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https://www.readbyqxmd.com/read/28538597/surgical-management-of-thoracolumbar-kyphosis-in-patients-with-mucopolysaccharidosis-a-systematic-review
#1
Nicole Williams, Peter Cundy, Deborah Eastwood
STUDY DESIGN: Systematic Review OBJECTIVE.: To determine the indications and outcomes for surgical treatment of thoracolumbar kyphosis in patients with mucopolysaccharidoses (MPS) in order to define future studies. SUMMARY OF BACKGROUND DATA: Improvements in the medical treatment of MPS have increased lifespan and improved quality of life for many patients, but with no effect on thoracolumbar kyphosis. A greater number of these challenging patients may now be considered spinal surgical candidates...
May 22, 2017: Spine
https://www.readbyqxmd.com/read/28535791/clinical-outcomes-in-a-subpopulation-of-adults-with-morquio-a-syndrome-results-from-a-long-term-extension-study-of-elosulfase-alfa
#2
D Hughes, R Giugliani, N Guffon, S A Jones, K E Mengel, R Parini, R Matousek, S M Hawley, A Quartel
BACKGROUND: This post hoc subanalysis examined outcomes in adult patients with Morquio A (mucopolysaccharidosis IVA) who received enzyme replacement therapy (ERT) with elosulfase alfa over a 120-weeks period. Patients ≥18 years of age evaluated in an open-label, long-term extension study of elosulfase alfa (modified per protocol [MPP], n = 32; intent-to-treat [ITT], n = 37; MOR-005; NCT01415427) were compared with the ≥18-year-old untreated population with 2-years follow-up from a Morquio A natural history study (n = 10; MorCAP; NCT00787995)...
May 23, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28533011/effect-of-surface-treatments-on-repair-with-composite-resin-of-a-partially-monoclinic-phase-transformed-yttrium-stabilized-tetragonal-zirconia
#3
Beatriz Regalado Galvão Ribeiro, Marilia Regalado Galvão Rabelo Caldas, Antonio Alves Almeida, Renata Garcia Fonseca, Gelson Luis Adabo
STATEMENT OF PROBLEM: Studies of composite resin repairs of yttrium-tetragonal zirconia polycrystal (Y-TZP) are usually performed in its tetragonal phase, but it may be partially transformed into a monoclinic phase in a clinical fracture. PURPOSE: The purpose of this in vitro study was to evaluate the effect of airborne-particle abrasion (APA) and a bonding agent on the shear bond strength (SBS) between a composite resin and hydrothermally aged Y-TZP. MATERIAL AND METHODS: Specimens (7...
May 20, 2017: Journal of Prosthetic Dentistry
https://www.readbyqxmd.com/read/28530135/prolonged-expression-of-secreted-enzymes-in-dogs-after-liver-directed-delivery-of-i-sleeping-beauty-i-transposons-implications-for-non-viral-gene-therapy-of-systemic-disease
#4
Elena L Aronovich, Kendra Anne Hyland, Bryan C Hall, Jason B Bell, Erik R Olson, Myra Urness Rusten, David W Hunter, N Matthew Ellinwood, R Scott McIvor, Perry B Hackett
The non-viral, integrating Sleeping Beauty (SB) transposon system is efficient in treating systemic monogenic disease in mice including hemophilias A and B caused by deficiency of blood clotting factors and mucopolysaccharidosis (MPS) types I and VII caused by α-L-iduronidase (IDUA) and β-glucuronidase (GUSB) deficiency, respectively. We recently reported modified approaches of the hydrodynamics-based procedure to deliver transposons to the liver in dogs. Using the transgenic canine reporter secreted alkaline phosphatase (cSEAP), we demonstrated transgenic protein in the plasma for up to six weeks post-infusion...
May 20, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/28513999/microparticles-of-healthy-origins-improve-endothelial-progenitor-cell-dysfunction-via-microrna-transfer-in-an-atherosclerotic-hamster-model
#5
Nicoleta Alexandru, Eugen Andrei, Loredan Niculescu, Emanuel Dragan, Violeta Ristoiu, Adriana Georgescu
AIM: In this study we aimed: (1) to obtain and functionally characterize the cultures of late endothelial progenitor cells (EPCs) from the animal blood; (2) to investigate the potential beneficial effects of circulating microparticles (MPs) of healthy origins on EPC dysfunctionality in atherosclerosis as well as involved mechanisms. METHODS: Late EPCs were obtained and expanded in culture from peripheral blood isolated from two animal groups: hypertensive-hyperlipidemic (HH) and control (C) hamsters...
May 17, 2017: Acta Physiologica
https://www.readbyqxmd.com/read/28511172/donor-specific-anti-human-leukocyte-antigens-antibodies-acute-rejection-renal-function-and-histology-in-kidney-transplant-recipients-receiving-tacrolimus-and-everolimus
#6
Alexandra Ferreira, Claudia Felipe, Marina Cristelli, Laila Viana, Geovana Basso, Suelen Stopa, Juliana Mansur, Mayara Ivani, Adrieli Bessa, Priscila Ruppel, Wilson Aguiar, Erika Campos, Maria Gerbase-DeLima, Henrique Proença, Helio Tedesco-Silva, José Medina-Pestana
BACKGROUND: This analysis compared efficacy, renal function, and histology in kidney transplant recipients receiving tacrolimus (TAC) combined with everolimus (EVR) or mycophenolate (MPS). METHODS: This was a retrospective analysis from a randomized trial in kidney transplant recipients who received a single 3 mg/kg dose of rabbit antithymocyte globulin (r-ATG), TAC, EVR, and prednisone (PRED; r-ATG/EVR, n = 85), basiliximab (BAS), TAC, EVR, and PRED (BAS/EVR, n = 102) or BAS, TAC, MPS, and PRED (BAS/MPS, n = 101)...
May 17, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28495847/inflammatory-responses-to-acute-elevations-of-carbon-dioxide-in-mice
#7
Stephen R Thom, Veena M Bhopale, Jingping Hu, Ming Yang
Health risks are described from elevated indoor air carbon dioxide (CO2), which often ranges from 1000 to 4000 parts per million (ppm), but mechanisms are unknown. Here, we demonstrate that mice exposed for 2 hours to 2000 or 4000 ppm CO2 exhibit, respectively, 3.4 + 0.9 (SE, n=6) and 4.1 + 0.7 (n=10)-fold elevations in circulating microparticles (MPs); neutrophil and platelet activation and vascular leak in brain, muscle and distal colon. Interleukin (IL)-1β content of MPs also increases after 2000 ppm by 3...
May 11, 2017: Journal of Applied Physiology
https://www.readbyqxmd.com/read/28494282/anthropogenic-fibres-in-the-baltic-sea-water-column-field-data-laboratory-and-numerical-testing-of-their-motion
#8
A Bagaev, A Mizyuk, L Khatmullina, I Isachenko, I Chubarenko
Distribution of microplastics particles (MPs) in the water column is investigated on the base of 95 water samples collected from various depths in the Baltic Sea Proper in 2015-2016. Fibres are the prevalent type of MPs: 7% of the samples contained small films; about 40% had (presumably) paint flakes, while 63% contained coloured fibres in concentrations from 0.07 to 2.6 items per litre. Near-surface and near-bottom layers (defined as one tenth of the local depth) have 3-5 times larger fibre concentrations than intermediate layers...
May 7, 2017: Science of the Total Environment
https://www.readbyqxmd.com/read/28476725/mobile-phone-surveys-for-collecting-population-level-estimates-in-low-and-middle-income-countries-a-literature-review
#9
Dustin G Gibson, Amanda Pereira, Brooke A Farrenkopf, Alain B Labrique, George W Pariyo, Adnan A Hyder
BACKGROUND: National and subnational level surveys are important for monitoring disease burden, prioritizing resource allocation, and evaluating public health policies. As mobile phone access and ownership become more common globally, mobile phone surveys (MPSs) offer an opportunity to supplement traditional public health household surveys. OBJECTIVE: The objective of this study was to systematically review the current landscape of MPSs to collect population-level estimates in low- and middle-income countries (LMICs)...
May 5, 2017: Journal of Medical Internet Research
https://www.readbyqxmd.com/read/28476720/moving-the-agenda-on-noncommunicable-diseases-policy-implications-of-mobile-phone-surveys-in-low-and-middle-income-countries
#10
George W Pariyo, Adaeze C Wosu, Dustin G Gibson, Alain B Labrique, Joseph Ali, Adnan A Hyder
The growing burden of noncommunicable diseases (NCDs), for example, cardiovascular diseases and chronic respiratory diseases, in low- and middle-income countries (LMICs) presents special challenges for policy makers, due to resource constraints and lack of timely data for decision-making. Concurrently, the increasing ubiquity of mobile phones in LMICs presents possibilities for rapid collection of population-based data to inform the policy process. The objective of this paper is to highlight potential benefits of mobile phone surveys (MPS) for developing, implementing, and evaluating NCD prevention and control policies...
May 5, 2017: Journal of Medical Internet Research
https://www.readbyqxmd.com/read/28474406/analysis-of-rbc-microparticles-in-stored-whole-blood-bags-a-promising-marker-to-detect-blood-doping-in-sports
#11
Sven Christian Voss, Morana Jaganjac, Amna Mohamed Al-Thani, Jean Charles Grivel, Christophe Raynaud, Hind Al-Jaber, Afnan Saleh Al-Menhali, Zeyed Ahmad Merenkov, Mohammed Alsayrafi, Aishah Latiff, Costas Georgakopoulos
Blood doping in sports is prohibited by the World Anti-Doping Agency (WADA). To find a possible biomarker for the detection of blood doping, we investigated the changes in blood stored in CPDA-1 blood bags of eight healthy subjects who donated one unit of blood. Aliquots were taken on days 0, 14 and 35. Platelet free Plasma was prepared and stored at -80 °C until analysis on a flow cytometer dedicated for the analysis of Microparticles (MPs). Changes in the number of Red Blood Cell (RBC) -MPs were highly significant (p < 0...
May 4, 2017: Drug Testing and Analysis
https://www.readbyqxmd.com/read/28467847/spin-projected-matrix-product-states-versatile-tool-for-strongly-correlated-systems
#12
Zhendong Li, Garnet Kin-Lic Chan
We present a new wave function ansatz that combines the strengths of spin projection with the language of matrix product states (MPS) and matrix product operators (MPO) as used in the density matrix renormalization group (DMRG). Specifically, spin-projected matrix product states (SP-MPS) are constructed as [Formula: see text], where [Formula: see text] is the spin projector for total spin S and |ΨMPS((N,M))⟩ is an MPS wave function with a given particle number N and spin projection M. This new ansatz possesses several attractive features: (1) It provides a much simpler route to achieve spin adaptation (i...
May 16, 2017: Journal of Chemical Theory and Computation
https://www.readbyqxmd.com/read/28462595/intranasal-aav-mediated-gene-delivery-and-expression-of-human-iduronidase-in-the-cns-a-non-invasive-and-effective-approach-for-prevention-of-neurologic-disease-in-mucopolysaccharidosis-type-i
#13
Lalitha R Belur, Alexa Temme, Kelly M Podetz-Pedersen, Maureen Riedl, Lucy Vulchanova, Nicholas Robinson, Leah R Hanson, Karen F Kozarsky, Paul J Orchard, William H Frey Ii, Walter C Low, R Scott McIvor
Mucopolysaccharidosis type I (MPS I) is a progressive, multisystemic, inherited metabolic disease caused by deficiency of -L-iduronidase (IDUA). Current treatments for this disease are ineffective in treating CNS disease due to the inability of lysosomal enzymes to traverse the blood-brain barrier. We have taken a non-invasive and effective approach to the treatment of CNS disease by intranasal administration of an IDUA-encoding adeno-associated virus serotype 9 (AAV9) vector. Adult IDUA-deficient mice were immunotolerized at birth with human iduronidase (Aldurazyme), to prevent anti-IDUA immune response, and at 3 months of age were instilled intranasally with AAV9-IDUA vector...
May 2, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/28457718/the-effect-of-galsulfase-enzyme-replacement-therapy-on-the-growth-of-patients-with-mucopolysaccharidosis-vi-maroteaux-lamy-syndrome
#14
P Harmatz, C J Hendriksz, C Lampe, J J McGill, R Parini, E Leão-Teles, V Valayannopoulos, T J Cole, R Matousek, S Graham, N Guffon, A Quartel
Mucopolysaccharidosis (MPS) VI is an autosomal recessive lysosomal storage disorder arising from deficient activity of N-acetylgalactosamine-4-sulfatase (arylsulfatase B) and subsequent intracellular accumulation of the glycosaminoglycans (GAGs) dermatan sulfate and chondroitin-4-sulfate. Manifestations are multi-systemic and include skeletal abnormalities such as dysostosis multiplex and short stature. Reference height-for-age growth charts for treatment-naïve MPS VI patients have been published for both the slowly and rapidly progressing populations...
March 31, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28455159/metal-versus-plastic-stents-for-anastomotic-biliary-strictures-after-liver-transplantation-a-randomized-controlled-trial
#15
Fernanda Prata Martins, Gustavo Andrade De Paulo, Mônica L C Contini, Angelo Paulo Ferrari
BACKGROUND AND AIMS: biliary anastomotic stricture occurs in 15% to 20% of patients after deceased orthotopic liver transplantation (OLT). Usually treated endoscopically with multiple plastic stents (MPSs), although the use of fully covered self-expandable metal stents (cSEMSs) is emerging. This study aims to compare efficacy and safety of cSEMSs versus MPSs in these patients. METHODS: single center, open-label, randomized clinical trial. Patients were randomized to single cSEMSs for 6 months or to MPS placement, exchanged every 3 months during 1 year...
April 25, 2017: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28450313/enteric-coated-mycophenolate-sodium-versus-azathioprine-in-patients-with-active-systemic-lupus-erythematosus-a-randomised-clinical-trial
#16
Josep Ordi-Ros, Luis Sáez-Comet, Mercedes Pérez-Conesa, Xavier Vidal, Francesca Mitjavila, Antoni Castro Salomó, Jordi Cuquet Pedragosa, Vera Ortiz-Santamaria, Montserrat Mauri Plana, Josefina Cortés-Hernández
OBJECTIVE: To compare the efficacy and safety of enteric-coated mycophenolate sodium (EC-MPS) versus azathioprine (AZA) in patients with active systemic lupus erythematosus (SLE) disease. METHODS: A multicentre, 24-month, superiority, open-label, randomised controlled trial (NCT01112215) was conducted with 240 patients (120 per arm) receiving either EC-MPS (target dose: 1440 mg/day) or AZA (target dose: 2 mg/kg/day) in addition to prednisone and/or antimalarials...
April 27, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28449327/skeletal-muscle-il-15-il-15r%C3%AE-and-myofibrillar-protein-synthesis-after-resistance-exercise
#17
Alberto Pérez-López, James McKendry, Marcos Martin-Rincon, David Morales-Alamo, Bárbara Pérez-Köhler, David Valadés, Julia Buján, Jose A L Calbet, Leigh Breen
In vitro and in vivo studies described the myokine IL-15 and its receptor IL-15Rα as anabolic/anti-atrophy agents, however the protein expression of IL-15Rα has not been measured in human skeletal muscle and data regarding IL-15 expression remain inconclusive. The purpose of the study was to determine serum and skeletal muscle IL-15 and IL-15Rα responses to resistance exercise session and to analyse their association with myofibrillar protein synthesis (MPS). Fourteen participants performed a bilateral leg resistance exercise composed of 4 sets of leg press and 4 sets of knee extension at 75% 1RM to task failure...
April 27, 2017: Scandinavian Journal of Medicine & Science in Sports
https://www.readbyqxmd.com/read/28432840/detection-of-egfr-mutations-in-patients-with-non-small-cell-lung-cancer-by-high-resolution-melting-comparison-with-other-methods
#18
Carlos Martínez-Carretero, Fernando Iguaz Pascual, Antonio Rus, Ivan Bernardo
BACKGROUND: The discovery of mutations in the epidermal growth factor receptor gene (EGFR) related to the clinical response to tyrosine kinase inhibitors, has transformed the management of non-small cell lung cancer (NSCLC). Several methods have been developed for determination of mutations in EGFR, with different sensitivity and potential ability to detect a different number of mutations. METHODS: We developed a screening method by high resolution melting (HRM) to detect EGFR mutations, and compared the results of 123 fixed in formalin and paraffin embedded (FFPE) tumor tissue samples with the detection of mutations by allele-specific PCR...
April 22, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28429088/an-snp-panel-for-the-analysis-of-paternally-inherited-alleles-in-maternal-plasma-using-ion-torrent-pgm
#19
Donggui Yang, Hao Liang, Shaobin Lin, Qing Li, Xiaoyan Ma, Jun Gao, Hongyu Sun, Qingqing Chen, Jianzhu Wu, Xueling Ou
Researchers have sought to develop an effective protocol for paternity analysis using cell-free DNA (cfDNA) in maternal plasma. The use of massively parallel sequencing (MPS) technology for SNP testing is attractive because of its high-throughput capacity and resolution to single-base precision. In this study, we designed a customized SNP panel for cfDNA sequencing that includes 720 short amplicons (< 140 bp) targeting SNPs on the autosome and Y chromosome. The systemic performance was evaluated using the Ion Torrent PGM, indicating balanced coverage among most of the included loci, except for 78 poorly performing SNPs that were observed to have an inconsistent allele balance, lower coverage reads or high background signals...
April 20, 2017: International Journal of Legal Medicine
https://www.readbyqxmd.com/read/28428471/motion-frozen-imaging-by-gated-myocardial-perfusion-single-photon-emission-computed-tomography-using-multi-focus-fan-beam-collimator-in-thallium-201-study
#20
Kaito Takahashi, Takashi Takeuchi, Yoshiyuki Hosokai, Hayato Odagiri, Haruo Saito
PURPOSE: This study aimed to evaluate the statistical noise of motion-frozen (MF) image generated by gated myocardial perfusion single photon emission computed tomography (SPECT) imaging using IQ · SPECT and to determine the optimal acquisition and reconstruction parameters for MF image using IQ · SPECT. METHODS: A movement cardiac phantom and static cardiac phantom were used to acquire the MF images. The acquisition times used were different in 8 and 16 frames per R-R interval, and varying reconstruction parameters (subset and iteration) were used...
2017: Nihon Hoshasen Gijutsu Gakkai Zasshi
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