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https://www.readbyqxmd.com/read/28637335/astrocyte-produced-mir-146a-as-a-mediator-of-motor-neuron-loss-in-spinal-muscular-atrophy
#1
Samantha L Sison, Teresa N Patitucci, Emily R Seminary, Eric Villalon, Christian L Lorson, Allison D Ebert
Spinal muscular atrophy (SMA), the leading genetic cause of infant mortality, is caused by loss of the survival motor neuron-1 (SMN1) gene, which leads to motor neuron loss, muscle atrophy, respiratory distress, and death. Motor neurons exhibit the most profound loss, but the mechanisms underlying disease pathogenesis are not fully understood. Recent evidence suggests that motor neuron extrinsic influences, such as those arising from astrocytes, contribute to motor neuron malfunction and loss. Here we investigated both loss-of-function and toxic gain-of-function astrocyte mechanisms that could play a role in SMA pathology...
June 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28636407/pulmonary-endpoints-in-duchenne-muscular-dystrophy-a-workshop-summary
#2
Jonathan Finder, Oscar Henry Mayer, Daniel Sheehan, Hemant Sawnani, R Ted Abresch, Joshua Benditt, David J Birnkrant, Tina Duong, Erik Henricson, Kathi Kinnett, Craig M McDonald, Anne M Connolly
Development of novel therapeutics for treatment of Duchenne muscular dystrophy (DMD) has led to clinical trials that include pulmonary endpoints that allow assessment of respiratory muscle status, especially in non-ambulatory subjects. Parent Project Muscular Dystrophy (PPMD) convened a workshop in Bethesda, MD from April 14-15, 2016 to summarize published respiratory data in DMD and give guidance to clinical researchers assessing the effect of interventions on pulmonary outcomes in DMD.
June 21, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28635954/phenotypic-extremes-of-bicd2-opathies-from-lethal-congenital-muscular-atrophy-with-arthrogryposis-to-asymptomatic-with-subclinical-features
#3
Markus Storbeck, Beate Horsberg Eriksen, Andreas Unger, Irmgard Hölker, Ingvild Aukrust, Lilian A Martínez-Carrera, Wolfgang A Linke, Andreas Ferbert, Raoul Heller, Matthias Vorgerd, Gunnar Houge, Brunhilde Wirth
Heterozygous variants in BICD cargo adapter 2 (BICD2) cause autosomal dominant spinal muscular atrophy, lower extremity-predominant 2 (SMALED2). The disease is usually characterized by a benign or slowly progressive, congenital or early onset muscle weakness and atrophy that mainly affects the lower extremities, although some affected individuals show involvement of the arms and the shoulder girdle. Here we report unusual extremes of BICD2-related diseases: A severe form of congenital muscular atrophy with arthrogryposis multiplex, respiratory insufficiency and lethality within four months...
June 21, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28635482/diagnosis-and-management-of-myasthenia-gravis
#4
Christopher Barber
Myasthenia gravis is a rare long-term neurological condition that is characterised by fluctuating skeletal muscle weakness and fatigue, as well as respiratory difficulties. It is both an acquired autoimmune disease and a chronic neuromuscular disorder. Because of its rarity, myasthenia gravis is relatively unknown and may be unfamiliar to many nurses. While there are various types of myasthenia, this article focuses on myasthenia gravis, exploring its symptoms, diagnosis and treatment, and examining the nurse's role in managing the condition...
June 21, 2017: Nursing Standard
https://www.readbyqxmd.com/read/28635361/role-of-licochalcone-a-in-vegf-induced-proliferation-of-human-airway-smooth-muscle-cells-implications-for-asthma
#5
Sung-Ho Kim, Qing-Mei Pei, Ping Jiang, Min Yang, Xue-Jiao Qian, Jiang-Bo Liu
Asthma is a chronic respiratory disease characterized by reversible airway obstruction with persistent airway inflammation and airway remodeling, which is associated with increased airway smooth muscle (ASM) mass. Licochalcone A is the predominant characteristic chalcone in licorice root. We found that licochalcone A inhibited vascular endothelial growth factor (VEGF)-induced ASM cell proliferation and induced cell cycle arrest. Additionally, VEGF-induced ASM cell proliferation was suppressed via inhibition of extracellular signal-regulated kinase 1/2 (ERK1/2) activity, but not that of Akt...
February 2017: Growth Factors
https://www.readbyqxmd.com/read/28635194/volitional-rehabilitative-assessments-in-patients-admitted-in-a-post-intensive-care-step-down-unit-a-feasibility-study
#6
Mara Paneroni, Francesco D' Abrosca, Georges Fokom, Laura Comini, Michele Vitacca
A high variability in functional tests and activities used during the pulmonary rehabilitation has been observed in post-intensive care unit (ICU) patients, and the best battery of tests to adopt has not been described yet. We tested in patients admitted in a post-ICU Step Down Unit the ability to perform the more frequent functional volitional tests. The relations of each single volitional test with general disability and dyspnea at discharge were also evaluated. Ten volitional tests including: bedside spirometry test (ST: FEV1%, FVC%), maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), Peak Expiratory Flow during Cough (PCEF), Quadriceps Muscle Strength (QMS), latissimus Dorsi and teres Major Strength (DMS), Brachial biceps Muscle Strength (BMS), effort tolerance measured by sit-to-stand test, Takahashi test and 6-Min Walking Test (6MWT), were evaluated in post-ICU patients at entry and discharge from in-hospital rehabilitation...
May 18, 2017: Monaldi Archives for Chest Disease, Archivio Monaldi Per le Malattie del Torace
https://www.readbyqxmd.com/read/28635190/characteristics-of-structured-physical-training-currently-provided-in-cardiac-patients-insights-from-the-exercise-training-in-cardiac-rehabilitation-etcr-italian-survey
#7
Marco Ambrosetti, Patrick Doherty, Pompilio Faggiano, Ugo Corrà, Carlo Vigorito, Dominique Hansen, Patrizio Sarto, Ana Abreu, Roberto F E Pedretti
BACKGROUND:   Uncertainty exists about current delivery levels of exercise training (ET) during Cardiac Rehabilitation (CR) programmes. AIM OF THE STUDY: To evaluate ET modalities in the real world of CR facilities in Italy. METHODS: This was an observational survey of aggregate data, collected from CR facilities on a voluntary basis. Snapshots of a single working day at a local site were made, in terms of characteristics of patients and ET programmes delivered...
May 18, 2017: Monaldi Archives for Chest Disease, Archivio Monaldi Per le Malattie del Torace
https://www.readbyqxmd.com/read/28632833/-tetanus-after-cat-scratch-and-bites-in-a-previously-immunized-patient
#8
Alberto Fica, Daniela Gaínza, Pablo Ortigosa
Tetanus is declining due to vaccination, professional labor management and appropriate wound care. Tetanus cases have been reported despite immunization. We report the case of a previously healthy 21 years old female patient that presented a mild generalized tetanus requiring admission after mild and recurrent cat scratch and bites. She had received six vaccine shots during childhood, and a booster dose five years earlier after a rabbit bite. Symptoms appeared seven weeks after the last contact, and included headache, muscle spasms and mild opisthotonus...
April 2017: Revista Chilena de Infectología: órgano Oficial de la Sociedad Chilena de Infectología
https://www.readbyqxmd.com/read/28631296/hydrogen-sulphide-as-a-signaling-molecule-regulating-physiopathological-processes-in-gastrointestinal-motility
#9
REVIEW
M Jimenez, V Gil, M Martinez-Cutillas, N Mañé, D Gallego
The biology of H2 S is an emerging area of research since several biological functions have been recently attributed to this gaseous molecule in a vast number of regions including the cardiovascular, urogenital, respiratory, digestive and central nervous system. H2 S exerts anti-inflammatory effects and can be considered an endogenous mediator with potential effects on gastrointestinal motility. During the last years, our research group has investigated the role of H2 S as a regulator of gastrointestinal motility using both animal and human tissues...
June 20, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28627353/-ultrasonographic-evaluation-of-diaphragm-structure-and-function
#10
Feifei Wang, Xiaoping Zhu, Shaolin Ma
Diaphragm is the principal respiratory muscle. Diaphragm dysfunction resulting from mechanical ventilation, chronic cardiopulmonary disease, medicine, cardio-thoracic surgery, septic shock and chronic malnutrition predisposes to weaning failure from mechanical ventilation and prolongs intensive care unit (ICU) stay. Traditionally, the method of assessment of diaphragm structure and function was invasive, lacking specific, and was difficult to be used bedside. A number of recent studies have been suggested that ultrasonographic evaluation has been proposed as an accurate, safe and noninvasive method of quantification of diaphragm structure and function...
March 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/28626633/mediastinal-hemangioendothelioma-case-report-and-review-of-the-literature
#11
Davide Patrini, Laura Scolamiero, Reena Khiroya, David Lawrence, Elaine Borg, Martin Hayward, Nikolaos Panagiotopoulos
BACKGROUND: Epithelioid haemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that can arise in the lung, liver, soft tissues or, less commonly, bone. Due to its low prevalence of less than one in a million and its non-specific clinical features, EHE is often misdiagnosed and managed inappropriately. Here we discuss the case of a 58 year-old gentleman with mediastinal EHE and review existing literature on pulmonary EHE (PEH). CASE HISTORY: A 58 year-old gentleman presented to our outpatient Clinic with chest discomfort and palpitations...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28626302/the-immediate-effect-of-diaphragm-taping-with-breathing-exercise-on-muscle-tone-and-stiffness-of-respiratory-muscles-and-spo2-in-stroke-patient
#12
Joong-San Wang, Kyun-Hee Cho, Shin-Jun Park
[Purpose] This study aimed to examine the immediate effects of diaphragm taping with breathing exercise on the tone and stiffness in the respiratory muscles of patient with stroke. [Subjects and Methods] A total of 28 subjects, 14 in the diaphragm taping with breathing exercise group and 14 in the breathing exercise group, were administered respective intervention methods. Subsequently, the muscle tone and stiffness in upper trapezius, scalene, external oblique abdominal and ractus abdominis muscle of both the respiratory muscles were measured...
June 2017: Journal of Physical Therapy Science
https://www.readbyqxmd.com/read/28625735/analgesic-and-physiological-effect-of-electroacupuncture-combined-with-epidural-lidocaine-in-goats
#13
Lu-Ying Cui, Ni-Ni Guo, Yu-Lin Li, Meng Li, Ming-Xing Ding
OBJECTIVE: To investigate physiological and antinociceptive effects of electroacupuncture (EA) with lidocaine epidural nerve block in goats. STUDY DESIGN: Prospective experimental trial. ANIMALS: Forty-eight hybrid male goats weighing 27 ± 2 kg. METHODS: The goats were randomly assigned to six groups: L2.2, epidural lidocaine (2.2 mg kg(-1)); L4.4, epidural lidocaine (4.4 mg kg(-1)); EA; EA-L1.1, EA with epidural lidocaine (1...
January 12, 2017: Veterinary Anaesthesia and Analgesia
https://www.readbyqxmd.com/read/28625661/resting-limb-muscle-perfusion-during-inspiratory-muscle-loading-in-hypoxia-and-normoxia
#14
Hannes Klenze, Thomas Christian Köhler, Franziska Farquharson, Stephan Walterspacher, Daniel Duerschmied, Kai Roecker, Hans-Joachim Kabitz, David Johannes Walker
INTRODUCTION: Fatiguing of respiratory muscles reduces peripheral muscle perfusion. Further, acute hypoxia enhances respiratory muscle fatigue. This study investigated the effects of inspiratory muscle loading (IML) on resting locomotor muscle perfusion in hypoxia compared to normoxia. METHODS: Ten subjects completed two study days of fatiguing IML (blinded, randomized) in normobaric hypoxia (targeted oxygen saturation 80%) and normoxia, respectively. Contrast-enhanced ultrasound (CEUS) of the gastrocnemius muscle and popliteal doppler ultrasonography were used to monitor muscle perfusion...
June 15, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28624507/effect-of-pyridostigmine-on-in-vivo-and-in-vitro-respiratory-muscle-of-mdx-mice
#15
Gabriela de Cássia Sousa Amancio, Andrea Grabe-Guimarães, Dridi Haikel, Johan Moreau, Neila Marcia Silva Barcellos, Alain Lacampagne, Stefan Matecki, Olivier Cazorla
The current work was conducted to verify the contribution of neuromuscular transmission defects at the neuromuscular junction to Duchenne Muscular Dystrophy disease progression and respiratory dysfunction. We tested pyridostigmine and pyridostigmine encapsulated in liposomes (liposomal PYR), an acetylcholinesterase inhibitor to improve muscular contraction on respiratory muscle function in mdx mice at different ages. We evaluated in vivo with the whole-body plethysmography, the ventilatory response to hypercapnia, and measured in vitro diaphragm strength in each group...
June 14, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28623885/monitoring-the-electric-activity-of-the-diaphragm-during-noninvasive-positive-pressure-ventilation-a-case-report
#16
Fabia Diniz-Silva, Anna Miethke-Morais, Adriano M Alencar, Henrique T Moriya, Pedro Caruso, Eduardo L V Costa, Juliana C Ferreira
BACKGROUND: In patients with post-extubation respiratory distress, delayed reintubation may worsen clinical outcomes. Objective measures of extubation failure at the bedside are lacking, therefore clinical parameters are currently used to guide the need of reintubation. Electrical activity of the diaphragm (EAdi) provides clinicians with valuable, objective information about respiratory drive and could be used to monitor respiratory effort. CASE PRESENTATION: We describe the case of a patient with Chronic Obstructive Pulmonary Disease (COPD), from whom we recorded EAdi during four different ventilatory conditions: 1) invasive mechanical ventilation, 2) spontaneous breathing trial (SBT), 3) unassisted spontaneous breathing, and 4) Noninvasive Positive Pressure Ventilation (NPPV)...
June 17, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28623106/the-role-of-physiotherapy-in-patients-undergoing-pulmonary-surgery-for-lung-cancer-a-literature-review
#17
F Kendall, P Abreu, P Pinho, J Oliveira, P Bastos
This review aims to appraise the role of physiotherapy care in patients submitted to pulmonary surgery, in preoperative, perioperative, and postoperative phases. Pulmonary surgery is the gold standard treatment for patients with lung cancer if it is completely resectable. However, the major impairments and complications induced by surgery are well known. Physiotherapy has been regularly used both in the preparation of the surgical candidates; in their functional recovery in the immediate postoperative period, and in the medium/long term but there is a lack of concise evidence-based recommendations...
June 13, 2017: Revista Portuguesa de Pneumologia
https://www.readbyqxmd.com/read/28622081/effects-of-inspiratory-muscle-training-in-professional-women-football-players-a-randomized-sham-controlled-trial
#18
Bruno Archiza, Daniela Kuguimoto Andaku, Flávia Cristina Rossi Caruso, José Carlos Bonjorno, Cláudio Ricardo de Oliveira, Paula Angélica Ricci, André Capaldo do Amaral, Stela Márcia Mattiello, Cleiton Augusto Libardi, Shane A Phillips, Ross Arena, Audrey Borghi-Silva
This study was conducted to determine the effects of inspiratory muscle training (IMT) on respiratory and peripheral muscles oxygenation during a maximal exercise tolerance test and on repeated-sprint ability (RSA) performance in professional women football players. Eighteen athletes were randomly assigned to one of the following groups: SHAM (n = 8) or IMT (n = 10). After a maximal incremental exercise test, all participants performed (on a different day) a time-to-exhaustion (Tlim) test. Peripheral and respiratory muscles oxygenation by near-infrared spectroscopy, breath-by-breath ventilatory and metabolic variables, and blood lactate concentration were measured...
June 16, 2017: Journal of Sports Sciences
https://www.readbyqxmd.com/read/28622013/acetazolamide-usage-in-severe-chronic-obstructive-pulmonary-disease-pros-and-cons
#19
Rosemary Adamson, Erik R Swenson
Acetazolamide is a carbonic anhydrase (CA) inhibitor sometimes used as a respiratory stimulant for patients with COPD with the goal of improving oxygenation, reducing carbon dioxide retention and aiding liberation from mechanical ventilation and/or attempting to correct a metabolic alkalosis. However, the net effect of CA inhibition is multifactorial and complex since CA is inhibited in many tissues that may negatively affect the patient with lung disease. The full impact of acetazolamide and other CA inhibitors depends critically on dosing, age, and pulmonary, renal, hepatic, hematological, and respiratory muscle function and reserves...
June 16, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28618190/relation-between-upper-limb-muscle-strength-with-exercise-capacity-quality-of-life-and-dyspnea-in-patients-with-severe-chronic-obstructive-pulmonary-disease
#20
Dicle Kaymaz, İpek Çaylı Candemir, Pınar Ergün, Neşe Demir, Filiz Taşdemir, Pervin Demir
OBJECTIVES: In chronic obstructive pulmonary disease (COPD), skeletal muscle weakness is characterized by reduced muscle strength, reduced muscle endurance, and the presence of muscle fatigue, especially in the lower limbs; there has been little research into upper limb skeletal muscles. In this study we aimed to investigate the relation of upper limb muscle strength with pulmonary function, exercise capacity, quality of life, and dyspnea sensation. METHODS: Eigthy-eight patients (89...
June 15, 2017: Clinical Respiratory Journal
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