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neuromuscular respiratory disorders

Fernando R Carvalho, Débora A Lentini-Oliveira, Lucila Bf Prado, Gilmar F Prado, Luciane Bc Carvalho
BACKGROUND: Apnoea is a breathing disorder marked by the absence of airflow at the nose or mouth. In children, risk factors include adenotonsillar hypertrophy, obesity, neuromuscular disorders and craniofacial anomalies. The most common treatment for obstructive sleep apnoea syndrome (OSAS) in childhood is adeno-tonsillectomy. This approach is limited by its surgical risks, mostly in children with comorbidities and, in some patients, by recurrence that can be associated with craniofacial problems...
October 5, 2016: Cochrane Database of Systematic Reviews
Paolo Manzoni, Bosco Paes, Krista L Lanctôt, Alberto DallʼAgnola, Ian Mitchell, Sara Calabrese, Milena Maule, Elisa Girardi, Tetsuhiro Harimoto, Abby Li
BACKGROUND: Respiratory syncytial virus (RSV) infection frequently results in RSV-related hospitalization (RSVH) in young infants. We examined the outcomes of palivizumab recipients within the Canadian Registry (CARESS) and the Torino-Verona Italian Registry over the 2002-2014 RSV seasons. METHODS: RSVHs were captured during the study seasons. Premature infants who received palivizumab (≤35 completed weeks gestational age (wGA); Group1) were compared with infants given palivizumab for underlying disorders regardless of gestational age (Group2)...
September 19, 2016: Pediatric Infectious Disease Journal
Susan E George, Diane F Borello-France
Functional constipation is a common bowel disorder leading to activity restrictions, and reduced health-related quality of life. Typically, this condition is initially managed with prescription of laxatives and/or fiber supplementation. However, these interventions are often ineffective and fail to address the underlying pathophysiology and impairments contributing to this condition. Physical therapists possess knowledge and skills to diagnose and manage a wide range of musculoskeletal and motor coordination impairments that may contribute to functional constipation...
September 15, 2016: Physical Therapy
H Coleman Robinson
Restrictive lung diseases are a heterogeneous group of conditions characterized by a restrictive pattern on spirometry and confirmed by a reduction in total lung volume. Patients with more severe symptoms may have a reduced diffusing capacity of the lung for carbon monoxide. Etiologies can be intrinsic with lung parenchymal involvement, as in interstitial lung diseases, or extrinsic to the lung, as in obesity and neuromuscular disorders. Idiopathic pulmonary fibrosis is a chronic progressive interstitial pneumonia with fibrosis for which treatment is primarily supportive with oxygen therapy, pulmonary rehabilitation, and management of comorbid conditions...
September 2016: FP Essentials
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
Florian Stehling, Katharina Alfen, Christian Dohna-Schwake, Uwe Mellies
Background In pediatric neuromuscular disorders (NMD), respiratory muscle weakness parallels respiratory failure. The objectives of this study are (1) to evaluate respiratory muscle capacity in neuromuscular children and (2) to assess the relationship between vital capacity, respiratory muscle performance, and alveolar ventilation during sleep and wakefulness. Methods Inspiratory vital capacity (IVC), peak inspiratory pressure (PIP), mouth occlusion pressure (P0.1), and noninvasive tension-time index of the respiratory muscles (TTImus) were studied in 80 NMD subjects (12...
August 23, 2016: Neuropediatrics
Abdallah Fayssoil, Arnaud Lazarus, Karim Wahbi, Adam Ogna, Olivier Nardi, Frederic Lofaso, Bernard Clair, David Orlikowski, Djillali Annane
BACKGROUND/OBJECTIVES: Muscular dystrophies are genetic muscle disorders, in which heart involvement and chronic respiratory impairment affect survival. Cardiac conduction disturbances require implantable cardiac pacemaker. Implantable defibrillators may also be necessary to prevent cardiac sudden death. The safety and risk of cardiac electronic devices' implantation are not known in patients with muscular dystrophy. We aimed to assess the risks related to cardiac implantable electronic devices (CIED) in muscular dystrophy patients ventilated by tracheostomy...
November 1, 2016: International Journal of Cardiology
Peter J Cowan
PURPOSE OF REVIEW: In this review, I will summarize recent developments in the use of the clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated 9 (Cas9) genome editing system for cell transplant applications, ranging from transplantation of corrected autologous patient stem cells to treat inherited diseases, to the tailoring of donor pigs for cell xenotransplantation. Rational engineering of the Cas9 nuclease to improve its specificity will also be discussed...
October 2016: Current Opinion in Organ Transplantation
Kristi Hylan, An-Duyen Nguyen Vu, Katherine Stammen
Stiff-person syndrome (SPS) is a neurologic disorder characterized by painful involuntary episodes of severe muscle rigidity affecting the axial muscles and extremities. Although the etiology of SPS is unknown, it is suspected to involve the synthesis of γ-aminobutyric acid (GABA). Symptoms of SPS are precipitated by sudden unexpected movements, noises, and stress. Additionally, SPS has been linked with various autoimmune disorders, including diabetes mellitus, thyroid disease, pernicious anemia, and certain cancers...
June 2016: AANA Journal
Anju A Bendon, Khristine A George, Davandra Patel
INTRODUCTION: Neuromuscular scoliosis is a known risk factor for postoperative complications after corrective spine surgery. Few studies have looked at the preoperative factors affecting postoperative complications in children with cerebral palsy. AIM: The aim of this study was to examine the factors that might influence postoperative course in patients with cerebral palsy undergoing spine surgery for scoliosis. METHODS: Nineteen case notes of children with cerebral palsy who had spine surgery (2008-2014) were reviewed retrospectively...
October 2016: Paediatric Anaesthesia
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
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
Josef Finsterer, Sinda Zarrouk-Mahjoub
INTRODUCTION: Weakness is one of the predominant clinical manifestations of neuromuscular disorders (NMDs), which strongly influences daily life, prognosis, and outcome of affected patients. One of the major therapeutic goals in NMD-patients is to completely resolve muscle weakness. Various treatment options are available and include physical therapy, electrotherapy, diet, drugs, avoidance or withdrawal of muscle-toxic and weakness-inducing agents, detoxification, stem-cell-therapy, plasma-exchange, respiratory therapy, or surgery...
July 13, 2016: Expert Review of Neurotherapeutics
Bruno Vie, Patricia Griffon, Audrey Bijoux, Julie Cadiere, Jean Paul Weber, Yves Jammes
The six-minute walk test (6MWT) is used to evaluate the ambulatory capacity of patients suffering from respiratory disorders, obesity or neuromuscular diseases. Our primary aim was to evaluate the effects of the 6MWT on the postural sway and the ankle plantar flexion forces in healthy subjects. We measured the ankle plantar flexion forces and the plantar contact area before and after a 6MWT in normal weight and overweight subjects with no history of respiratory, cardiac, and neuromuscular disorders. A post-6MWT sensation of bodily fatigue was evaluated by Multidimensional Fatigue Inventory (MFI) and Pichot fatigue scales...
September 2016: Gait & Posture
Lina Ghaloul-Gonzalez, Amy Goldstein, Catherine Walsh Vockley, Steven F Dobrowolski, Amy Biery, Afifa Irani, Jordan Ibarra, D Holmes Morton, Al-Walid Mohsen, Jerry Vockley
The Old Order Amish populations in the US are one of the Plain People groups and are descendants of the Swiss Anabaptist immigrants who came to North America in the early eighteenth century. They live in numerous small endogamous demes that have resulted in reduced genetic diversity along with a high prevalence of specific genetic disorders, many of them autosomal recessive. Mitochondrial respiratory chain deficiencies arising from mitochondrial or nuclear DNA mutations have not previously been reported in the Plain populations...
August 2016: Molecular Genetics and Metabolism
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
Gabriela Dumitrita Stanciu, Gheorghe Solcan
BACKGROUND: Acute Idiopathic Polyradiculoneuritis, an animal model for the axonal form of the Guillain - Barre Syndrome in humans and the acquired myasthenia gravis are different autoimmune disorders affecting the peripheral nerves and the neuromuscular junction, respectively. Both lead to muscle weakness and possible respiratory failure. The coexistence of these two entities combined in the same patient is rare in humans and, to our knowledge, the present case is the first reported in dogs...
2016: BMC Veterinary Research
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