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https://www.readbyqxmd.com/read/29145830/differences-in-vertebral-morphology-around-the-apical-vertebrae-between-neuromuscular-scoliosis-and-idiopathic-scoliosis-in-skeletally-immature-patients-a-three-dimensional-morphometric-analysis
#1
Takahiro Makino, Yusuke Sakai, Masafumi Kashii, Shota Takenaka, Kazuomi Sugamoto, Hideki Yoshikawa, Takashi Kaito
BACKGROUND: Recent morphological analyses of vertebrae in patients with scoliosis have revealed three-dimensional (3D) deformities in the vertebral bodies. However, it remains controversial whether these deformities are secondary changes caused by asymmetrical vertebral loading or primary changes caused by aberrant asymmetrical vertebral growth. Furthermore, the difference in vertebral morphology between scoliosis with different pathogeneses remains unclear. This study was aimed to investigate the difference in the coronal asymmetry of vertebral bodies between neuromuscular scoliosis (NS) in Duchenne muscular dystrophy (DMD) and idiopathic scoliosis (IS) using in vivo 3D analysis...
November 16, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/29130550/-1-h-nmrs-of-carnosine-combined-with-31-p-nmrs-to-better-characterize-skeletal-muscle-ph-dysregulation-in-duchenne-muscular-dystrophy
#2
Harmen Reyngoudt, Suna Turk, Pierre G Carlier
In recent years, quantitative nuclear magnetic resonance imaging and spectroscopy (NMRI and NMRS) have been used more systematically as outcome measures in natural history and clinical trial studies for Duchenne muscular dystrophy (DMD). Whereas most of these studies have emphasized the evaluation of the fat fraction as an assessment for disease severity, less focus has been placed on metabolic indices measured by NMRS. (31) P NMRS in DMD reveals an alkaline inorganic phosphate (Pi ) pool, originating from either leaky dystrophic myocytes or an increased interstitial space...
November 12, 2017: NMR in Biomedicine
https://www.readbyqxmd.com/read/29127875/derivation-of-the-duchenne-muscular-dystrophy-patient-derived-induced-pluripotent-stem-cell-line-lacking-dmd-exons-49-and-50-ccmi001dmd-a-3-%C3%A2-49-%C3%A2-50
#3
Gabriella Spaltro, Vera Vigorelli, Federica Casalnuovo, Pietro Spinelli, Elisa Castiglioni, Davide Rovina, Stefania Paganini, Marina Di Segni, Patrizia Nigro, Cristina Gervasini, Giulio Pompilio, Aoife Gowran
Duchenne muscular dystrophy (DMD) is caused by abnormalities in the dystrophin gene and is clinically characterised by childhood muscle degeneration and cardiomyopathy. We produced an induced pluripotent stem cell line from a DMD patient's dermal fibroblasts by electroporation with episomal vectors containing: hL-MYC, hLIN28, hSOX2, hKLF4, hOCT3/4. The resultant DMD iPSC line (CCMi001DMD-A-3) displayed iPSC morphology, expressed pluripotency markers, possessed trilineage differentiation potential and was karyotypically normal...
October 28, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29125504/clinical-outcomes-in-duchenne-muscular-dystrophy-a-study-of-5345-patients-from-the-treat-nmd-dmd-global-database
#4
Zaïda Koeks, Catherine L Bladen, David Salgado, Erik van Zwet, Oksana Pogoryelova, Grace McMacken, Soledad Monges, Maria Foncuberta, Kyriaki Kekou, Konstantina Kosma, Hugh Dawkins, Leanne Lamont, Matthew I Bellgard, Anna J Roy, Teodora Chamova, Velina Guergueltcheva, Sophelia Chan, Lawrence Korngut, Craig Campbell, Yi Dai, Jen Wang, Nina Barišić, Petr Brabec, Jaana Lähdetie, Maggie C Walter, Olivia Schreiber-Katz, Veronika Karcagi, Marta Garami, Agnes Herczegfalvi, Venkatarman Viswanathan, Farhad Bayat, Filippo Buccella, Alessandra Ferlini, En Kimura, Janneke C van den Bergen, Miriam Rodrigues, Richard Roxburgh, Anna Lusakowska, Anna Kostera-Pruszczyk, Rosário Santos, Elena Neagu, Svetlana Artemieva, Vedrana Milic Rasic, Dina Vojinovic, Manuel Posada, Clemens Bloetzer, Andrea Klein, Jordi Díaz-Manera, Eduard Gallardo, A Ayşe Karaduman, Tunca Oznur, Haluk Topaloğlu, Rasha El Sherif, Angela Stringer, Andriy V Shatillo, Ann S Martin, Holly L Peay, Jan Kirschner, Kevin M Flanigan, Volker Straub, Kate Bushby, Christophe Béroud, Jan J Verschuuren, Hanns Lochmüller
BACKGROUND: Recent short-term clinical trials in patients with Duchenne Muscular Dystrophy (DMD) have indicated greater disease variability in terms of progression than expected. In addition, as average life-expectancy increases, reliable data is required on clinical progression in the older DMD population. OBJECTIVE: To determine the effects of corticosteroids on major clinical outcomes of DMD in a large multinational cohort of genetically confirmed DMD patients...
November 10, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/29124971/motor-learning-from-virtual-reality-to-natural-environments-in-individuals-with-duchenne-muscular-dystrophy
#5
Virgínia Helena Quadrado, Talita Dias da Silva, Francis Meire Favero, James Tonks, Thais Massetti, Carlos Bandeira de Mello Monteiro
PURPOSE: To examine whether performance improvements in the virtual environment generalize to the natural environment. STUDY DESIGN: we had 64 individuals, 32 of which were individuals with DMD and 32 were typically developing individuals. METHODS: The groups practiced two coincidence timing tasks. In the more tangible button-press task, the individuals were required to 'intercept' a falling virtual object at the moment it reached the interception point by pressing a key on the computer...
November 10, 2017: Disability and Rehabilitation. Assistive Technology
https://www.readbyqxmd.com/read/29123500/efficacy-and-the-safety-of-granulocyte-colony-stimulating-factor-treatment-in-patients-with-muscular-dystrophy-a-non-randomized-clinical-trial
#6
Dorota Sienkiewicz, Wojciech Kułak, Bożena Okurowska-Zawada, Grażyna Paszko-Patej, Janusz Wojtkowski, Karolina Sochoń, Anna Kalinowska, Kamila Okulczyk, Jerzy Sienkiewicz, Edward McEachern
Introduction: The current standard treatment for patients with Duchenne muscular dystrophy (DMD) involves corticosteroids. Granulocyte colony-stimulating factor (G-CSF) induces the proliferation of satellite cells and myoblasts and, in turn, muscle regeneration. Beneficial effects of G-CSF were also described for skeletal muscle disorders. Aim: We assessed the safety and effects of using G-CSF to promote muscle strength in patients with DMD. Materials and methods: Inclusion criteria were as follows: patients aged 5-15 years with diagnosed with DMD confirmed by genetic test or biopsy...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29122570/relationships-between-facial-divergence-and-dmd-parameters
#7
Abdelali Halimi, Hicham Benyahia, Mohamed-Faouzi Azeroual, Loubna Bahije, Fatima Zaoui
INTRODUCTION: The aim of this study was to examine the relationship between facial divergence and the parameters of dentomaxillary discrepancy (DMD), in particular crowding, the curve of Spee and the position of the incisors in the sagittal dimension. MATERIAL AND METHODS: A total of 90 young adult patients was selected from among the Moroccan orthodontic population attending the dentofacial orthopedic department and satisfying the following inclusion criteria: complete permanent dentition and a skeletal class I pattern with no previous orthodontic treatment, no crossbite, no periodontal disease, no mandibular asymmetry and no condylodiscal disunion...
November 6, 2017: International Orthodontics
https://www.readbyqxmd.com/read/29121992/a-mouse-anti-myostatin-antibody-increases-muscle-mass-and-improves-muscle-strength-and-contractility-in-the-mdx-mouse-model-of-duchenne-muscular-dystrophy-and-its-humanized-equivalent-domagrozumab-pf-06252616-increases-muscle-volume-in-cynomolgus-monkeys
#8
Michael St Andre, Mark Johnson, Prashant N Bansal, Jeremy Wellen, Andrew Robertson, Alan Opsahl, Peter M Burch, Peter Bialek, Carl Morris, Jane Owens
BACKGROUND: The treatments currently approved for Duchenne muscular dystrophy (DMD), a progressive skeletal muscle wasting disease, address the needs of only a small proportion of patients resulting in an urgent need for therapies that benefit all patients regardless of the underlying mutation. Myostatin is a member of the transforming growth factor-β (TGF-β) family of ligands and is a negative regulator of skeletal muscle mass. Loss of myostatin has been shown to increase muscle mass and improve muscle function in both normal and dystrophic mice...
November 9, 2017: Skeletal Muscle
https://www.readbyqxmd.com/read/29120231/impact-of-clear-corneal-incision-morphology-on-incision-site-descemet-membrane-detachment-in-conventional-and-femtosecond-laser-assisted-phacoemulsification
#9
Jeewan S Titiyal, Manpreet Kaur, Priyanka Ramesh, Pooja Shah, Ruchita Falera, Lalit M S Bageshwar, Ashutosh Kinkar, Namrata Sharma
PURPOSE: To assess intraoperative morphology of clear corneal incisions (CCI) and its impact on incision-site descemet membrane detachment (DMD) in conventional phacoemulsification and femtosecond laser-assisted cataract surgery (FLACS). METHODS: Prospective comparative study of 129 eyes that underwent either conventional phacoemulsification (Group I, n = 77) or FLACS (Group II, n = 52) was undertaken at an apex tertiary care ophthalmic setup. In group I, a 2...
November 9, 2017: Current Eye Research
https://www.readbyqxmd.com/read/29119933/pharmacological-and-non-pharmacological-therapies-of-cognitive-impairment-in-multiple-sclerosis
#10
Elzbieta Miller, Agnieszka Morel, Justyna Redlicka, Igor Miller, Joanna Saluk
Cognitive impairment is one of the most important clinical features of neurodegenerative disorders including multiple sclerosis (MS). Conducted research shows that up to 65 percent of MS patients have cognitive deficits such as episodic memory, sustained attention, reduced verbal fluency; however, the cognitive MS domain is information processing speed. It is the first syndrome of cognitive dysfunction and the most widely affected in MS. Occasionally these impairments occur even before the appearance of physical symptoms...
November 9, 2017: Current Neuropharmacology
https://www.readbyqxmd.com/read/29111824/binary-complementary-filters-for-compressive-raman-spectroscopy
#11
Owen G Rehrauer, Vu C Dinh, Bharat R Mankani, Gregery T Buzzard, Bradley J Lucier, Dor Ben-Amotz
The previously described optimized binary compressive detection (OB-CD) strategy enables fast hyperspectral Raman (and fluorescence) spectroscopic analysis of systems containing two or more chemical components. However, each OB-CD filter collects only a fraction of the scattered photons and the remainder of the photons are lost. Here, we present a refinement of OB-CD, the OB-CD2 strategy, in which all of the collected Raman photons are detected using a pair of complementary binary optical filters that direct photons of different colors to two photon counting detectors...
January 1, 2017: Applied Spectroscopy
https://www.readbyqxmd.com/read/29103911/cyclic-peptides-to-improve-delivery-and-exon-skipping-of-antisense-oligonucleotides-in-a-mouse-model-for-duchenne-muscular-dystrophy
#12
Silvana M G Jirka, Peter A C 't Hoen, Valeriano Diaz Parillas, Christa L Tanganyika-de Winter, Ruurd C Verheul, Begona Aguilera, Peter C de Visser, Annemieke M Aartsma-Rus
Duchenne muscular dystrophy (DMD) is a severe, progressive muscle wasting disorder caused by reading frame disrupting mutations in the DMD gene. Exon skipping is a therapeutic approach for DMD. It employs antisense oligonucleotides (AONs) to restore the disrupted open reading frame, allowing the production of shorter, but partly functional dystrophin protein as seen in less severely affected Becker muscular dystrophy patients. To be effective, AONs need to be delivered and effectively taken up by the target cells, which can be accomplished by the conjugation of tissue-homing peptides...
October 12, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/29102745/gold-nanostar-mediated-neural-activity-control-using-plasmonic-photothermal-effects
#13
Jee Woong Lee, Hyunjun Jung, Hui Hun Cho, Jung Heon Lee, Yoonkey Nam
Nanomaterials have emerged as an essential tool for the understanding of cellular level mechanism in the fields of biology and medical science. Recently, researchers have been studying the regulation of neuronal activity using plasmonic nanoparticles and light, and it has been reported that photothermal effects could lead to both excitation and inhibition of neuronal cells. So far, only a few photothermal transducers have been applied to modulate neural activity. In this paper, we synthesized biocompatible gold nanostars (AuNSs) which generate heat by absorbing near-infrared (NIR) light...
October 27, 2017: Biomaterials
https://www.readbyqxmd.com/read/29099477/parents-reactions-to-the-diagnosis-of-duchenne-muscular-dystrophy-associations-between-resolution-family-functioning-and-child-behavior-problems
#14
Roberto Baiocco, Paola Castelli Gattinara, Giorgia Cioccetti, Salvatore Ioverno
BACKGROUND: Duchenne muscular dystrophy (DMD) is the most frequent inherited form of muscular dystrophy during childhood. DMD is a severe and progressive disease. Children initially have no symptoms, but the diagnosis is often delayed until the child is about 5 years old. PURPOSE: Although few studies have addressed parent reactions to DMD, parental reactions to other serious childhood conditions have been documented. This study aims to understand the resolution styles that parents use in the context of their children with DMD...
December 2017: Journal of Nursing Research: JNR
https://www.readbyqxmd.com/read/29097503/ryanodine-channel-complex-stabilizer-compound-s48168-arm210-as-a-disease-modifier-in-dystrophin-deficient-mdx-mice-proof-of-concept-study-and-independent-validation-of-efficacy
#15
Roberta Francesca Capogrosso, Paola Mantuano, Kitipong Uaesoontrachoon, Anna Cozzoli, Arcangela Giustino, Todd Dow, Sadish Srinivassane, Marina Filipovic, Christina Bell, Jack Vandermeulen, Ada Maria Massari, Michela De Bellis, Elena Conte, Sabata Pierno, Giulia Maria Camerino, Antonella Liantonio, Kanneboyina Nagaraju, Annamaria De Luca
Muscle fibers lacking dystrophin undergo a long-term alteration of Ca(2+) homeostasis, partially caused by a leaky Ca(2+) release ryanodine (RyR) channel. S48168/ARM210, an RyR calcium release channel stabilizer (a Rycal compound), is expected to enhance the rebinding of calstabin to the RyR channel complex and possibly alleviate the pathologic Ca(2+) leakage in dystrophin-deficient skeletal and cardiac muscle. This study systematically investigated the effect of S48168/ARM210 on the phenotype of mdx mice by means of a first proof-of-concept, short (4 wk), phase 1 treatment, followed by a 12 wk treatment (phase 2) performed in parallel by 2 independent laboratories...
November 2, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29096367/electrochemical-immunosensors-for-the-detection-of-survival-motor-neuron-smn-protein-using-different-carbon-nanomaterials-modified-electrodes
#16
Shimaa Eissa, Nawal Alshehri, Anas M Abdel Rahman, Majed Dasouki, Khalid M Abu Salah, Mohammed Zourob
Spinal muscular atrophy is an untreatable potentially fatal hereditary disorder caused by loss-of-function mutations in the survival motor neuron (SMN) 1 gene which encodes the SMN protein. Currently, definitive diagnosis relies on the demonstration of biallelic pathogenic variants in SMN1 gene. Therefore, there is an urgent unmet need to accurately quantify SMN protein levels for screening and therapeutic monitoring of symptomatic newborn and SMA patients, respectively. Here, we developed a voltammetric immunosensor for the sensitive detection of SMN protein based on covalently functionalized carbon nanofiber-modified screen printed electrodes...
October 10, 2017: Biosensors & Bioelectronics
https://www.readbyqxmd.com/read/29095865/beneficial-effects-of-high-dose-taurine-treatment-in-juvenile-dystrophic-mdx-mice-are-offset-by-growth-restriction
#17
Jessica R Terrill, Gavin J Pinniger, Keshav V Nair, Miranda D Grounds, Peter G Arthur
Duchenne Muscular Dystrophy (DMD) is a fatal muscle wasting disease manifested in young boys, for which there is no current cure. We have shown that the amino acid taurine is safe and effective at preventing dystropathology in the mdx mouse model for DMD. This study aimed to establish if treating growing mdx mice with a higher dose of taurine was more effective at improving strength and reducing inflammation and oxidative stress. Mice were treated with a dose of taurine estimated to be 16 g/kg/day, in drinking water from 1-6 weeks of age, after which in vivo and ex vivo muscle strength was assessed, as were measures of inflammation, oxidative stress and taurine metabolism...
2017: PloS One
https://www.readbyqxmd.com/read/29093790/perioperative-evaluation-of-respiratory-muscle-strength-after-scoliosis-correction-in-patients-with-duchenne-muscular-dystrophy
#18
Wataru Saito, Kosuke Mizuno, Gen Inoue, Takayuki Imura, Toshiyuki Nakazawa, Masayuki Miyagi, Eiki Shirasawa, Kentaro Uchida, Masashi Takaso
Study Design: Retrospective cohort study. Purpose: To investigate the effect of spinal correction on respiratory muscle strength in patients with Duchenne muscular dystrophy (DMD). Overview of Literature: Several studies have reported that scoliosis correction in patients with DMD does not improve pulmonary function. In these studies, pulmonary function was evaluated using the traditional spirometric values of percent vital capacity (%VC) and percent forced vital capacity (%FVC)...
October 2017: Asian Spine Journal
https://www.readbyqxmd.com/read/29092410/extended-dynamic-mode-decomposition-with-dictionary-learning-a-data-driven-adaptive-spectral-decomposition-of-the-koopman-operator
#19
Qianxiao Li, Felix Dietrich, Erik M Bollt, Ioannis G Kevrekidis
Numerical approximation methods for the Koopman operator have advanced considerably in the last few years. In particular, data-driven approaches such as dynamic mode decomposition (DMD)(51) and its generalization, the extended-DMD (EDMD), are becoming increasingly popular in practical applications. The EDMD improves upon the classical DMD by the inclusion of a flexible choice of dictionary of observables which spans a finite dimensional subspace on which the Koopman operator can be approximated. This enhances the accuracy of the solution reconstruction and broadens the applicability of the Koopman formalism...
October 2017: Chaos
https://www.readbyqxmd.com/read/29090245/do-lumen-apposing-metal-stents-lams-improve-treatment-outcomes-of-walled-off-pancreatic-necrosis-over-plastic-stents-using-dual-modality-drainage
#20
Nadav Sahar, Richard Kozarek, Zaheer S Kanji, Andrew S Ross, Michael Gluck, S Ian Gan, Michael Larsen, Shayan Irani
BACKGROUND AND STUDY AIMS : Endoscopic ultrasound-guided drainage of symptomatic walled-off pancreatic necrosis (WON) usually has been performed with double pigtail plastic stents (DPS) and more recently, with lumen-apposing metal stents (LAMS). However, LAMS are significantly more expensive and there are no comparative studies with DPS. Accordingly, we compared our experience with combined endoscopic and percutaneous drainage (dual-modality drainage [DMD]) for symptomatic WON using LAMS versus DPS...
November 2017: Endoscopy International Open
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