as they relate to disease progression and survival may help determine which tests are most suitable...
March 23, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29557601/sedation-and-analgesia-in-patients-on-mechanical-ventilation-in-pediatric-intensive-care-units-in-argentina
#8
Pedro Taffarel, German Bonetto, Facundo Jorro Barón, Claudia Meregalli
INTRODUCTION: Children in pediatric intensive care units (PICUs) are exposed to experiencing pain, stress and anxiety due to their disease, treatment or care setting. Adequate sedation and analgesia are key to their care, particularly in patients requiring mechanical ventilation (MV). OBJECTIVE: To determine the usual practice in sedation and analgesia management in patients requiring MV in PICUs in Argentina. MATERIAL AND METHODS: Descriptive, crosssectional, multi-center study conducted by means of e-mailed surveys...
April 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29547457/retrospective-application-of-new-pediatric-ventilator-associated-pneumonia-criteria-identifies-a-high-risk-population
#9
Ashley Gionfriddo, Mika L Nonoyama, Peter C Laussen, Peter N Cox, Megan Clarke, Alejandro A Floh
OBJECTIVES: To promote standardization, the Centers for Disease Control and Prevention introduced a new ventilator-associated pneumonia classification, which was modified for pediatrics (pediatric ventilator-associated pneumonia according to proposed criteria [PVAP]). We evaluated the frequency of PVAP in a cohort of children diagnosed with ventilator-associated pneumonia according to traditional criteria and compared their strength of association with clinically relevant outcomes. DESIGN: Retrospective cohort study...
March 15, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29543311/unusual-case-of-central-alveolar-hypoventilation
#10
Hadil Ak AlOtair, Abdulaziz H Alzeer, Mohammed A Abdou, Shaden O Qasrawi
Central alveolar hypoventilation is rarely encountered. This case report describes a young woman who was recently diagnosed with hypertension and ischemic heart disease. She presented to the emergency room with hypercapnic respiratory failure, for which she was mechanically ventilated. This was preceded by an acute upper respiratory tract infection. She was initially suspected to have Guillain-Barré syndrome, but further investigations ruled out neuromuscular or autoimmune disorders. Sleep-related hypoventilation was suspected after she experienced recurrent apneas at night that resulted in re-intubation...
March 2018: Saudi Medical Journal
https://www.readbyqxmd.com/read/29521672/effects-of-prone-positioning-on-transpulmonary-pressures-and-end-expiratory-volumes-in-patients-without-lung-disease
#11
Abirami Kumaresan, Robert Gerber, Ariel Mueller, Stephen H Loring, Daniel Talmor
BACKGROUND: The effects of prone positioning on esophageal pressures have not been investigated in mechanically ventilated patients. Our objective was to characterize effects of prone positioning on esophageal pressures, transpulmonary pressure, and lung volume, thereby assessing the potential utility of esophageal pressure measurements in setting positive end-expiratory pressure (PEEP) in prone patients. METHODS: We studied 16 patients undergoing spine surgery during general anesthesia and neuromuscular blockade...
June 2018: Anesthesiology
https://www.readbyqxmd.com/read/29511037/analysis-of-a-pediatric-home-mechanical-ventilator-population
#12
Rambod Amirnovin, Sara Aghamohammadi, Carley Riley, Marlyn S Woo, Sylvia Del Castillo
BACKGROUND: The population of children requiring home mechanical ventilation has evolved over the years and has grown to include a variety of diagnoses and needs that have led to changes in the care of this unique population. The purpose of this study was to provide a descriptive analysis of pediatric patients requiring home mechanical ventilation after hospitalization and how the evolution of this technology has impacted their care. METHODS: A retrospective, observational, longitudinal analysis of 164 children enrolled in a university-affiliated home mechanical ventilation program over 26 years was performed...
May 2018: Respiratory Care
https://www.readbyqxmd.com/read/29501252/pulmonary-function-in-former-very-low-birth-weight-preterm-infants-in-the-first-year-of-life
#13
Daniela de Melo Miranda Gonçalves, Gustavo Falbo Wandalsen, Ana Sílvia Scavacini, Fernanda Cordoba Lanza, Ana Lucia Goulart, Dirceu Solé, Amélia Miyashiro Nunes Dos Santos
BACKGROUND: Pulmonary function in former preterm infants may be compromised during childhood. OBJECTIVES: To assess pulmonary function in very-low-birth-weight preterm infants at 6-12 months of corrected age and analyze the factors associated with abnormal pulmonary function. METHODS: Cross-sectional study with preterm infants at 6-12 months of corrected age with birth weight <1500 g. Children with malformations or affected by neuromuscular and respiratory diseases were excluded...
March 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29484287/long-term-non-invasive-ventilation-in-infants-a-systematic-review-and-meta-analysis
#14
Prabhjot K Bedi, Maria Luisa Castro-Codesal, Robin Featherstone, Mohammed M AlBalawi, Bashar Alkhaledi, Anita L Kozyrskyj, Carlos Flores-Mir, Joanna E MacLean
Background: The use of long-term non-invasive ventilation (NIV) to treat sleep and breathing disorders in children has increased substantially in the last decade; however, less data exist about its use in infants. Given that infants have distinct sleep and breathing patterns when compared to older children, the outcomes of infants on long-term NIV may differ as well. The aim of this study is to systematically review the use and outcomes of long-term NIV in infants. Methods: Ovid Medline, Ovid Embase, CINAHL (via EbscoHOST), PubMed, and Wiley Cochrane Library were systematically searched from January 1990 to July 2017...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29453639/upper-airway-obstruction-induced-by-non-invasive-ventilation-using-an-oronasal-interface
#15
Valentin Schellhas, Christian Glatz, Ingo Beecken, Angelika Okegwo, Anna Heidbreder, Peter Young, Matthias Boentert
BACKGROUND: On initiation of long-term non-invasive ventilation (NIV), intermittent upper airway obstruction has rarely been described as possibly treatment-induced. Inspiratory pressure effects and the use of an oronasal interface may promote obstructive events in some patients with neuromuscular disease (NMD) and amyotrophic lateral sclerosis (ALS) in particular. METHODS: We evaluated clinical data from 212 patients in whom NIV was initiated using an oronasal mask...
February 17, 2018: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/29449118/shot-by-a-gun-%C3%A2-missed-by-a-provider
#16
Eddie Garcia, Abdul H Zaid, Diane P Calello, Lisa McHugh, Grant Arzumanov, Najaf Asrar, Ari Sapin, Kristin G Fless
BACKGROUND: Botulism is a paralytic disease caused by the neurotoxin produced by Clostridium botulinum. The majority of cases are due to ingestion or injection drug use. Wound botulism from traumatic injury is exceedingly rare, with only one to two cases reported each year in the United States. CASE REPORT: A 27-year-old man presented to the Emergency Department with diplopia, dysphagia, and progressive weakness 10 days after sustaining a gunshot wound to his right lower leg...
April 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29424188/revolving-door-respiratory-patients-a-rehabilitative-perspective
#17
Massimiliano Polastri, Lara Pisani, Andrea Dell'Amore, Stefano Nava
Rehabilitation is an integral component of care for patients affected by either acute or chronic pulmonary diseases. The key elements of rehabilitation treatment for critical respiratory patients are as follows: weaning from mechanical ventilation, respiratory therapy, physical reconditioning, and occupational therapy. It should be noted that patients affected by pulmonary diseases are prone to hospital re-admission due to frequent exacerbations, especially in cases with more severe stages of chronic obstructive pulmonary disease...
September 22, 2017: Monaldi Archives for Chest Disease, Archivio Monaldi Per le Malattie del Torace
https://www.readbyqxmd.com/read/29419414/motor-neuron-degeneration-correlates-with-respiratory-dysfunction-in-sca1
#18
James P Orengo, Meike E van der Heijden, Shuang Hao, Jianrong Tang, Harry T Orr, Huda Y Zoghbi
Spinocerebellar ataxia type 1 (SCA1) is characterized by adult-onset cerebellar degeneration with attendant loss of motor coordination. Bulbar function is eventually impaired and patients typically die from an inability to clear the airway. We investigated whether motor neuron degeneration is at the root of bulbar dysfunction by studying SCA1 knock-in ( Atxn1154Q/+ ) mice. Spinal cord and brainstem motor neurons were assessed in Atxn1154Q/+ mice at 1, 3 and 6 months of age. Specifically, we assessed breathing physiology, diaphragm histology and electromyography, and motor neuron histology and immunohistochemistry...
February 26, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29415556/patterns-of-use-survival-and-prognostic-factors-in-patients-receiving-home-mechanical-ventilation-in-western-australia-a-single-centre-historical-cohort-study
#19
Geak Poh Tan, Nigel McArdle, Satvinder Singh Dhaliwal, Jane Douglas, Clare Siobhan Rea, Bhajan Singh
Home mechanical ventilation (HMV) is used in a wide range of disorders associated with chronic hypoventilation. We describe the patterns of use, survival and predictors of death in Western Australia. We identified 240 consecutive patients (60% male; mean age 58 years and body mass index 31 kg m-2 ) referred for HMV between 2005 and 2010. The patients were grouped into four categories: motor neurone disorders (MND; 39%), pulmonary disease (PULM; 25%, mainly chronic obstructive pulmonary disease), non-MND neuromuscular and chest wall disorders (NMCW; 21%) and the obesity hypoventilation syndrome (OHS; 15%)...
January 1, 2018: Chronic Respiratory Disease
https://www.readbyqxmd.com/read/29406371/pediatric-specialty-care-model-for-management-of-chronic-respiratory-failure-cost-and-savings-implications-and-misalignment-with-payment-models
#20
Robert J Graham, Michael L McManus, Angie Mae Rodday, Ruth Ann Weidner, Susan K Parsons
OBJECTIVE: To describe program design, costs, and savings implications of a critical care-based care coordination model for medically complex children with chronic respiratory failure. DESIGN: All program activities and resultant clinical outcomes were tracked over 4 years using an adapted version of the Care Coordination Measurement Tool. Patient characteristics, program activity, and acute care resource utilization were prospectively documented in the adapted version of the Care Coordination Measurement Tool and retrospectively cross-validated with hospital billing data...
February 3, 2018: Pediatric Critical Care Medicine
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