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https://www.readbyqxmd.com/read/28729303/amotosalen-uva-pathogen-inactivation-technology-reduces-platelet-activatability-induces-apoptosis-and-accelerates-clearance
#1
Simona Stivala, Sara Gobbato, Laura Infanti, Martin F Reiner, Nicole Bonetti, Sara C Meyer, Giovanni G Camici, Thomas F Lüscher, Andreas Buser, Jürg H Beer
Amotosalen and ultraviolet A photochemical-based pathogen reduction using the InterceptTM Blood System is an effective and established technology for platelet and plasma components, which is adopted in more than 40 countries worldwide. Several reports point towards a reduced platelet function after Amotosalen/UVA exposure. The current study was undertaken to identify the mechanisms responsible for the early impairment of platelet function by the InterceptTM Blood System. Twenty-five platelet apheresis units were collected from healthy volunteers following standard procedures and split into 2 components, one untreated and the other treated with Amotosalen/UVA...
July 20, 2017: Haematologica
https://www.readbyqxmd.com/read/28728811/newborn-screening-for-lysosomal-storage-disorders-in-illinois-the-initial-15-month-experience
#2
Barbara K Burton, Joel Charrow, George E Hoganson, Darrell Waggoner, Brad Tinkle, Stephen R Braddock, Michael Schneider, Dorothy K Grange, Claudia Nash, Heather Shryock, Rebecca Barnett, Rong Shao, Khaja Basheeruddin, George Dizikes
OBJECTIVES: To assess the outcomes of newborn screening for 5 lysosomal storage disorders (LSDs) in the first cohort of infants tested in the state of Illinois. STUDY DESIGN: Tandem mass spectrometry was used to assay for the 5 LSD-associated enzymes in dried blood spot specimens obtained from 219 973 newborn samples sent to the Newborn Screening Laboratory of the Illinois Department of Public Health in Chicago. RESULTS: The total number of cases with a positive diagnosis and the incidence for each disorder were as follows: Fabry disease, n = 26 (1 in 8454, including the p...
July 17, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28723748/clinical-characteristics-and-mutation-spectrum-of-gla-in-korean-patients-with-fabry-disease-by-a-nationwide-survey-underdiagnosis-of-late-onset-phenotype
#3
Jin-Ho Choi, Beom Hee Lee, Sun Hee Heo, Gu-Hwan Kim, Yoo-Mi Kim, Dae-Seong Kim, Jung Min Ko, Young Bae Sohn, Yong Hee Hong, Dong-Hwan Lee, Hoon Kook, Han Hyuk Lim, Kyung Hee Kim, Woo-Shik Kim, Geu-Ru Hong, Su-Hyun Kim, Sang Hyun Park, Chan-Duck Kim, So Mi Kim, Jeong-Sook Seo, Han-Wook Yoo
Fabry disease is a rare X-linked lysosomal storage disorder caused by an α-galactosidase A deficiency. The progressive accumulation of globotriaosylceramide (GL-3) results in life-threatening complications, including renal, cardiac, and cerebrovascular diseases. This study investigated the phenotypic and molecular spectra of GLA mutations in Korean patients with Fabry disease using a nationwide survey.This study included 94 patients from 46 independent pedigrees: 38 adult males, 46 symptomatic females, and 10 pediatric males...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28720788/preservative-solution-that-stabilizes-erythrocyte-morphology-and-leukocyte-viability-under-ambient-conditions
#4
Rebecca D Sandlin, Keith H K Wong, Leo Boneschansker, Thomas R Carey, Kathleen L Miller, Gregory Rose, Daniel A Haber, Shyamala Maheswaran, Daniel Irimia, Shannon L Stott, Mehmet Toner
The deterioration of whole blood ex vivo represents a logistical hurdle in clinical and research settings. Here, a cocktail preservative is described that stabilizes leukocyte viability and erythrocyte morphology in whole blood under ambient storage. Neutrophil biostabilization was explored using a sophisticated microfluidic assay to examine the effectiveness of caspase inhibition to stabilize purified neutrophils. Following 72 h ambient storage, neutrophils remained fully functional to migrate towards chemical cues and maintained their ability to undergo NETosis after stimulation...
July 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28717320/coxiellosis-in-domestic-livestock-of-puducherry-and-tamil-nadu-detection-of-coxiella-burnetii-dna-by-polymerase-chain-reaction-in-slaughtered-ruminants
#5
Jothimani Pradeep, Selvaraj Stephen, Pratheesh Pooja, Anbalagan Akshayavardhini, Balakrishnan Sangeetha, Prabakar Xavier Antony
BACKGROUND AND AIM: In the course of our Indian Council of Medical Research project on coxiellosis in Puducherry and Tamil Nadu, 5.64% goat, 1.85% sheep, 1.06% buffaloes, and 0.97% cattle were positive for Coxiella burnetii antibodies by enzyme linked immunosorbent assay kit (IDEXX, Liebefeld, Switzerland). In this preliminary study, we have proceeded to look for C. burnetii DNA in those antibody positive specimens employing an imported commercial C. burnetii polymerase chain reaction (PCR) kit...
June 2017: Veterinary World
https://www.readbyqxmd.com/read/28716758/oral-delivery-of-vancomycin-by-tetraether-lipid-liposomes
#6
Philipp Uhl, Silvia Pantze, Philip Storck, Johannes Parmentier, Dominik Witzigmann, Götz Hofhaus, Jörg Huwyler, Walter Mier, Gert Fricker
Despite the outstanding progress in modern medicine, the oral delivery of peptide drugs is limited until today due to their instability in the gastrointestinal tract and low mucosa penetration. To overcome these hurdles, liposomes containing the specific tetraether lipid GCTE (glycerylcaldityltetraether lipid) were examined. For this purpose, the glycopeptide antibiotic vancomycin was used as model substance and liposomes were prepared by DAC (dual assymetric centrifugation). These liposomes showed a size and polydispersity index comparable to standard liposomes...
July 14, 2017: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28716755/construction-of-an-amperometric-glycated-hemoglobin-biosensor-based-on-au-pt-bimetallic-nanoparticles-and-poly-indole-5-carboxylic-acid-modified-au-electrode
#7
Utkarsh Jain, Shaivya Gupta, Nidhi Chauhan
The glucose level measurement in the diabetic patient plays a vital role in identification of the treatments going on and it also provides the control over the diabetics. A new electrochemical sensing device was constructed for determination of glycated hemoglobin (HbA1c) in whole blood samples. Fructosyl amine oxidase (FAO) was bioconjugated onto hybrid nanocomposite i.e., gold nanoparticles-platinum nanoparticles (AuNPs-PtNPs) and poly indole-5-carboxylic acid (PIN5COOH), deposited electrochemically on gold electrode...
July 14, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28716596/quality-comparison-of-umbilical-cord-blood-cryopreserved-with-conventional-versus-automated-systems
#8
Kyeong Mi Kim, Ji Young Huh, Jin Ju Kim, Myung Seo Kang
Umbilical cord blood (CB) banks usually freeze and store CB for clinical transplantation using conventional controlled-rate freezer or the automated BioArchive system. The aim of this study is to compare the quality of CB cryopreserved with conventional and automated methods and to make clear the cause of the quality difference between the two methods. The experiment used 80 CB units: 40 were conventionally cryopreserved and the remainder were cryopreserved with a BioArchive. After thawing, the following measures of CB quality were compared: recovery rates of cell count, cell viability of total nucleated cells (TNCs), mononuclear cells (MNCs), and CD34+ cells, as well as colony-forming unit-granulocyte/macrophage (CFU-GM) content...
July 14, 2017: Cryobiology
https://www.readbyqxmd.com/read/28713035/intracerebral-gene-therapy-in-children-with-mucopolysaccharidosis-type-iiib-syndrome-an-uncontrolled-phase-1-2-clinical-trial
#9
Marc Tardieu, Michel Zérah, Marie-Lise Gougeon, Jérome Ausseil, Stéphanie de Bournonville, Béatrice Husson, Dimitrios Zafeiriou, Giancarlo Parenti, Philippe Bourget, Béatrice Poirier, Valérie Furlan, Cécile Artaud, Thomas Baugnon, Thomas Roujeau, Ronald G Crystal, Christian Meyer, Kumaran Deiva, Jean-Michel Heard
BACKGROUND: Mucopolysaccharidosis type IIIB syndrome (also known as Sanfilippo type B syndrome) is a lysosomal storage disease resulting in progressive deterioration of cognitive acquisition after age 2-4 years. No treatment is available for the neurological manifestations of the disease. We sought to assess the safety and efficacy of a novel intracerebral gene therapy. METHODS: Local regulatory authorities in France allowed inclusion of up to four children in this phase 1/2 study...
July 13, 2017: Lancet Neurology
https://www.readbyqxmd.com/read/28712305/from-donor-to-recipient-considerations-for-blood-transfusion-outcomes-research
#10
Allison R Jones, Michelle R Brown, David E Vance
Donated blood can be broken down into blood components for use in patient care. This article focuses primarily on packed red blood cells (PRBCs), as they experience breakdown during storage that may adversely impact patient outcomes. Patients require PRBC transfusions for a number of clinical reasons. Although transfusions of PRBCs provide some clinical benefit, they are also associated with increased morbidity and mortality across multiple patient populations, albeit the mechanisms underlying this relationship remain unclear...
January 1, 2017: Biological Research for Nursing
https://www.readbyqxmd.com/read/28712037/introduction-of-sample-tubes-with-sodium-azide-as-a-preservative-for-ethyl-glucuronide-in-urine
#11
Marc Luginbühl, Wolfgang Weinmann, Ali Al-Ahmad
Ethyl glucuronide (EtG) is a direct alcohol marker, which is widely used for clinical and forensic applications, mainly for abstinence control. However, the instability of EtG in urine against bacterial degradation or the post-collectional synthesis of EtG in contaminated samples may cause false interpretation of EtG results in urine samples. This study evaluates the potential of sodium azide in tubes used for urine collection to hinder degradation of ethyl glucuronide by bacterial metabolism taking place during growth of bacterial colonies...
July 15, 2017: International Journal of Legal Medicine
https://www.readbyqxmd.com/read/28710204/bio-plex-immunoassay-measuring-the-quantity-of-lysosomal-n-acetylgalactosamine-6-sulfatase-protein-in-dried-blood-spots-for-the-screening-of-mucopolysaccharidosis-iva-in-newborn-a-pilot-study
#12
Chih-Kuang Chuang, Hsiang-Yu Lin, Tuan-Jen Wang, Sung-Fa Huang, Shuan-Pei Lin
OBJECTIVE: Mucopolysaccharidosis (MPS) IVA (Morquio syndrome A) is an autosomal-recessive lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine-6-sulfatase (GALNS) resulting in excessive lysosomal storage of keratan sulfate. Treatments for MPS IVA have recently become available with optimal outcomes associated with early diagnosis and treatment which can be achieved by newborn screening. DESIGN: Newborn screening programme for MPS IVA pilot study...
July 13, 2017: BMJ Open
https://www.readbyqxmd.com/read/28709159/packed-red-blood-cells-accumulate-oxidative-stress-with-increased-storage-duration
#13
Kelsey Preston, Sarah Harm, Nathan Dreyfus, Nuria Villalba, Kalev Freeman
No abstract text is available yet for this article.
August 2017: Shock
https://www.readbyqxmd.com/read/28707754/non-sibling-hematopoietic-stem-cell-transplantation-using-myeloablative-conditioning-regimen-in-children-with-maroteaux-lamy-syndrome-a-brief-report
#14
Maryam Behfar, S Sharareh Dehghani, Tahereh Rostami, Ardeshir Ghavamzadeh, Amir Ali Hamidieh
Maroteaux-Lamy syndrome is a rare inherited lysosomal storage disorder with a progressive course. HSCT is a curable option for treatment in these patients. The following report describes our experience in HSCT for three patients with Maroteaux-Lamy syndrome using non-sibling donors. All of the patients received the same myeloablative regimen consisting of intravenous busulfan, cyclophosphamide, and rabbit antithymocyte globulin. Patients underwent HSCT from haploidentical other-related (n=1), full-matched other-related (n=1), and one-locus-mismatched unrelated donor...
August 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28706084/rationale-and-protocol-for-the-after-diabetes-diagnosis-research-support-system-address-an-incident-and-high-risk-type-1-diabetes-uk-cohort-study
#15
Helen C Walkey, Akaal Kaur, Vassiliki Bravis, Ian F Godsland, Shivani Misra, Alistair J K Williams, Polly J Bingley, David B Dunger, Nick Oliver, Desmond G Johnston
INTRODUCTION: Type 1 diabetes is heterogeneous in its presentation and progression. Variations in clinical presentation between children and adults, and with ethnic group warrant further study in the UK to improve understanding of this heterogeneity. Early interventions to limit beta cell damage in type 1 diabetes are undergoing evaluation, but recruitment is challenging. The protocol presented describes recruitment of people with clinician-assigned, new-onset type 1 diabetes to understand the variation in their manner of clinical presentation, to facilitate recruitment into intervention studies and to create an open-access resource of data and biological samples for future type 1 diabetes research...
July 12, 2017: BMJ Open
https://www.readbyqxmd.com/read/28704937/attenuation-of-red-blood-cell-storage-lesions-with-vitamin-c
#16
Kimberly Sanford, Bernard J Fisher, Evan Fowler, Alpha A Fowler, Ramesh Natarajan
Stored red blood cells (RBCs) undergo oxidative stress that induces deleterious metabolic, structural, biochemical, and molecular changes collectively referred to as "storage lesions". We hypothesized that vitamin C (VitC, reduced or oxidized) would reduce red cell storage lesions, thus prolonging their storage duration. Whole-blood-derived, leuko-reduced, SAGM (saline-adenine-glucose-mannitol)-preserved RBC concentrates were equally divided into four pediatric storage bags and the following additions made: (1) saline (saline); (2) 0...
July 12, 2017: Antioxidants (Basel, Switzerland)
https://www.readbyqxmd.com/read/28696433/elevated-plasma-ferritin-in-elderly-individuals-with-high-neocortical-amyloid-%C3%AE-load
#17
K Goozee, P Chatterjee, I James, K Shen, H R Sohrabi, P R Asih, P Dave, C ManYan, K Taddei, S J Ayton, M L Garg, J B Kwok, A I Bush, R Chung, J S Magnussen, R N Martins
Ferritin, an iron storage and regulation protein, has been associated with Alzheimer's disease (AD); however, it has not been investigated in preclinical AD, detected by neocortical amyloid-β load (NAL), before cognitive impairment. Cross-sectional analyses were carried out for plasma and serum ferritin in participants in the Kerr Anglican Retirement Village Initiative in Aging Health cohort. Subjects were aged 65-90 years and were categorized into high and low NAL groups via positron emission tomography using a standard uptake value ratio cutoff=1...
July 11, 2017: Molecular Psychiatry
https://www.readbyqxmd.com/read/28696211/rab8a-deficiency-in-skeletal-muscle-causes-hyperlipidemia-and-hepatosteatosis-via-impairment-of-muscle-lipid-uptake-and-storage
#18
Qiaoli Chen, Ping Rong, Dijin Xu, Sangsang Zhu, Liang Chen, Bingxian Xie, Qian Du, Chao Quan, Yang Sheng, Tong-Jin Zhao, Peng Li, Hong Yu Wang, Shuai Chen
Skeletal muscle absorbs long chain fatty acids (LCFA) that are either oxidized in mitochondria or temporarily stored as triglycerides in lipid droplets (LDs). So far, it is still not fully understood how muscle lipid uptake and storage is regulated and whether it is important for whole-body lipid homeostasis. Here we show that the small GTPase Rab8a regulates muscle lipid uptake and storage in skeletal muscle. Muscle-specific Rab8a deletion caused hyperlipidemia and exacerbated high fat diet (HFD)-induced hepatosteatosis...
July 10, 2017: Diabetes
https://www.readbyqxmd.com/read/28693362/veterinary-utility-of-dried-blood-spots-for-analysis-of-toxic-chlorinated-hydrocarbons
#19
Andreas F Lehner, Margaret Johnson, John Buchweitz
Dried blood spots (DBS) on filter paper provide a simple and convenient means of collecting, storing and shipping samples for veterinary diagnostics related to toxin exposures. This paper presents validation data on analysis of DBS for chlorinated persistent organic pollutants, specifically 4,4'-DDT and its breakdown product 4,4'-DDE, lindane and a representative polychlorinated biphenyl (PCB) congener PCB-153. Analysis was by gas chromatography with electron capture detection (GC-ECD). The method required one 12...
July 11, 2017: Toxicology Mechanisms and Methods
https://www.readbyqxmd.com/read/28691021/evaluation-of-stability-and-in-vitro-security-of-nanoemulsions-containing-eucalyptus-globulus-oil
#20
Samantha Nunes de Godoi, Priscilla Maciel Quatrin, Michele Rorato Sagrillo, Kátia Nascimento, Roger Wagner, Bruna Klein, Roberto Christ Vianna Santos, Aline Ferreira Ourique
Essential oil of Eucalyptus globulus presents several pharmacological properties. However, their therapeutic efficacy may be affected by limitations due to several conditions, rendering it difficult to obtain stable and effective pharmaceutical formulations. The use of nanotechnology is an alternative to improve their characteristics aiming to ensure their stability and effectiveness. Furthermore, studies about the possible toxic effects of nanostructures are necessary to evaluate safety when the formulation comes into contact with human cells...
2017: BioMed Research International
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