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Lung and muscular dystrophy

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https://www.readbyqxmd.com/read/28594857/noninvasive-assessment-of-respiratory-muscle-strength-and-activity-in-myotonic-dystrophy
#1
Morgana de Araújo Evangelista, Fernando Augusto Lavezzo Dias, Mário Emílio Teixeira Dourado Júnior, George Carlos do Nascimento, Antonio Sarmento, Lucien Peroni Gualdi, Andrea Aliverti, Vanessa Resqueti, Guilherme Augusto de Freitas Fregonezi
OBJECTIVE: To evaluate sensitivity/specificity of the maximum relaxation rate (MRR) of inspiratory muscles, amplitude of electromyographic activity of the sternocleidomastoid (SCM), scalene (SCA), parasternal (2ndIS) and rectus abdominis (RA) muscles; lung function and respiratory muscle strength in subjects with Myotonic dystrophy type 1 (DM1) compared with healthy subjects. DESIGN AND METHODS: Quasi-experimental observational study with control group. MRR of inspiratory muscles, lung function and amplitude of the electromyographic activity of SCM, SCA, 2ndIS and RA muscles during maximum inspiratory pressure (PImax), maximum expiratory pressure (PEmax) and sniff nasal inspiratory pressure (SNIP) tests were assessed in eighteen DM1 subjects and eleven healthy...
2017: PloS One
https://www.readbyqxmd.com/read/28453658/androgen-receptor-agonists-increase-lean-mass-improve-cardiopulmonary-functions-and-extend-survival-in-preclinical-models-of-duchenne-muscular-dystrophy
#2
Suriyan Ponnusamy, Ryan D Sullivan, Dahui You, Nadeem Zafar, Chuan He Yang, Thirumagal Thiyagarajan, Daniel L Johnson, Maron L Barrett, Nikki J Koehler, Mayra Star, Erin J Stephenson, Dave Bridges, Stephania A Cormier, Lawrence M Pfeffer, Ramesh Narayanan
Duchenne muscular dystrophy (DMD) is a neuromuscular disease that predominantly affects boys as a result of mutation(s) in the dystrophin gene. DMD is characterized by musculoskeletal and cardiopulmonary complications, resulting in shorter life-span. Boys afflicted by DMD typically exhibit symptoms within 3-5 years of age and declining physical functions before attaining puberty. We hypothesized that rapidly deteriorating health of pre-pubertal boys with DMD could be due to diminished anabolic actions of androgens in muscle, and that intervention with an androgen receptor (AR) agonist will reverse musculoskeletal complications and extend survival...
July 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28451990/chest-wall-kinematics-using-triangular-cosserat-point-elements-in-healthy-and-neuromuscular-subjects
#3
Dana Solav, Henri Meric, M B Rubin, Didier Pradon, Frédéric Lofaso, Alon Wolf
Optoelectronic plethysmography (OEP) is a noninvasive method for assessing lung volume variations and the contributions of different anatomical compartments of the chest wall (CW) through measurements of the motion of markers attached to the CW surface. The present study proposes a new method for analyzing the local CW kinematics from OEP measurements based on the kinematics of triangular Cosserat point elements (TCPEs). 52 reflective markers were placed on the anterior CW to create a mesh of 78 triangles according to an anatomical model...
April 27, 2017: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/28416348/respiratory-function-in-facioscapulohumeral-muscular-dystrophy-1
#4
M Wohlgemuth, C G C Horlings, E L van der Kooi, H J Gilhuis, J C M Hendriks, S M van der Maarel, B G M van Engelen, Y F Heijdra, G W Padberg
To test the hypothesis that wheelchair dependency and (kypho-)scoliosis are risk factors for developing respiratory insufficiency in facioscapulohumeral muscular dystrophy, we examined 81 patients with facioscapulohumeral muscular dystrophy 1 of varying degrees of severity ranging from ambulatory patients to wheelchair-bound patients. We examined the patients neurologically and by conducting pulmonary function tests: Forced Vital Capacity, Forced Expiratory Volume in 1 second, and static maximal inspiratory and expiratory mouth pressures...
June 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28338488/respiratory-complications-management-and-treatments-for-neuromuscular-disease-in-children
#5
MyMy C Buu
PURPOSE OF REVIEW: To summarize current literature describing the respiratory complications of neuromuscular disease (NMD) and the effect of respiratory interventions and to explore new gene therapies for patients with NMD. RECENT FINDINGS: Measurements of respiratory function focus on vital capacity and maximal inspiratory and expiratory pressure and show decline over time. Management of respiratory complications includes lung volume recruitment, mechanical insufflation-exsufflation, chest physiotherapy and assisted ventilation...
June 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28288529/a-comparison-of-swallowing-dysfunction-in-becker-muscular-dystrophy-and-duchenne-muscular-dystrophy
#6
Yuka Yamada, Michiyuki Kawakami, Ayako Wada, Tomoyoshi Otsuka, Kaori Muraoka, Meigen Liu
PURPOSE: Swallowing dysfunction has been reported in Duchenne muscular dystrophy (DMD), but has not been studied in Becker muscular dystrophy (BMD). The aims of this study were to report the characteristics of swallowing dysfunction in BMD compared with DMD. MATERIALS AND METHODS: The study participants were 18 patients with BMD and 18 patients with DMD. All the patients were examined using videofluorography during swallowing of 5 mL of fluid. The penetration-aspiration scale (P-A scale) and the videofluorographic dysphagia scale (VDS) were used to evaluate dysphagia...
March 13, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28210319/physiology-of-respiratory-disturbances-in-muscular-dystrophies
#7
Antonella Lo Mauro, Andrea Aliverti
Muscular dystrophy is a group of inherited myopathies characterised by progressive skeletal muscle wasting, including of the respiratory muscles. Respiratory failure, i.e. when the respiratory system fails in its gas exchange functions, is a common feature in muscular dystrophy, being the main cause of death, and it is a consequence of lung failure, pump failure or a combination of the two. The former is due to recurrent aspiration, the latter to progressive weakness of respiratory muscles and an increase in the load against which they must contract...
December 2016: Breathe
https://www.readbyqxmd.com/read/28163851/a-systematic-review-of-the-relationship-between-dystemprament-sue-mizaj-and-treatments-and-management-of-diseases-ilaj-and-eslah-e-mizaj
#8
REVIEW
Sepide Miraj, Samira Alesaeidi, Sara Kiani
BACKGROUND: Temperament refers to four different humors differentiating in individuals and, as a result, proposes specific therapy for diseases as well as special types of management (avoidance). OBJECTIVE: The aim of this study was to overview the relationship between dystemprament and treatment and management of diseases. METHODS: A computerized search of published articles was performed using PubMed, Scopus, Web of Science, and Medline databases as well as local sources from 1965 to 2016...
December 2016: Electronic Physician
https://www.readbyqxmd.com/read/28108217/cdk3-is-a-major-target-of-mir-150-in-cell-proliferation-and-anti-cancer-effect
#9
Liang Wang, Yongyong Xi, Chengcao Sun, Feng Zhang, Heng Jiang, Qiqiang He, Dejia Li
MiR-150, a member of small non-coding RNAs, has been proven to dysregulate in different types of tumor and bear on carcinogenesis and cancer prognosis by regulating the expression of a series of gene including utrophin. Given that utrophin can compensate for dystrophin's absence and be regarded as a promising therapeutic target for Duchenne Muscular Dystrophy (DMD), we further detected the deep role of miR-150 in dystrophic muscle. Using a range of bioinformatic, molecular and cell biology techniques, we declared that miR-150 directly targets cyclin-dependent kinase 3 (CDK3) and leads to the regulation of CDK3 gene expression in both muscle-derived and non-muscle cells...
January 17, 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/27794082/impact-of-noninvasive-ventilation-on-lung-volumes-and-maximum-respiratory-pressures-in-duchenne-muscular-dystrophy
#10
Dante Brasil Santos, Isabelle Vaugier, Ghilas Boussaïd, David Orlikowski, Hélène Prigent, Frédéric Lofaso
BACKGROUND: Duchenne muscular dystrophy (DMD) is a sex-linked genetic disorder in which progressive impairment of skeletal muscle function eventually leads to severe respiratory failure requiring continuous noninvasive ventilation (NIV) at home. A current focus of debate is whether NIV may slow the decline in respiratory function or, on the contrary, worsen respiratory function when started early. Our objective here was to describe the effects of NIV on vital capacity (VC) and maximum respiratory pressures in DMD...
November 2016: Respiratory Care
https://www.readbyqxmd.com/read/27679799/prognostic-value-of-initial-assessment-of-residual-hypoventilation-using-nocturnal-capnography-in-mechanically-ventilated-neuromuscular-patients-a-5-year-follow-up-study
#11
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
https://www.readbyqxmd.com/read/27630091/active-lung-volume-recruitment-to-preserve-vital-capacity-in-duchenne-muscular-dystrophy
#12
Michael Chiou, John R Bach, Lavina Jethani, Michael F Gallagher
OBJECTIVE: To consider the effect of active lung volume recruitment ("air stacking") on rate of decline in vital capacity. DESIGN: Retrospective cross-sectional design. PATIENTS: People with Duchenne muscular dystrophy. METHODS: Vital capacity was measured at every patient visit and then graphed. Air stacking using volume-preset ventilation or manual resuscitator bag was introduced to all patients after their vital capacity plateaued (reached a lifetime maximum)...
January 19, 2017: Journal of Rehabilitation Medicine
https://www.readbyqxmd.com/read/27330957/a-tell-tale-handshake
#13
Praneet Wander, Adedapo Iluyomade, Paul Sanmartin, Akriti Gupta, Mary O'Sullivan
Myotonic dystrophy is a group of inherited disorders called muscular dystrophies. Clinical presentation of this disease is characterised by progressive muscle weakness with myotonia, cataracts, infertility (in males) and cardiac conduction defects. We present a case of a 35 year old male with lung abscess, later diagnosed to be a case of myotonic dystrophy. Lung abscess is an uncommon presentation of this disease and has never been reported before.
2016: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27239414/cancer-cachexia-causes-skeletal-muscle-damage-via-transient-receptor-potential-vanilloid-2-independent-mechanisms-unlike-muscular-dystrophy
#14
Yuko Iwata, Nobuyuki Suzuki, Hitomi Ohtake, Shinya Kamauchi, Naohiro Hashimoto, Tohru Kiyono, Shigeo Wakabayashi
BACKGROUND: Muscle wasting during cancer cachexia contributes to patient morbidity. Cachexia-induced muscle damage may be understood by comparing its symptoms with those of other skeletal muscle diseases, but currently available data are limited. METHODS: We modelled cancer cachexia in mice bearing Lewis lung carcinoma/colon adenocarcinoma and compared the associated muscle damage with that in a murine muscular dystrophy model (mdx mice). We measured biochemical and immunochemical parameters: amounts/localization of cytoskeletal proteins and/or Ca(2+) signalling proteins related to muscle function and abnormality...
June 2016: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/27165423/activation-of-the-wnt-%C3%AE-catenin-signaling-pathway-in-polymyositis-dermatomyositis-and-duchenne-muscular-dystrophy
#15
Fuchen Liu, Zonglai Liang, Jingwen Xu, Wei Li, Dandan Zhao, Yuying Zhao, Chuanzhu Yan
BACKGROUND AND PURPOSE: The wnt/β-catenin signaling pathway plays a critical role in embryonic development and adult-tissue homeostasis. Recent investigations implicate the importance of wnt/β-catenin signaling in normal wound healing and its sustained activation being associated with fibrogenesis. We investigated the immunolocalization and activation of wnt/β-catenin in polymyositis (PM), dermatomyositis (DM), and Duchenne muscular dystrophy (DMD). METHODS: Immunofluorescence staining and Western blot analysis of β-catenin were performed in muscle specimens from 6 PM, 8 DM, and 6 DMD subjects...
July 2016: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/27122162/valproic-acid-attenuates-skeletal-muscle-wasting-by-inhibiting-c-ebp%C3%AE-regulated-atrogin1-expression-in-cancer-cachexia
#16
Rulin Sun, Santao Zhang, Wenjun Hu, Xing Lu, Ning Lou, Zhende Yang, Shaoyong Chen, Xiaoping Zhang, Hongmei Yang
Muscle wasting is the hallmark of cancer cachexia and is associated with poor quality of life and increased mortality. Valproic acid (VPA), a histone deacetylase (HDAC) inhibitor, has important biological effects in the treatment of muscular dystrophy. To verify whether VPA could ameliorate muscle wasting induced by cancer cachexia, we explored the role of VPA in two cancer cachectic mouse models [induced by colon-26 (C26) adenocarcinoma or Lewis lung carcinoma (LLC)] and atrophied C2C12 myotubes [induced by C26 cell conditioned medium (CCM) or LLC cell conditioned medium (LCM)]...
July 1, 2016: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/27041384/causes-of-creatine-kinase-levels-greater-than-1000%C3%A2-iu-l-in-patients-referred-to-rheumatology
#17
David Leverenz, Oana Zaha, Leslie J Crofford, Cecilia P Chung
Patients with severely elevated creatine kinase (CK) concentrations are commonly referred to rheumatologists to evaluate for the presence of an idiopathic inflammatory myopathy (IIM). However, no studies have evaluated the frequency with which IIMs are encountered in this clinical scenario. The Vanderbilt Synthetic Derivative, a de-identified copy of over 2 million patient records, was searched to identify adult patients with a CK greater than 1000 IU/L who had been evaluated by a rheumatologist. Each patient was assigned a diagnosis using a pre-determined algorithm...
June 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/26789431/-respiratory-muscle-training-state-of-the-art
#18
REVIEW
O Göhl, D J Walker, S Walterspacher, D Langer, C M Spengler, T Wanke, M Petrovic, R-H Zwick, S Stieglitz, R Glöckl, D Dellweg, H-J Kabitz
Specific respiratory muscle training (IMT) improves the function of the inspiratory muscles. According to literature and clinical experience, there are 3 established methods: 1.) resistive load 2.) threshold load and 3.) normocapnic hyperpnea. Each training method and the associated devices have specific characteristics. Setting up an IMT should start with specific diagnostics of respiratory muscle function and be followed by detailed individual introduction to training. The aim of this review is to take a closer look at the different training methods for the most relevant indications and to discuss these results in the context of current literature...
January 2016: Pneumologie
https://www.readbyqxmd.com/read/26773840/body-weight-supported-training-in-becker-and-limb-girdle-2i-muscular-dystrophy
#19
Bente R Jensen, Martin P Berthelsen, Edith Husu, Sofie B Christensen, Kira P Prahm, John Vissing
INTRODUCTION: We studied the functional effects of combined strength and aerobic anti-gravity training in severely affected patients with Becker and Limb-Girdle muscular dystrophies. METHODS: Eight patients performed 10-week progressive combined strength (squats, calf raises, lunges) and aerobic (walk/run, jogging in place or high knee-lift) training 3 times/week in a lower-body positive pressure environment. Closed-kinetic-chain leg muscle strength, isometric knee strength, rate of force development (RFD), and reaction time were evaluated...
August 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/26679108/anti-fibrotic-effect-of-pirfenidone-in-muscle-derived-fibroblasts-from-duchenne-muscular-dystrophy-patients
#20
Simona Zanotti, Cinzia Bragato, Andrea Zucchella, Lorenzo Maggi, Renato Mantegazza, Lucia Morandi, Marina Mora
AIMS: Tissue fibrosis, characterized by excessive deposition of extracellular matrix proteins, is the end point of diseases affecting the kidney, bladder, liver, lung, gut, skin, heart and muscle. In Duchenne muscular dystrophy (DMD), connective fibrotic tissue progressively substitutes muscle fibers. So far no specific pharmacological treatment is available for muscle fibrosis. Among promising anti-fibrotic molecules, pirfenidone has shown anti-fibrotic and anti-inflammatory activity in animal and cell models, and has already been employed in clinical trials...
January 15, 2016: Life Sciences
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