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https://www.readbyqxmd.com/read/28098417/delivery-of-anesthesia-for-children-with-mucopolysaccharidosis-type-iii-sanfilippo-syndrome-a-review-of-86-anesthetics
#1
Marc A Cohen, Grant M Stuart
BACKGROUND: Sanfilippo syndrome (MPS III) is rare, with 97 cases in the United Kingdom between 1988 and 1998. Mucopolysaccharide infiltration of tissues in mucopolysaccharidosis (MPS) causes multi-systemic pathology including difficult airways and cardiac disease. Published anesthesia case reviews of Sanfilippo syndrome have included limited numbers of patients to date. AIM: To identify the perioperative management and complications of anesthesia in children with mucopolysaccharidosis Type III at Great Ormond Street Hospital...
January 18, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28096000/effects-of-oral-care-with-glutamine-in-preventing-ventilator-associated-pneumonia-in-neurosurgical-intensive-care-unit-patients
#2
Hatice Kaya, Yüksel Turan, Yusuf Tunalı, Gülsün Özdemir Aydın, Neslihan Yüce, Şengül Gürbüz, Keziban Tosun
PURPOSE: The study was designed as a randomized, controlled, experimental study aiming to determine the effects of oral care with glutamine in preventing ventilator-associated pneumonia in patients admitted to neurosurgical intensive care unit. METHODS: The universe consisted of patients who are admitted to neurosurgical intensive care unit between January 2014 and August 2015, while the sample consisted of 88 patients who fulfilled the inclusion criteria and were randomly selected...
February 2017: Applied Nursing Research: ANR
https://www.readbyqxmd.com/read/28092382/a-vascularized-and-perfused-organ-on-a-chip-platform-for-large-scale-drug-screening-applications
#3
Duc T T Phan, Xiaolin Wang, Brianna M Craver, Agua Sobrino, Da Zhao, Jerry C Chen, Lilian Y N Lee, Steven C George, Abraham P Lee, Christopher C W Hughes
There is a growing awareness that complex 3-dimensional (3D) organs are not well represented by monolayers of a single cell type - the standard format for many drug screens. To address this deficiency, and with the goal of improving screens so that drugs with good efficacy and low toxicity can be identified, microphysiological systems (MPS) are being developed that better capture the complexity of in vivo physiology. We have previously described an organ-on-a-chip platform that incorporates perfused microvessels, such that survival of the surrounding tissue is entirely dependent on delivery of nutrients through the vessels...
January 16, 2017: Lab on a Chip
https://www.readbyqxmd.com/read/28089550/assessment-of-microplastic-sorbed-contaminant-bioavailability-through-analysis-of-biomarker-gene-expression-in-larval-zebrafish
#4
Victoria A Sleight, Adil Bakir, Richard C Thompson, Theodore B Henry
Microplastics (MPs) are prevalent in marine ecosystems. Because toxicants (termed here "co-contaminants") can sorb to MPs, there is potential for MPs to alter co-contaminant bioavailability. Our objective was to demonstrate sorption of two co-contaminants with different physicochemistries [phenanthrene (Phe), log10Kow=4.57; and 17α-ethinylestradiol (EE2), log10Kow=3.67] to MPs; and assess whether co-contaminant bioavailability was increased after MP settlement. Bioavailability was indicated by gene expression in larval zebrafish...
January 12, 2017: Marine Pollution Bulletin
https://www.readbyqxmd.com/read/28088454/alpha-l-iduronidase-and-arylsulfatase-b-in-dried-blood-spots-on-filter-paper-biochemical-parameters-and-time-stability
#5
Ana Carolina Breier, Jaqueline Cé, Jamila Mezzalira, Vanessa V Daitx, Vitoria C Moraes, Mariana P S Goldim, Janice C Coelho
BACKGROUND: The goal of this study was to assess the biochemical parameters of the enzymes α-l-iduronidase (IDUA) and arylsulfatase B (ASB), which are deficient in mucopolysaccharidosis (MPS) I and VI, respectively, in dried blood spot (DBS) samples impregnated on filter paper. METHODS AND RESULTS: The optimal pH, Km, and Vmax of IDUA and ASB in DBS are hereby presented. After these analyses, the reference values for the activities of these enzymes in DBS with cutoff of 3...
January 11, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28081172/influence-of-corneal-opacity-on-intraocular-pressure-assessment-in-patients-with-lysosomal-storage-diseases
#6
Joanna Wasielica-Poslednik, Giuseppe Politino, Irene Schmidtmann, Katrin Lorenz, Katharina Bell, Norbert Pfeiffer, Susanne Pitz
AIMS: To investigate an influence of mucopolysaccharidosis (MPS)- and Morbus Fabry-associated corneal opacities on intraocular pressure (IOP) measurements and to evaluate the concordance of the different tonometry methods. METHODS: 25 MPS patients with or without corneal clouding, 25 Fabry patients with cornea verticillata ≥ grade 2 and 25 healthy age matched controls were prospectively included into this study. Outcome measures: Goldmann applanation tonometry (GAT); palpatory assessment of IOP; Goldmann-correlated intraocular pressure (IOPg), corneal-compensated intraocular pressure (IOPcc), corneal resistance factor (CRF) and corneal hysteresis (CH) assessed by Ocular Response Analyzer (ORA); central corneal thickness (CCT) and density assessed with Pentacam...
2017: PloS One
https://www.readbyqxmd.com/read/28077431/enantiomers-recognition-of-propranolol-based-on-organic-inorganic-hybrid-open-tubular-mips-cec-column-using-3-trimethoxysilyl-propyl-methacrylate-as-a-cross-linking-monomer
#7
Guo-Ning Chen, Ning Li, Tian Luo, Yu-Ming Dong
In this study, 3-(trimethoxysilyl)propyl methacrylate (γ-MPS), a bifunctional group compound, was used as a single cross-linking agent to prepare molecular imprinted inorganic-organic hybrid polymers by in situ polymerization for open-tubular capillary electro chromatography (CEC) column. The optimal preparation conditions were: the ratio between template molecule and functional monomer was 1:4; the volume proportion of porogen toluene and methanol was 1:1 and the volume of cross-linking agent γ-MPS was 69 μL...
January 10, 2017: Journal of Chromatographic Science
https://www.readbyqxmd.com/read/28059427/evaluation-of-peg-coated-iron-oxide-nanoparticles-as-blood-pool-tracers-for-preclinical-magnetic-particle-imaging
#8
A P Khandhar, P Keselman, S J Kemp, R M Ferguson, P W Goodwill, S M Conolly, K M Krishnan
Superparamagnetic iron oxide (SPIO) nanoparticles with optimized and well-characterized properties are critical for Magnetic Particle Imaging (MPI). MPI is a novel in vivo imaging modality that promises to integrate the speed of X-ray CT, safety of MRI and sensitivity of PET. Since SPIOs are the source of MPI signal, both the core and surface properties must be optimized to enable efficient in vivo imaging with pharmacokinetics tailored for specific imaging applications. Existing SPIOs like Resovist (ferucarbotran) provide a suboptimal MPI signal, and further limit MPI's in vivo utility due to rapid systemic clearance...
January 19, 2017: Nanoscale
https://www.readbyqxmd.com/read/28054207/mortality-after-hematopoietic-stem-cell-transplantation-for-severe-mucopolysaccharidosis-type-i-the-30-year-university-of-minnesota-experience
#9
Nathan J Rodgers, Alexander M Kaizer, Weston P Miller, Kyle D Rudser, Paul J Orchard, Elizabeth A Braunlin
BACKGROUND AND AIM: Mucopolysaccharidosis IH (MPS IH, Hurler syndrome) naturally leads to death within the first decade of life, primarily from cardiac and pulmonary causes. To determine how hematopoietic stem cell transplantation (HSCT) has altered mortality, we analyzed our institution's 30-year experience of patients with MPS IH undergoing HSCT. METHODS: Using chart review and the National Death Index, we determined survival status of 134 patients (males = 69) with MPS IH transplanted between 9/16/1983 and 7/25/2013 on 12/31/2013...
January 4, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28053529/co-self-assembly-of-cationic-microparticles-to-deliver-pegfp-znf580-for-promoting-the-transfection-and-migration-of-endothelial-cells
#10
Yakai Feng, Mengyang Guo, Wen Liu, Xuefang Hao, Wei Lu, Xiangkui Ren, Changcan Shi, Wencheng Zhang
The gene transfection efficiency of polyethylenimine (PEI) varies with its molecular weight. Usually, high molecular weight of PEI means high gene transfection, as well as high cytotoxicity in gene delivery in vivo. In order to enhance the transfection efficiency and reduce the cytotoxicity of PEI-based gene carriers, a novel cationic gene carrier was developed by co-self-assembly of cationic copolymers. First, a star-shaped copolymer poly(3(S)-methyl-morpholine-2,5-dione-co-lactide) (P(MMD-co-LA)) was synthesized using D-sorbitol as an initiator, and the cationic copolymer (P(MMD-co-LA)-g-PEI) was obtained after grafting low-molecular weight PEI...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28052584/activated-platelet-derived-and-leukocyte-derived-circulating-microparticles-and-the-risk-of-thrombosis-in-heparin-induced-thrombocytopenia-a-role-for-pf4-bearing-microparticles
#11
Elena Campello, Claudia M Radu, Elena Duner, Anna M Lombardi, Luca Spiezia, Raffaele Bendo, Silvia Ferrari, Paolo Simioni, Fabrizio Fabris
BACKGROUND: Though the presence of platelets-derived microparticles (MPs) have previously been described in heparin-induced thrombocytopenia (HIT), the mechanism of thrombosis in HIT remains poorly understood. We aimed to asses the presence and origin of MPs in patients with HIT and their possible contribution to HIT with thrombosis (HITT). METHODS: Forty-five patients with HIT and 45 matched hospitalized patients with not confirmed HIT (HIT-negative) were enrolled...
January 3, 2017: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/28050459/cardiovascular-abnormalities-in-egyptian-children-with-mucopolysaccharidoses
#12
Laila Selim, Nehal Abdelhamid, Emad Salama, Amera Elbadawy, Iman Gamaleldin, Mohamed Abdelmoneim, Abeer Selim
INTRODUCTION: The Mucopolysaccharidoses (MPS) are rare inherited metabolic disorders. They are characterized by the progressive systemic deposition of Glycosaminoglycans (GAGs). GAGs accumulate in the myocardium and the cardiac valves. Enzyme Replacement Therapy (ERT) is available for MPS I, II, and VI. However, ERT does not appear to improve cardiac valve disease in patients with valve disease present at the start of ERT. AIM: To evaluate the cardiac involvement in Egyptian children with MPS...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28045254/homogeneous-catalysis-under-ultradilute-conditions-taml-naclo-oxidation-of-persistent-metaldehyde
#13
Liang L Tang, Matthew A DeNardo, Christopher J Schuler, Matthew R Mills, Chakicherla Gayathri, Roberto R Gil, Rakesh Kanda, Terrence J Collins
TAML activators enable homogeneous oxidation catalysis where the catalyst and substrate (S) are ultradilute (pM-low μM) and the oxidant is very dilute (high nM-low mM). Water contamination by exceptionally persistent micropollutants (MPs), including metaldehyde (Met), provides an ideal space for determining the characteristics and utilitarian limits of this ultradilute catalysis. The low MP concentrations decrease throughout catalysis with S oxidation (kII) and catalyst inactivation (ki) competing for the active catalyst...
January 18, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28036084/preferential-enhancement-of-sensory-and-motor-axon-regeneration-by-combining-extracellular-matrix-components-with-neurotrophic-factors
#14
Daniel Santos, Francisco González-Pérez, Guido Giudetti, Silvestro Micera, Esther Udina, Jaume Del Valle, Xavier Navarro
After peripheral nerve injury, motor and sensory axons are able to regenerate but inaccuracy of target reinnervation leads to poor functional recovery. Extracellular matrix (ECM) components and neurotrophic factors (NTFs) exert their effect on different neuronal populations creating a suitable environment to promote axonal growth. Here, we assessed in vitro and in vivo the selective effects of combining different ECM components with NTFs on motor and sensory axons regeneration and target reinnervation. Organotypic cultures with collagen, laminin and nerve growth factor (NGF)/neurotrophin-3 (NT3) or collagen, fibronectin and brain-derived neurotrophic factor (BDNF) selectively enhanced sensory neurite outgrowth of DRG neurons and motor neurite outgrowth from spinal cord slices respectively...
December 29, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28018442/long-term-clinical-course-of-a-patient-with-mucopolysaccharidosis-type-iiib
#15
Ja Hye Kim, Yang Hyun Chi, Gu-Hwan Kim, Han-Wook Yoo, Jun Hwa Lee
Mucopolysaccharidosis type III (MPS III) is a rare genetic disorder caused by lysosomal storage of heparan sulfate. MPS IIIB results from a deficiency in the enzyme alpha-N-acetyl-D-glucosaminidase (NAGLU). Affected patients begin showing behavioral changes, progressive profound mental retardation, and severe disability from the age of 2 to 6 years. We report a patient with MPS IIIB with a long-term follow-up duration. He showed normal development until 3 years. Subsequently, he presented behavioral changes, sleep disturbance, and progressive motor dysfunction...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28004441/referred-symptom-from-myofascial-pain-syndrome-one-of-the-most-important-causes-of-sensory-disturbance-in-breast-cancer-patients-using-taxanes
#16
E J Ko, J Y Jeon, W Kim, J Y Hong, Y G Yi
The aim of this retrospective study was to evaluate common causes of upper extremity sensory disturbance in breast cancer patients. Breast cancer patients who received surgery and taxane chemotherapy (CTx) with upper extremity sensory disturbance that began after CTx were included. With comprehensive clinical history, physical examination and electrodiagnostic results, diagnosis for each patient was made. Fifty-two patients were included: 23 (44.2%) were diagnosed with chemotherapy-induced peripheral neuropathy (CIPN), 7 (13...
December 22, 2016: European Journal of Cancer Care
https://www.readbyqxmd.com/read/28004150/assessment-of-urinary-microparticles-in-normotensive-patients-with-type-1-diabetes
#17
Yuliya Lytvyn, Fengxia Xiao, Christopher R J Kennedy, Bruce A Perkins, Heather N Reich, James W Scholey, David Z Cherney, Dylan Burger
AIMS/HYPOTHESIS: Assessment of urinary extracellular vesicles including exosomes and microparticles (MPs) is an emerging approach for non-invasive detection of renal injury. We have previously reported that podocyte-derived MPs are increased in diabetic mice in advance of albuminuria. Here, we hypothesised that type 1 diabetes and acute hyperglycaemia would increase urinary podocyte MP levels in uncomplicated diabetes. METHODS: In this post hoc exploratory analysis, we examined archived urine samples from normoalbuminuric patients with uncomplicated type 1 diabetes studied under clamped euglycaemia and hyperglycaemia and compared with healthy controls...
December 21, 2016: Diabetologia
https://www.readbyqxmd.com/read/27997116/cross-linked-fluorescent-supramolecular-nanoparticles-as-finite-tattoo-pigments-with-controllable-intradermal-retention-times
#18
Jin-Sil Choi, Yazhen Zhu, Hongsheng Li, Parham Peyda, Thuy Tien Nguyen, Mo Yuan Shen, Yang Michael Yang, Jingyi Zhu, Mei Liu, Mandy M Lee, Shih-Sheng Sun, Yang Yang, Hsiao-Hua Yu, Kai Chen, Gary S Chuang, Hsian-Rong Tseng
Tattooing has been utilized by the medical community for precisely demarcating anatomic landmarks. This practice is especially important for identifying biopsy sites of nonmelanoma skin cancer (NMSC) due to the long interval (i.e., up to 3 months) between the initial diagnostic biopsy and surgical treatment. Commercially available tattoo pigments possess several issues, which include causing poor cosmesis, being mistaken for a melanocytic lesion, requiring additional removal procedures when no longer desired, and potentially inducing inflammatory responses...
December 20, 2016: ACS Nano
https://www.readbyqxmd.com/read/27991992/neurological-outcomes-after-hematopoietic-stem-cell-transplantation-for-cerebral-x-linked-adrenoleukodystrophy-late-onset-metachromatic-leukodystrophy-and-hurler-syndrome
#19
Jonas Alex Morales Saute, Carolina Fischinger Moura de Souza, Fabiano de Oliveira Poswar, Karina Carvalho Donis, Lillian Gonçalves Campos, Adriana Vanessa Santini Deyl, Maira Graeff Burin, Carmen Regla Vargas, Ursula da Silveira Matte, Roberto Giugliani, Maria Luiza Saraiva-Pereira, Leonardo Modesti Vedolin, Lauro José Gregianin, Laura Bannach Jardim
Objective: To describe survival and neurological outcomes after HSCT for these disorders. Methods: Seven CALD, 2 MLD and 2 MPS-IH patients underwent HSCT between 2007 and 2014. Neurological examinations, magnetic resonance imaging, molecular and biochemical studies were obtained at baseline and repeated when appropriated. Results: Favorable outcomes were obtained with 4/5 related and 3/6 unrelated donors. Two patients died from procedure-related complications...
December 2016: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/27981585/improving-the-water-solubility-of-monascus-pigments-under-acidic-conditions-with-gum-arabic
#20
Wenjie Jian, Yuanming Sun, Jian-Yong Wu
BACKGROUND: Monascus pigments (Mps) are natural food colorants and their stability in acidic solutions is important for application in the food industry. This study aimed to evaluate the use of gum arabic (GA) as a stabilizer for maintaining the solubility of Mps in an acidic aqueous solution exposed to a high temperature, and to analyze the molecular interactions between GA and Mps. RESULTS: Mps dispersed (0.2 g kg(-1) ) in deionized water at pH 3.0-4.0 without GA formed precipitates but remained in a stable solution in the presence of GA (1 g kg(-1) )...
November 7, 2016: Journal of the Science of Food and Agriculture
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