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https://www.readbyqxmd.com/read/29761311/comparison-of-fatigue-responses-and-rapid-force-characteristics-between-explosive-and-traditional-resistance-trained-males
#1
Cameron S Mackey, Ryan M Thiele, Eric C Conchola, Jason M DeFreitas
PURPOSE: To compare maximal and rapid force characteristics, as well as fatigability, between traditional (TRT) and explosive (ERT) resistance-trained men. METHODS: Fourteen TRT (mean age = 25 years) and twelve ERT (mean age = 22 years) men performed rapid maximal contractions followed by an isokinetic fatigue protocol consisting of 50 maximal knee extension (KE) and flexions (KF) at a moderate speed (180° s- ¹). Baseline measures included: isokinetic peak torque (PT), isometric rate of torque development (RTD0-50 ), peak acceleration (ACCmax ), and peak velocity (Vmax )...
May 14, 2018: European Journal of Applied Physiology
https://www.readbyqxmd.com/read/29760141/efficacy-of-daptomycin-monotherapy-and-in-combination-with-%C3%AE-lactams-for-daptomycin-susceptible-enterococcus-faecium-harboring-liasr-substitutions-influence-of-the-inoculum-effect
#2
Razieh Kebriaei, Seth A Rice, Kavindra V Singh, Kyle C Stamper, An Q Dinh, Rafael Rios, Lorena Diaz, Barbara E Murray, Jose M Munita, Truc T Tran, Cesar A Arias, Michael J Rybak
Enterococcus faecium that harbor LiaFSR substitutions but are phenotypically susceptible to daptomycin (DAP) by current breakpoints are problematic since predisposition to resistance may lead to therapeutic failure. Using a simulated endocardial vegetation (SEV) PK/PD model, we investigated DAP regimens (6, 8 and 10 mg/kg/day) as monotherapy and in combination with ampicillin (AMP), ceftaroline (CPT) or ertapenem (ERT) against E. faecium HOU503, a DAP-susceptible strain that harbors common LiaS and LiaR substitutions found in clinical isolates (T120S and W73C, respectively)...
May 14, 2018: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/29750286/enzyme-replacement-therapy-a-review-and-its-role-in-treating-lysosomal-storage-diseases
#3
Mindy Li
Lysosomal storage diseases (LSDs) are a heterogeneous group of genetic disorders caused by defects in lysosomal function that lead to multiorgan system damage. Due to wide clinical variability within even a single disorder, making a diagnosis can be difficult and identification may be delayed. Enzyme replacement therapy (ERT) was first approved as a treatment for the LSD Gaucher disease in 1991. ERT development for other LSDs followed, and ERT is currently approved for eight LSDs in the United States. ERT may help slow progression and improve clinical symptoms, but it cannot affect neurologic features due to its inability to cross the blood-brain barrier...
May 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29749992/a-new-mutation-causing-severe-infantile-onset-pompe-disease-responsive-to-enzyme-replacement-therapy
#4
Hossein Moravej, Anis Amirhakimi, Alireza Showraki, Hamid Amoozgar, Zahra Hadipour, Ghasem Nikfar
Pompe disease (PD), also known as "glycogen storage disease type II (OMIM # 232300)" is a rare autosomal recessive disorder characterized by progressive glycogen accumulation in cellular lysosomes. It ultimately leads to cellular damage. Infantile-onset Pompe disease (IOPD) is the most severe type of this disease and is characterized by severe hypertrophic cardiomyopathy and generalized hypotonia. Mutations in the acid alpha-glucosidase ( GAA ) gene, located at locus 17q25.3, are responsible for the disease leading to reduced activity of the acid alpha-glucosidase enzyme...
March 2018: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29747997/the-beneficial-effects-of-long-term-enzyme-replacement-therapy-on-cardiac-involvement-in-japanese-fabry-patients
#5
Kenichi Hongo, Keiichi Ito, Taro Date, Ikuko Anan, Yasunori Inoue, Satoshi Morimoto, Kazuo Ogawa, Makoto Kawai, Hiroshi Kobayashi, Masahisa Kobayashi, Hiroyuki Ida, Toya Ohashi, Ikuo Taniguchi, Michihiro Yoshimura, Yoshikatsu Eto
Fabry disease is a hereditary disorder that occurs due to the reduction or absence of alpha-galactosidase A activity, which leads to cardiac involvement including left ventricular hypertrophy (LVH). Enzyme replacement therapy (ERT) provides better patient outcomes by preventing serious complications. However, there have been very few studies on the long-term effects of ERT on the cardiac manifestations in Japanese Fabry patients. We retrospectively analyzed the data from the medical records of 42 Fabry patients (male, n = 17; female, n = 25) who were followed at Jikei University Hospital, and in whom the long-term effects of ERT could be evaluated (median follow-up period: male, 11 years; female, 8 years)...
April 26, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29744787/ada-deficiency-evaluation-of-the-clinical-and-laboratory-features-and-the-outcome
#6
Deniz Cagdas, Pınar Gur Cetinkaya, Betül Karaatmaca, Saliha Esenboga, Cagman Tan, Togay Yılmaz, Ersin Gümüş, Safa Barış, Barış Kuşkonmaz, Tuba Turul Ozgur, Pawan Bali, Ines Santisteban, Diclehan Orhan, Aysel Yüce, Duygu Cetinkaya, Kaan Boztug, Michael Hershfield, Ozden Sanal, İlhan Tezcan
INTRODUCTION: Adenosine deaminase (ADA) deficiency is an autosomal recessive primary immunodeficiency. It results in the intracellular accumulation of toxic metabolites which have effects particularly on lymphocytes and the brain. The aim of this study was to evaluate the outcome of 13 ADA-deficient patients. We planned to evaluate their clinical and laboratory findings before and after enzyme replacement therapy (ERT), allogeneic hematopoietic stem cell transplantation (aHSCT), and hematopoietic stem cell gene therapy (HSCGT)...
May 9, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29743894/lycium-barbarum-polysaccharide-protects-hsf-cells-against-ultraviolet-induced-damage-through-the-activation-of-nrf2
#7
Bihua Liang, Liqian Peng, Runxiang Li, Huaping Li, Ziyin Mo, Xinyue Dai, Na Jiang, Qing Liu, Erting Zhang, Huiyan Deng, Zhenjie Li, Huilan Zhu
Background: Lycium barbarum polysaccharide (LBP) is considered an antioxidant agent. NF-E2-related factor-2 (Nrf2) is an important regulator for protection against UV damage. In this study, we verified the performance of LBP and the correlation between LBP and Nrf2. Methods: HSF cells were treated with LBP to determine dose and time dependencies. An antioxidant response element (ARE) reporter was designed to monitor the activity of the Nrf2 antioxidant pathway. Results: For HSF cells, the optimal LBP treatment was 300 μg/ml for 3 h...
2018: Cellular & Molecular Biology Letters
https://www.readbyqxmd.com/read/29729453/the-slam-family-receptors-potential-therapeutic-targets-for-inflammatory-and-autoimmune-diseases
#8
REVIEW
Matthew A Dragovich, Adam Mor
The signaling lymphocytic activation molecule (SLAM) family is comprised of nine distinct receptors (SLAMF1 through SLAMF9) that are expressed on hematopoietic cells. All of these receptors, with the exception of SLAMF4, are homotypic by nature as downstream signaling occurs when hematopoietic cells that express the same SLAM receptor interact. The SLAM family receptor function is largely controlled via SLAM associated protein (SAP) family adaptors. The SAP family adaptors consist of SAP, Ewing sarcoma associated transcript (EAT)-2, and EAT-2-related transducer (ERT)...
May 3, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29716835/enzyme-replacement-therapy-with-velmanase-alfa-human-recombinant-alpha-mannosidase-novel-global-treatment-response-model-and-outcomes-in-patients-with-alpha-mannosidosis
#9
Paul Harmatz, Federica Cattaneo, Diego Ardigò, Silvia Geraci, Julia B Hennermann, Nathalie Guffon, Allan Lund, Christian J Hendriksz, Line Borgwardt
Alpha-mannosidosis is an ultra-rare monogenic disorder resulting from a deficiency in the lysosomal enzyme alpha-mannosidase, with a prevalence estimated to be as low as 1:1,000,000 live births. The resulting accumulation of mannose-rich oligosaccharides in all tissues leads to a very heterogeneous disorder with a continuum of clinical manifestations with no distinctive phenotypes. Long-term enzyme replacement therapy (ERT) with velmanase alfa is approved in Europe for the treatment of patients with non-neurological manifestations of mild to moderate alpha-mannosidosis...
April 18, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29713479/pulmonary-involvement-in-fabry-disease-effect-of-plasma-globotriaosylsphingosine-and-time-to-initiation-of-enzyme-replacement-therapy
#10
Daniel Franzen, Sarah R Haile, David C Kasper, Thomas P Mechtler, Andreas J Flammer, Pierre A Krayenbühl, Albina Nowak
Introduction: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by mutations of GLA gene leading to reduced α-galactosidase activity and resulting in a progressive accumulation of globotriaosylceramide (Gb3) and its deacylated derivative, globotriaosyl-sphingosine (Lyso-Gb3). Plasma Lyso-Gb3 levels serve as a disease severity and treatment monitoring marker during enzyme replacement therapy (ERT). Methods: Adult patients with AFD who had yearly pulmonary function tests between 1999 and 2015 were eligible for this observational study...
2018: BMJ Open Respiratory Research
https://www.readbyqxmd.com/read/29705972/p-tau-and-subunit-c-mitochondrial-atp-synthase-accumulation-in-the-central-nervous-system-of-a-woman-with-hurler-scheie-syndrome-treated-with-enzyme-replacement-therapy-for-12-years
#11
Hiroshi Kobayashi, Masamichi Ariga, Yohei Sato, Masako Fujiwara, Nei Fukasawa, Takahiro Fukuda, Hiroyuki Takahashi, Masahiro Ikegami, Motomichi Kosuga, Torayuki Okuyama, Yoshikatsu Eto, Hiroyuki Ida
We report an autopsy case of a woman with mucopolysaccharidosis type I (MPS I) Hurler-Scheie syndrome who was treated with enzyme replacement therapy (ERT) for 12 years. This was the first case of MPS I treated with ERT in Japan. Pathological analysis showed no glycosaminoglycan accumulation in the liver and spleen as a result of long-term ERT, although severe aortic stenosis, diffuse intimal hyperplasia of the coronary artery, and fibrous hypertrophy of the endocardium were observed. Additionally, we detected subunit c mitochondrial ATP synthase (SCMAS) accumulation and mild tauopathy (hyperphosphorylated tau or p-tau, both 3-repeat and 4-repeat tau accumulation) in the same area of the cerebral limbic system and central gray matter of the mid brain and pons...
April 29, 2018: JIMD Reports
https://www.readbyqxmd.com/read/29704969/a-pilot-study-of-emotion-regulation-therapy-for-generalized-anxiety-and-depression-findings-from-a-diverse-sample-of-young-adults
#12
Megan E Renna, Jean M Quintero, Ariella Soffer, Martin Pino, Leslie Ader, David M Fresco, Douglas S Mennin
Emotion regulation therapy (ERT) for generalized anxiety disorder (GAD) and accompanying major depressive disorder (MDD) is a theoretically derived, evidence-based treatment that integrates principles from traditional and contemporary cognitive-behavioral and experiential approaches with basic and translational findings from affect science to offer a blueprint for improving intervention by focusing on the motivational responses and corresponding self-referential regulatory characteristics. Preliminary evidence supports the efficacy of a 20-session version of ERT...
May 2018: Behavior Therapy
https://www.readbyqxmd.com/read/29704721/hydraulic-reliability-of-a-horizontal-wetland-for-wastewater-treatment-in-sicily
#13
A Marzo, D Ventura, G L Cirelli, R Aiello, D Vanella, R Rapisarda, S Barbagallo, S Consoli
The purpose of this study was to evaluate how the hydraulic behavior of a horizontal subsurface wetland (HF), that is part of the hybrid wetland (hybrid-TW) of the IKEA® store in Eastern Sicily (Italy), influences the overall wastewater treatment performance. The HF unit experiences frequent overloading peaks due to the extreme variability in the number of visitors at the store, and after 2 years of operation it showed signals of partial clogging at the inlet area. The hydraulics of the HF unit has been monitored through measurements of hydraulic conductivity at saturation (Ks), tracer tests, and geophysical (i...
April 25, 2018: Science of the Total Environment
https://www.readbyqxmd.com/read/29696380/geophysical-and-hydrological-data-assimilation-to-monitor-water-content-dynamics-in-the-rocky-unsaturated-zone
#14
Lorenzo De Carlo, Marco Berardi, Michele Vurro, Maria Clementina Caputo
In recent years, geophysics is increasingly used to study the flow and transport processes in the vadose zone. Particularly, when the vadose zone is made up of rocks, it is difficult to install sensors in the subsurface to measure hydrological state variables directly. In these cases, the electrical resistivity tomography (ERT) represents a useful tool to monitor the hydrodynamics of the infiltration and to estimate hydraulic parameters and state variables, such as hydraulic conductivity and water content. We propose an integrated approach aimed at predicting water content dynamics in calcarenite, a sedimentary carbonatic porous rock...
April 25, 2018: Environmental Monitoring and Assessment
https://www.readbyqxmd.com/read/29674318/nicotiana-benthamiana-%C3%AE-galactosidase-a1-1-can-functionally-complement-human-%C3%AE-galactosidase-a-deficiency-associated-with-fabry-disease
#15
Kassiani Kytidou, Jules Beekwilder, Marta Artola, Eline van Meel, Ruud H P Wilbers, Geri F Moolenaar, Nora Goosen, Maria J Ferraz, Rebecca Katzy, Patrick Voskamp, Bogdan I Florea, Cornelis H Hokke, Herman S Overkleeft, Arjen Schots, Dirk Bosch, Navraj Pannu, Johannes M F G Aerts
α-Galactosidases (EC 3.2.1.22) are retaining glycosidases that cleave terminal α-linked galactose residues from glycoconjugate substrates. α-Galactosidases take part in the turnover of cell wall-associated galactomannans in plants and in the lysosomal degradation of glycosphingolipids in animals. Deficiency of human α-galactosidase A (α-Gal A) causes Fabry disease (FD), a heritable, X-linked lysosomal storage disorder, characterized by accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lysoGb3)...
April 19, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29671540/minimal-effect-of-ert-on-sexual-function-in-menopausal-women
#16
Allen F Shaughnessy
No abstract text is available yet for this article.
March 15, 2018: American Family Physician
https://www.readbyqxmd.com/read/29671225/effectiveness-of-early-hematopoietic-stem-cell-transplantation-in-preventing-neurocognitive-decline-in-mucopolysaccharidosis-type-ii-a-case-series
#17
A Selvanathan, C Ellaway, C Wilson, P Owens, P J Shaw, K Bhattacharya
The early progressive form of the X-linked disorder, Hunter syndrome or mucopolysaccharidosis type II (MPS II) (OMIM #309900), is characterized by cognitive decline, and pulmonary and cardiac complications that often cause death before 20 years of age. Deficiency of the lysosomal enzyme, iduronate-2-sulfatase (EC 3.1.6.13) results in deposition of the glycosaminoglycans, dermatan, and heparan sulfate in various tissues. In recent years, enzyme replacement therapy (ERT) has become the mainstay of treatment, but is expensive and ineffective in arresting cognitive decline...
April 19, 2018: JIMD Reports
https://www.readbyqxmd.com/read/29668480/the-6th-rare-disease-south-eastern-europe-see-meeting-skopje-macedonia-november-11th-2017
#18
Zoran Gucev, Velibor Tasic, Momir Polenakovic
The sixth SEE meeting on rare diseases (RDs) was held in MASA the November 10th, 2017. A block of lectures on rare renal diseases started the meeting: nephrotic syndrome, Alport syndrome, atypical HUS, hypophosphatemic rickets, CAKUT were presented in all complexities. Their molecular and genetic mechanisms were discussed. The discovery of a dozen of newly genes in CAKUT, congenital overgrowth, spodilocostal dysplasia, precocious puberty has been done with collaboration of Macedonian and foreign researchers...
December 1, 2017: Prilozi (Makedonska Akademija Na Naukite i Umetnostite. Oddelenie za Medicinski Nauki)
https://www.readbyqxmd.com/read/29656334/four-gaucher-disease-type-ii-patients-with-three-novel-mutations-a-single-centre-experience-from-turkey
#19
Fatma Derya Bulut, Deniz Kör, Berna Şeker-Yılmaz, Özlem Hergüner, Serdar Ceylaner, Ferda Özkınay, Sebile Kılavuz, Neslihan Önenli-Mungan
Gaucher disease is the most common lysosomal storage disorder due to glucosylceramidase enzyme deficiency. There are three subtypes of the disease. Neurological involvement accompanies visceral and haematological findings only in type II and type III Gaucher patients. Type II is the acute progressive neuronopathic form which is the most severe and rare subtype. Clinical findings are recognized prenatally or in the first months of life and followed by death within the first two years of age. Among our 81 Gaucher patients, we identified 4 (4,9%) type II patients in our metabolic centre...
April 14, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/29648648/fgf-signaling-deregulation-is-associated-with-early-developmental-skeletal-defects-in-animal-models-for-mucopolysaccharidosis-type-ii-mpsii
#20
Stefania Bellesso, Marika Salvalaio, Susanna Lualdi, Elisa Tognon, Roberto Costa, Paola Braghetta, Chiara Giraudo, Roberto Stramare, Laura Rigon, Mirella Filocamo, Rosella Tomanin, Enrico Moro
Skeletal abnormalities represent a major clinical burden in patients affected by the lysosomal storage disorder mucopolysaccharidosis type II (MPSII, OMIM #309900). While extensive research has emphasized the detrimental role of stored glycosaminoglycans (GAGs) in the bone marrow (BM), a limited understanding of primary cellular mechanisms underlying bone defects in MPSII has hampered the development of bone-targeted therapeutic strategies beyond enzyme replacement therapy (ERT). We here investigated the involvement of key signaling pathways related to the loss of iduronate-2-sulfatase activity in two different MPSII animal models, D...
April 10, 2018: Human Molecular Genetics
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