Search results for "placement"

showing 10 items of 1142 documents

Enzyme replacement and gene therapy for mucopolysaccharidoses: current progress and future directions

2015

Introduction: Mucopolysaccharidoses (MPS) are lysosomal storage disorders caused by the deficiency of enzymes that are responsible for the stepwise degradation of complex carbohydrates, the glycosaminoglycans. Whereas in the past the treatment of MPS consisted mainly of palliative care, enzyme replacement therapy (ERT) is now possible for some MPS disorders, and in the future many other therapeutic options will become available.Areas covered: This review, based on personal experience and the currently available literature, will give an overview on the efficacy and limitations of ERT and will discuss new therapeutic approaches, such as anti-inflammatory drugs, substrate reduction therapy, ch…

congenital hereditary and neonatal diseases and abnormalitiesPalliative carebusiness.industryHealth PolicyGenetic enhancementmedicine.medical_treatmentnutritional and metabolic diseasesLysosomal storage disordersHematopoietic stem cell transplantationEnzyme replacement therapyBioinformaticsImmunologymedicinePharmacology (medical)Substrate reduction therapybusinessPharmacology Toxicology and Pharmaceutics (miscellaneous)Expert Opinion on Orphan Drugs
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Outcome of patients with classical infantile pompe disease receiving enzyme replacement therapy in Germany

2015

Enzyme replacement therapy (ERT) has been shown to improve outcome in classical infantile Pompe disease. The purpose of this study was to assess mortality, morbidity, and shortcomings of ERT in a larger cohort of patients treated outside clinical trials. To accomplish this, we retrospectively analyzed the data of all 23 subjects with classical infantile Pompe disease having started ERT in Germany between January 2003 and December 2010.Ten patients (43%) deceased and four others (17%) became ventilator dependent. Seven infants (30.5%) made no motor progress at all, while seven (30.5%) achieved free sitting, and nine (39%) gained free walking. Besides all the seven patients (100%) attaining n…

congenital hereditary and neonatal diseases and abnormalitiesPediatricsmedicine.medical_specialtybusiness.industryMEDLINEnutritional and metabolic diseases610 Medicine & healthDiseaseMetabolic myopathyEnzyme replacement therapymedicine.disease1301 Biochemistry Genetics and Molecular Biology (miscellaneous)ArticleClinical trial2712 Endocrinology Diabetes and Metabolism10036 Medical Clinic2724 Internal MedicineCohortmedicineGlycogen storage diseasebusiness
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Systemic therapies for mucopolysaccharidosis: ocular changes following haematopoietic stem cell transplantation or enzyme replacement therapy - a rev…

2010

The management of mucopolysaccharidosis (MPS) is focused on the multi-organ, sometimes life-threatening, clinical manifestations that occur over time. In the past, the limited, symptom-based treatment options led physicians to adopt a palliative approach towards individual disease-associated complications. The availability of systemic treatments such as haematopoietic stem cell transplantation (HSCT) and enzyme replacement therapy (ERT) has created a better prognosis for MPS patients, particularly when initiated early in life. As part of an integrated management approach, these therapies could be valuable in managing the ocular features that are present in many children with MPS. HSCT has b…

congenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyVisual acuitygenetic structuresbusiness.industryMucopolysaccharidosismedicine.medical_treatmentEye diseasenutritional and metabolic diseasesEnzyme replacement therapyHematopoietic stem cell transplantationmedicine.diseaseeye diseasesSurgeryTransplantationOphthalmologymedicineOptic nervesense organsStem cellmedicine.symptomIntensive care medicinebusinessClinical & Experimental Ophthalmology
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Emplazamiento de producto en bestsellers literarios: efectos de la familiaridad con la marca, la repetición y la congruencia sobre la memoria de los …

2015

El emplazamiento de producto es una herramienta emergente de las comunicaciones de marketing que se enmarca en una tendencia general de mercado en la que las líneas entre contenido de entretenimiento y publicidad se difuminan. A pesar de que en los últimos años el emplazamiento de producto ha suscitado un creciente interés tanto entre académicos como entre profesionales del marketing los trabajos académicos sobre el emplazamiento de producto en los medios impresos, y especialmente los trabajos de naturaleza empírica, son prácticamente inexistentes existiendo por tanto un vacío de investigación que debe ser atendido. Esta tesis tiene como objetivo principal aportar nuevo conocimiento a la ef…

congruenciarecuerdo de marcaproduct placementrepeticiónUNESCO::CIENCIAS ECONÓMICASemplazamiento de productoreconocimiento de marcabestsellers literarios:CIENCIAS ECONÓMICAS [UNESCO]familiaridad
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Exposing ecological and economic costs of the research-implementation gap and compromises in decision making

2018

The frequently discussed gap between conservation science and practice is manifest in the gap between spatial conservation prioritization plans and their implementation. We analyzed the research-implementation gap of one zoning case by comparing results of a spatial prioritization analysis aimed at avoiding ecological impact of peat mining in a regional zoning process with the final zoning plan. We examined the relatively complex planning process to determine the gaps among research, zoning, and decision making. We quantified the ecological costs of the differing trade-offs between ecological and socioeconomic factors included in the different zoning suggestions by comparing the landscape-l…

conservation implementationympäristöohjelmattaloudelliset vaikutuksetreplacement costpäätöksentekoinverse resource allocation problempeat miningluonnonsuojeluimpact avoidanceZonation software
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Management of Acute Kidney Injury and Extracorporeal Blood Purification Therapies During the COVID-19 Pandemic: The Italian SIN–SIAARTI Joint Survey …

2022

Background and AimThe novel coronavirus disease 2019 remains challenging. A large number of hospitalized patients are at a high risk of developing AKI. For this reason, we conducted a nationwide survey to assess the incidence and management of AKI in critically ill patients affected by the SARS-CoV-2 infection.MethodsThis is a multicenter, observational, nationwide online survey, involving the Italian Society of Nephrology and the critical care units in Italy, developed in partnership between the scientific societies such as SIN and SIAARTI. Invitations to participate were distributed through emails and social networks. Data were collected for a period of 1 week during the COVID-19 pandemic…

critical careCOVID-19; acute kidney injury; blood purification; critical care; renal replacement therapy; surveys and questionnairesacute kidney injurysurveys and questionnairesCOVID-19blood purificationGeneral Medicineurologic and male genital diseasesrenal replacement therapyFrontiers in Medicine
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Dibromido[N-(1-diethylamino-1-oxo-3-phenylpropan-2-yl)-N′-(pyridin-2-yl)imidazol-2-ylidene]palladium(II) dichloromethane monosolvate

2019

In the molecule of the title N,N′-disubstituted imidazol-2-ylidene palladium(II) complex, [PdBr2(C21H24N4O)]·CH2Cl2, the palladium(II) atom adopts a slightly distorted square-planar coordination (r.m.s. deviation = 0.0145 Å), and the five-membered chelate ring is almost planar [maximum displacement = 0.015 (8) Å]. The molecular conformation is enforced by intramolecular C—H...Br hydrogen bonds. In the crystal, complex molecules and dichloromethane molecules are linked into a three-dimensional network by C—H...O and C—H...Br hydrogen bonds.

crystal structureHydrogen bondchemistry.chemical_element02 engineering and technologyGeneral MedicineCrystal structure010402 general chemistry021001 nanoscience & nanotechnologyRing (chemistry)palladium01 natural sciencesMedicinal chemistryMethane0104 chemical scienceschemistry.chemical_compoundchemistrylcsh:QD901-999Chelationlcsh:Crystallography0210 nano-technologyMaximum displacementimidazol-2-ylidenePalladiumIUCrData
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Crystal structure of (E)-pent-2-enoic acid

2015

The molecule of the title compound, C5H8O2, a low-melting α,β-unsaturated carboxylic acid, is essentially planar [maximum displacement = 0.0239 (13) Å]. In the crystal, molecules are linked into centrosymmetric dimersviapairs of O—H...O hydrogen bonds.

crystal structurehydrogen bondunsaturated carb­oxy­lic acidHydrogen bondDimerGeneral ChemistryCrystal structureCondensed Matter Physicscomputer.software_genredimerData ReportsCrystallcsh:ChemistryCrystallographychemistry.chemical_compoundPlanarchemistrylcsh:QD1-999General Materials ScienceData miningMaximum displacementcomputerunsaturated carboxylic acidActa Crystallographica Section E: Crystallographic Communications
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An analysis of model predictive control with integral action applied to digital displacement cylinders

2020

This article aims to analyze Model Predictive Control (MPC) for the control of multi-chamber cylinders. MPC with and without integral action has been introduced. Three different algorithms have been used to solve the optimization problem in the MPC. The different algorithms have been compared with an industrial solver. The influence of changing mass, choosing a different middle line pressure, system delays, signal noise, velocity estimation, and changing pressure levels has been investigated. It is concluded that for the small prediction horizon used in the paper a simple algorithm such as A can produce results as good as the previously used Differential Evolution algorithm in less than hal…

digital displacement cylindersOptimizationbusiness.industryStructural engineeringVDP::Mechanical engineering: 570lcsh:QA75.5-76.95Computer Science ApplicationsDigital Displacement CylindersIntegral actionModel predictive controlModell-prediktiv reguleringControl and Systems EngineeringVDP::Maskinfag: 570Modeling and SimulationOptimaliseringDisplacement (orthopedic surgery)lcsh:Electronic computers. Computer scienceModel predictive controlbusinessSoftwareModel Predictive ControlMathematics
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Gingival displacement using diode laser or retraction cords: A comparative clinical study.

2018

PURPOSE: To compare two systems used for conditioning the gingival sulcus and exposing the finish line before the final impression for a fixed denture: retraction cords and diode laser. METHODS: All subjects participating in the study had healthy gingival and periodontal status before intervention for fixed prosthesis. 74 abutments for complete crown restoration were randomly divided into two groups for displacing the gingival sulcus before the final impression: gingival retraction cords (RC) and diode laser (DL). The height of the clinical crowns was measured by a blinded examiner in three points of the buccal surface (mesial, midline and distal) at four different times: after tooth prepar…

diode laserSettore MED/28 - Malattie Odontostomatologicheretraction cordsgingival displacement
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