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MPS 1H

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https://www.readbyqxmd.com/read/27636911/hip-morphology-in-mps-1h-patients-an-mri-based-study
#1
Sandra R Breyer, Nicole Muschol, Mona Schmidt, Martin Rupprecht, Kornelia Babin, Jochen Herrmann, Ralf Stücker
BACKGROUND: Hip dysplasia is common in mucopolysaccharidosis type-1H (MPS-1H) patients, but its morphology is not completely understood. No magnetic resonance imaging (MRI)-based studies have been reported in the literature. The purpose of this study was to improve knowledge of hip dysplasia pathology by describing the hip morphology of these patients in MRI scans, plain radiographs, and arthrograms. METHODS: We performed a retrospective chart review of 18 MPS-1H patients...
September 15, 2016: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/27107739/mononuclear-phagocytes-contribute-to-intestinal-invasion-and-dissemination-of-yersinia-enterocolitica
#2
Doreen Drechsler-Hake, Hanin Alamir, Julia Hahn, Manina Günter, Samuel Wagner, Monika Schütz, Erwin Bohn, Katja Schenke-Layland, Fabio Pisano, Petra Dersch, Ingo B Autenrieth, Stella E Autenrieth
Enteropathogenic Yersinia enterocolitica (Ye) enters the host via contaminated food. After colonisation of the small intestine Ye invades the Peyer's patches (PPs) via M cells and disseminates to the mesenteric lymph nodes (MLNs), spleen and liver. Whether Ye uses other invasion routes and which pathogenicity factors are required remains elusive. Oral infection of lymphotoxin-β-receptor deficient mice lacking PPs and MLNs with Ye revealed similar bacterial load in the spleen 1h post infection as wild-type mice, demonstrating a PP-independent dissemination route for Ye...
September 2016: International Journal of Medical Microbiology: IJMM
https://www.readbyqxmd.com/read/26918231/the-neurobehavioral-phenotype-in-mucopolysaccharidosis-type-iiib-an-exploratory-study
#3
E Shapiro, K King, A Ahmed, K Rudser, R Rumsey, B Yund, K Delaney, I Nestrasil, C Whitley, M Potegal
OBJECTIVES: Our goal was to describe the neurobehavioral phenotype in mucopolysaccharidosis Type IIIB (MPS IIIB). Parents report that behavioral abnormalities are a major problem in MPS III posing serious challenges to parenting and quality-of-life for both patient and parent. Our previous research on MPS IIIA identified autistic symptoms, and a Klüver-Bucy-type syndrome as indicated by reduced startle and loss of fear associated with amygdala atrophy. We hypothesized that MPS IIIB would manifest similar attributes when assessed with the same neurobehavioral protocol...
March 1, 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/24090814/carbon-monoxide-inhalation-increases-microparticles-causing-vascular-and-cns-dysfunction
#4
Jiajun Xu, Ming Yang, Paul Kosterin, Brian M Salzberg, Tatyana N Milovanova, Veena M Bhopale, Stephen R Thom
We hypothesized that circulating microparticles (MPs) play a role in pro-inflammatory effects associated with carbon monoxide (CO) inhalation. Mice exposed for 1h to 100 ppm CO or more exhibit increases in circulating MPs derived from a variety of vascular cells as well as neutrophil activation. Tissue injury was quantified as 2000 kDa dextran leakage from vessels and as neutrophil sequestration in the brain and skeletal muscle; and central nervous system nerve dysfunction was documented as broadening of the neurohypophysial action potential (AP)...
December 1, 2013: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/23562162/enzyme-replacement-therapy-started-at-birth-improves-outcome-in-difficult-to-treat-organs-in-mucopolysaccharidosis-i-mice
#5
Guilherme Baldo, Fabiana Q Mayer, Bárbara Z Martinelli, Talita G de Carvalho, Fabiola S Meyer, Patrícia G de Oliveira, Luise Meurer, Angela Tavares, Ursula Matte, Roberto Giugliani
Since we previously observed that in patients with mucopolysaccharidosis (MPS) the storage of undegraded glycosaminoglycans (GAG) occurs from birth, in the present study we aimed to compare normal, untreated MPS I mice (knockout for alpha-l-iduronidase-IDUA), and MPS I mice treated with enzyme replacement therapy (ERT, Laronidase, 1.2mg/kg every 2 weeks) started from birth (ERT-neo) or from 2 months of age (ERT-ad). All mice were sacrificed at 6 months. Both treatments were equally effective in normalizing GAG levels in the viscera but had no detectable effect on the joint...
May 2013: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/22549501/limited-sampling-strategy-for-mycophenolic-acid-in-renal-transplant-recipients-reciving-enteric-coated-mycophenolate-sodium-and-tacrolimus
#6
RANDOMIZED CONTROLLED TRIAL
Ana I Sánchez Fructuoso, Isabel Perez-Flores, Natividad Calvo, Rosalia Valero, Elisa Matilla, Dolores Ortega, Maria A Del Dedo, Cristina Fernández-Pérez, Alberto Barrientos
BACKGROUND: Substantial research has been conducted to develop limited-sampling strategies (LSS) to estimate the area under the curve (AUC(0-12h)) in patients treated with mycophenolate mofetil. However, no LSS has been validated for enteric-coated mycophenolate sodium (EC-MPS). Our aim was to develop an LSS to measure the AUC(0-12h) of mycophenolic acid in kidney recipients treated with tacrolimus and EC-MPS. STUDY DESIGN: Thirteen serial blood samples were collected over 12 hours from 71 patients treated with tacrolimus and EC-MPS...
June 2012: Therapeutic Drug Monitoring
https://www.readbyqxmd.com/read/22382905/-carpal-tunnel-syndrome-in-children-with-mucopolysaccharidosis-type-1h-diagnosis-and-therapy-in-an-interdisciplinary-centre
#7
M V Meyer-Marcotty, K Kollewe, R Dengler, L Grigull, M A Altintas, P M Vogt
Carpal tunnel syndrome is common in children with mucopolysaccharidosis type 1H (MPS type 1H). Clinical signs of carpal tunnel syndrome are frequently absent in these children and it is often very difficult to perform and interpret neurophysiological investigations. In this article we wish to present our experience and results regarding the diagnosis and postoperative results after decompression of the median nerve.In an interdisciplinary set-up we are currently treating 11 MPS type 1H children following blood stem cell transplantation...
January 2012: Handchirurgie, Mikrochirurgie, Plastische Chirurgie
https://www.readbyqxmd.com/read/22269664/controlled-shielding-and-deshielding-of-gene-delivery-polyplexes-using-hydroxyethyl-starch-hes-and-alpha-amylase
#8
Matthäus Noga, Daniel Edinger, Wolfgang Rödl, Ernst Wagner, Gerhard Winter, Ahmed Besheer
The non-viral delivery of nucleic acids faces many extracellular and intracellular hurdles on the way from injection site to the site of action. Among these, aggregation in the blood stream and rapid elimination by the mononuclear phagocytic system (MPS) represent strong obstacles towards successful development of these promising therapeutic modalities. Even the state-of-the-art solutions using PEGylation show low transfection efficiency due to limited uptake and hindered endosomal escape. Engineering the carriers with sheddable coats reduces aggregation and phagocytosis due to the effective shielding, while the controlled deshielding at the desired site of action enhances the uptake and intracellular release...
April 10, 2012: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/22197021/synthesis-and-characterisation-of-bonded-mercaptopropyl-silica-intermediate-stationary-phases-prepared-using-multifunctional-alkoxysilanes-in-supercritical-carbon-dioxide-as-a-reaction-solvent
#9
Benjamin A Ashu-Arrah, Jeremy D Glennon, Klaus Albert
This research employed (29)Si and (13)C Cross-Polarisation/Magic Angle Spinning (CP/MAS) NMR spectroscopy to characterise the nature and amount of surface species of di-and trifunctional mercaptopropylsilane (MPS) bonded silica using supercritical carbon dioxide (sc-CO(2)) as a reaction solvent without additives (co-solvent) or catalysts. The MPS stationary phases were prepared within 1h at a temperature and pressure of 70°C and 414 bar, respectively. Complementary analysis including elemental analysis, thermogravimetric analysis (TGA), DRIFT spectroscopy and BET surface area measurements were employed to characterise the bonded MPS intermediate stationary phases in support of data obtained from solid-state NMR analysis...
January 27, 2012: Journal of Chromatography. A
https://www.readbyqxmd.com/read/21392960/improved-bacteria-detection-by-coupling-magneto-immunocapture-and-amperometry-at-flow-channel-microband-electrodes
#10
Olivier Laczka, José-María Maesa, Neus Godino, Javier del Campo, Mikkel Fougt-Hansen, Jorg P Kutter, Detlef Snakenborg, Francesc-Xavier Muñoz-Pascual, Eva Baldrich
This paper describes the first immunosensing system reported for the detection of bacteria combining immunomagnetic capture and amperometric detection in a one-step sandwich format, and in a microfluidic environment. Detection is based on the electrochemical monitoring of the activity of horseradish peroxidase (HRP), an enzyme label, through its catalysis of hydrogen peroxide (H(2)O(2)) in the presence of the mediator hydroquinone (HQ). The enzymatic reaction takes place in an incubation micro-chamber where the magnetic particles (MPs) are confined, upstream from the working electrode...
April 15, 2011: Biosensors & Bioelectronics
https://www.readbyqxmd.com/read/17616138/structural-elucidation-of-phototransformation-products-of-azimsulfuron-in-water
#11
M Vittoria Pinna, Michele Zema, Carlo Gessa, Alba Pusino
The photodegradation of the sulfonylurea herbicide azimsulfuron, N-[[(4,6-dimethoxypyrimidin-2-yl)amino]carbonyl]-1-methyl-4-(2-methyl-2H-tetrazole-5-yl)-1H-pyrazole-5-sulfonamide (AZS), was studied in water at different wavelengths and in the presence of photocatalysts. AZS was rapidly degraded by UV light, affording three photoproducts. The main product, accounting for about 70% of photodegraded herbicide, was identified as 6-amino-5-[(4,6-dimethoxypyrimidin-2-yl)methylamino]-1,5,6,8-tetrahydro-7-oxa-8lambda(6)-tia-1,2,5,6-tetraza-azulen-4-one (ADTA) by single-crystal X-ray diffraction...
August 8, 2007: Journal of Agricultural and Food Chemistry
https://www.readbyqxmd.com/read/17431857/studies-of-interactions-between-silane-coupling-agents-and-cellulose-fibers-with-liquid-and-solid-state-nmr
#12
Marie-Christine B Salon, Guillaume Gerbaud, M Abdelmouleh, Cécile Bruzzese, Sami Boufi, Mohamed N Belgacem
The hydrolysis of three alkoxy-silane coupling agents, gamma-methacryloxypropyl trimethoxy silane (MPS), gamma-aminopropyl triethoxy silane (APS), and gamma-diethylenetriaminopropyl trimethoxy silane (TAS), was carried out in ethanol/water solutions (80/20 w/w) at different pH values and followed by 1H, 13C and 29Si NMR spectroscopy. Acidic media were found to stabilize the hydrolyzed forms. As expected, the formation of silanol groups was followed by their self-condensation to generate oligomeric structures, yielding, ultimately, solid homopolycondensated structures, as analyzed by 29Si and 13C high-resolution solid-state NMR...
June 2007: Magnetic Resonance in Chemistry: MRC
https://www.readbyqxmd.com/read/16715104/haematopoietic-cell-transplantation-hct-in-combination-with-enzyme-replacement-therapy-ert-in-patients-with-hurler-syndrome
#13
MULTICENTER STUDY
J Cox-Brinkman, J-J Boelens, J E Wraith, A O'meara, P Veys, F A Wijburg, N Wulffraat, R F Wynn
Hurler syndrome (MPS 1H) is the severe form of mucopolysaccharidosis type 1 (MPS 1). Haematopoietic cell transplantation (HCT) is the treatment of choice, but carries a high incidence of graft failure and morbidity. The use of enzyme replacement therapy (ERT) might improve the clinical signs and symptoms before HCT, resulting in less transplantation-related complications. Moreover, clearance of glycosaminoglycans (GAG's) from the bone marrow might improve engraftment. Twenty-two patients with MPS 1H received one or more HCT procedures in combination with ERT...
July 2006: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/16104751/solute-solvent-interactions-and-chiral-induction-in-liquid-crystals
#14
Giorgio Celebre, Giuseppina De Luca, Michela Maiorino, Francesca Iemma, Alberta Ferrarini, Silvia Pieraccini, Gian Piero Spada
The induction of a cholesteric phase by doping an achiral nematic liquid crystal with an enantiopure solute is a phenomenon that, as in all general supramolecular phenomena of chiral amplification, depends in a subtle way on intermolecular interactions. The micrometric helical deformation of the phase director in the cholesteric phase is generated by the interplay of anisotropy and chirality of probe-medium interactions. In the case of a flexible chiral dopant, the solvent can influence the twisting power in two ways, difficult to disentangle: it is responsible for the solute orientational order, an essential ingredient for the emergence of phase chirality; but also it can affect the dopant conformational distribution and then the chirality of the structures present in the solution...
August 24, 2005: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/15907861/silane-adsorption-onto-cellulose-fibers-hydrolysis-and-condensation-reactions
#15
Marie-Christine Brochier Salon, Makki Abdelmouleh, Sami Boufi, Mohamed Naceur Belgacem, Alessandro Gandini
The hydrolysis of three alkoxysilane coupling agents, gamma-methacryloxypropyltrimethoxysilane (MPS), gamma-aminopropyltriethoxysilane (APS), and gamma-diethylenetriaminopropyltrimethoxysilane (TAS), was carried out in an ethanol/water (80/20) solution and followed by 1H, 13C, and 29Si NMR spectroscopy, which showed that its rate increased in the order MPS < APS < TAS. The formation of the silanol groups was followed by their self-condensation to generate oligomeric structure. APS and MPS only gave soluble products, whereas colloidal particles precipitated in the medium when TAS was hydrolyzed...
September 1, 2005: Journal of Colloid and Interface Science
https://www.readbyqxmd.com/read/14558120/two-dimensional-nmr-spectroscopy-of-urinary-glycosaminoglycans-from-patients-with-different-mucopolysaccharidoses
#16
Michel Hochuli, Kurt Wüthrich, Beat Steinmann
Patients with different types of mucopolysaccharidoses (MPS) lack specific lysosomal enzymes, which leads to tissue accumulation and urinary excretion of glycosaminoglycans (GAGs). Since little is known about the molecular composition of the excreted GAG fragments, we used two-dimensional [1H,13C]-correlation nuclear magnetic resonance (NMR) spectroscopy for a detailed analysis of the urinary GAGs of patients with MPS types I, II, IIIA, IVA and VI. The method revealed that the molecular structures of the excreted GAGs, i...
June 2003: NMR in Biomedicine
https://www.readbyqxmd.com/read/11641441/block-of-cloned-human-t-type-calcium-channels-by-succinimide-antiepileptic-drugs
#17
J C Gomora, A N Daud, M Weiergräber, E Perez-Reyes
Inhibition of T-type Ca(2+) channels has been proposed to play a role in the therapeutic action of succinimide antiepileptic drugs. Despite the widespread acceptance of this hypothesis, recent studies using rat and cat neurons have failed to confirm inhibition of T-type currents at therapeutically relevant concentrations. The present study re-examines this issue using the three cloned human channels that constitute the T-type family: alpha 1G, alpha 1H, and alpha 1I. The cloned cDNAs were stably transfected and expressed into mammalian cells, leading to the appearance of typical T-type currents...
November 2001: Molecular Pharmacology
https://www.readbyqxmd.com/read/11265555/-mitral-stenosis-secondary-to-hurler-s-syndrome
#18
S Kraiem, D Lahidheb, N Chehaibi, A Sfaxi, M Terras, Y Ben Ameur, M L Slimane
Valvular disease in mucopolysaccharidosis type I-Hurler (MPS/1H) is relatively common, but mitral stenosis is very rare in this genetic abnormality. The authors describe the case of a 16-year old girl with Hurler's syndrome diagnosed at 4 years of age. The morphological features were characteristic: bridged nose, thickened lips, macroglassia, short neck (gargoylism, short, thick fingers and limitation of brachial and fore-arm flexion. She presented with stage II dyspnoea and paroxysmal nocturnal dyspnoea. Radiological and echocardiographic studies revealed severe mitral stenosis with haemodynamic complications requiring mitral valve replacement...
February 2001: Archives des Maladies du Coeur et des Vaisseaux
https://www.readbyqxmd.com/read/10929365/correction-of-odontoid-dysplasia-following-bone-marrow-transplantation-and-engraftment-in-hurler-syndrome-mps-1h
#19
S H Hite, C Peters, W Krivit
BACKGROUND: Odontoid dysplasia is recognized as a major component of the constellation of dysostosis multiplex lesions associated with Hurler's syndrome (MPS 1H). Because of this abnormality, there is an increased risk of atlantoaxial subluxation with potential cervical spinal cord injury. A significant alteration of the natural history of the disease with respect to the visceral, cardiac, and skeletal systems has resulted in an increased life span for MPS 1H patients associated with engraftment from normal donors...
July 2000: Pediatric Radiology
https://www.readbyqxmd.com/read/10568838/evidence-for-mutualism-between-a-plant-growing-in-a-phosphate-limited-desert-environment-and-a-mineral-phosphate-solubilizing-mps-rhizobacterium
#20
Goldstein, Braverman, Osorio
Alkaline desert soils are high in insoluble calcium phosphates but deficient in soluble orthophosphate (Pi) essential for plant growth. In this extreme environment, one adaptive strategy could involve specific associations between plant roots and mineral phosphate solubilizing (MPS) bacteria. The most efficient MPS phenotype in Gram-negative bacteria results from extracellular oxidation of glucose to gluconic acid via the quinoprotein glucose dehydrogenase. A unique bacterial population isolated from the roots of Helianthus annus jaegeri growing at the edge of an alkaline dry lake in the Mojave Desert showed no MPS activity and no gluconic acid production...
December 1, 1999: FEMS Microbiology Ecology
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