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https://www.readbyqxmd.com/read/28104572/progressive-heart-disease-in-mucopolysaccharidosis-type-i-mice-may-be-mediated-by-increased-cathepsin-b-activity
#1
Guilherme Baldo, Angela Maria Vicente Tavares, Esteban Gonzalez, Edina Poletto, Fabiana Quoos Mayer, Ursula da Silveira Matte, Roberto Giugliani
Mucopolysaccharidosis type I (MPS I) is a lysosomal disorder characterized by a deficiency of alpha-L-iduronidase and storage of undegraded glycosaminoglycans (GAGs). Clinical findings of the disease include heart failure, and patients often need valve replacement. It has been shown that, later in life, MPS I mice develop those abnormalities, but to date, there have not been studies on the progression and pathogenesis of the disease. Therefore, in the present study, we evaluated heart function in normal and MPS I male mice from 2 to 8 months of age...
January 6, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28098417/delivery-of-anesthesia-for-children-with-mucopolysaccharidosis-type-iii-sanfilippo-syndrome-a-review-of-86-anesthetics
#2
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
#3
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/28091969/quality-of-life-as-predictor-for-the-development-of-cardiac-ischemia-in-high-risk-asymptomatic-diabetic-patients
#4
Philip Haaf, Myriam Ritter, Leticia Grize, Matthias E Pfisterer, Michael J Zellweger
BACKGROUND: Ischemia induced by psychological stress and depression is a common phenomenon in stable coronary artery disease (CAD). We evaluated the quality of life (QoL) of diabetic patients screened for CAD and assessed the prognostic value of mental and physical QoL scores to predict the development of new cardiac ischemia. METHODS: Prospective multicentre outcome study. The study comprised 400 asymptomatic diabetic patients without history or symptoms of CAD...
January 13, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28081172/influence-of-corneal-opacity-on-intraocular-pressure-assessment-in-patients-with-lysosomal-storage-diseases
#5
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/28073896/patient-specific-circulating-tumor-dna-detection-during-neoadjuvant-chemotherapy-in-triple-negative-breast-cancer
#6
Francesca Riva, Francois-Clement Bidard, Alexandre Houy, Adrien Saliou, Jordan Madic, Aurore Rampanou, Caroline Hego, Maud Milder, Paul Cottu, Marie-Paule Sablin, Anne Vincent-Salomon, Olivier Lantz, Marc-Henri Stern, Charlotte Proudhon, Jean-Yves Pierga
BACKGROUND: In nonmetastatic triple-negative breast cancer (TNBC) patients, we investigated whether circulating tumor DNA (ctDNA) detection can reflect the tumor response to neoadjuvant chemotherapy (NCT) and detect minimal residual disease after surgery. METHODS: Ten milliliters of plasma were collected at 4 time points: before NCT; after 1 cycle; before surgery; after surgery. Customized droplet digital PCR (ddPCR) assays were used to track tumor protein p53 (TP53) mutations previously characterized in tumor tissue by massively parallel sequencing...
January 10, 2017: Clinical Chemistry
https://www.readbyqxmd.com/read/28063865/evaluation-of-a-mastery-learning-intervention-on-hospitalists-code-status-discussion-skills
#7
Rashmi K Sharma, Eytan Szmuilowicz, Adeboye Ogunseitan, Sasha F Jones, Jessica A Montalvo, Kevin J O'Leary, Diane B Wayne
CONTEXT: Although code status discussions (CSD) occur frequently in the hospital setting, discussions often lack content necessary for informed decision making. Simulation-based mastery learning (SBML) has been used to improve clinical skills among resident physicians and may provide a novel way to improve hospitalists' CSD skills. OBJECTIVES: The objective of this pilot randomized controlled trial was to develop and evaluate a CSD SBML intervention for hospitalists...
January 4, 2017: Journal of Pain and Symptom Management
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/28057436/comparison-of-clinically-relevant-mutation-profiles-between-preoperative-biopsy-and-corresponding-surgically-resected-specimens-in-japanese-patients-with-non-small-cell-lung-cancer-by-amplicon-based-massively-parallel-sequencing
#9
Mitsuhiro Isaka, Masakuni Serizawa, Hirotsugu Kenmotsu, Yasuhiro Koh, Shoji Takahashi, Tomohiro Maniwa, Kazushige Wakuda, Akira Ono, Tateaki Naito, Haruyasu Murakami, Keita Mori, Masahiro Endo, Masato Abe, Isamu Hayashi, Takashi Nakajima, Nobuyuki Yamamoto, Toshiaki Takahashi, Yasuhisa Ohde
BACKGROUND: Amplicon-based massively parallel sequencing (MPS) is an effective platform for identifying clinically actionable mutations across many genes in limited amounts of tissue. Most lung cancers are diagnosed and staged using small tissue samples obtained by transbronchial biopsy (TBB). To determine whether the mutations in TBB specimens detected by amplicon-based MPS reflect those present in the tumors, we compared the mutational profiles of preoperative TBB specimens and corresponding surgically resected specimens...
December 2, 2016: Clinical Lung Cancer
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/28050459/cardiovascular-abnormalities-in-egyptian-children-with-mucopolysaccharidoses
#11
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/28041839/microplastic-pollution-in-vembanad-lake-kerala-india-the-first-report-of-microplastics-in-lake-and-estuarine-sediments-in-india
#12
S Sruthy, E V Ramasamy
We present the first study of microplastics in the sediments of Vembanad Lake, a Ramsar site in India. Microplastics are emerging pollutants of increasing environmental concern with a particle size of <5 mm, which originate from successive degradation of larger plastic debris or are manufactured as small granules and used in many applications. The impact of microplastics pollution on the environment and biota is not well known. Vast data exist in the literature on marine microplastics while reports on freshwater ecosystems are scarce...
December 29, 2016: Environmental Pollution
https://www.readbyqxmd.com/read/28033261/elevated-thrombopoietin-and-platelet-indices-confirm-active-thrombopoiesis-but-fail-to-predict-clinical-severity-of-puumala-hantavirus-infection
#13
Outi Laine, Lotta Joutsi-Korhonen, Riitta Lassila, Heini Huhtala, Antti Vaheri, Satu Mäkelä, Jukka Mustonen
We evaluated the mechanisms of thrombocytopenia and procoagulant changes in relation with clinical variables in a cohort of patients with acute hantavirus disease.Blood samples of 33 prospectively recruited, consecutive, hospitalized patients with acute Puumala virus-induced hemorrhagic fever with renal syndrome (HFRS) were collected acutely and at the recovery visit (control). Serum thrombopoietin (TPO) and activity of plasma microparticles (MPs) from various cell sources were measured with enzyme-linked immunosorbent assay-based methods...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28026955/tunable-synthesis-of-mesoporous-silica-particles-with-unique-radially-oriented-pore-structures-from-tetramethyl-orthosilicate-via-oil-water-emulsion-process
#14
Lusi Ernawati, Ratna Balgis, Takashi Ogi, Kikuo Okuyama
Numerous studies of the synthesis of mesoporous silica (MPS) particles with tailored properties have been published. Among those studies, tetraethyl orthosilicate (TEOS) is commonly used as a silica source, but tetramethyl orthosilicate (TMOS) is rarely used because its reaction is fast and difficult to control. In this study, MPS particles were synthesized via one-step controlled polymerization of styrene and hydrolysis of TMOS, followed by the addition of hexadecyltrimethylammonium bromide (CTAB) and n-octane...
January 9, 2017: Langmuir: the ACS Journal of Surfaces and Colloids
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/28013294/mutation-in-vps33a-affects-metabolism-of-glycosaminoglycans-a-new-type-of-mucopolysaccharidosis-with-severe-systemic-symptoms
#16
Hidehito Kondo, Nadezda Maksimova, Takanobu Otomo, Hisakazu Kato, Atsuko Imai, Yoshihiro Asano, Kaori Kobayashi, Satoshi Nojima, Akihiro Nakaya, Yusuke Hamada, Kaori Irahara, Elizaveta Gurinova, Aitalina Sukhomyasova, Anna Nogovicina, Mira Savvina, Tamotsu Yoshimori, Keiichi Ozono, Norio Sakai
Mucopolysaccharidoses (MPS) are a group of genetic deficiencies of lysosomal enzymes that catabolize glycosaminoglycans (GAG). Here we describe a novel MPS-like disease caused by a specific mutation in the VPS33A gene. We identified several Yakut patients showing typical manifestations of MPS: coarse facial features, skeletal abnormalities, hepatosplenomegaly, respiratory problems, mental retardation, and excess secretion of urinary GAG. However, these patients could not be diagnosed enzymatically as MPS. They showed extremely high levels of plasma heparan sulphate (HS, one of GAG); 60 times the normal reference range and 6 times that of MPS patients...
December 23, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/28013102/core-shell-microparticles-for-protein-sequestration-and-controlled-release-of-a-protein-laden-core
#17
Torri E Rinker, Brandon D Philbrick, Johnna S Temenoff
: Development of multifunctional biomaterials that sequester, isolate, and redeliver cell-secreted proteins at a specific timepoint may be required to achieve the level of temporal control needed to more fully regulate tissue regeneration and repair. In response, we fabricated core-shell heparin-poly(ethylene-glycol) (PEG) microparticles (MPs) with a degradable PEG-based shell that can temporally control delivery of protein-laden heparin MPs. Core-shell MPs were fabricated via a re-emulsification technique and the number of heparin MPs per PEG-based shell could be tuned by varying the mass of heparin MPs in the precursor PEG phase...
December 21, 2016: Acta Biomaterialia
https://www.readbyqxmd.com/read/28011272/resolution-of-hydronephrosis-in-a-patient-with-mucopolysaccharidosis-type-ii-with-enzyme-replacement-therapy
#18
Kei Nishiyama, Takashi Imai, Kazuhiro Ohkubo, Masafumi Sanefuji, Hidetoshi Takada
Mucopolysaccharidosis type II (MPS II) is caused by deficiency of lysosomal enzyme iduronate-2-sulfatase. Insufficient activity of the enzyme results in accumulation of glycosaminoglycans leading to progressive multisystem pathologies. MPS II is less likely to be complicated by kidney and urinary tract problems. We report a boy with MPS II, who developed left hydronephrosis. His hydronephrosis improved after starting enzyme replacement therapy (ERT). It was suggested mucopolysaccharidosis type II was closely associated with the pathogenesis of hydronephrosis...
December 20, 2016: Urology
https://www.readbyqxmd.com/read/28009236/increased-blood-cell-phosphatidylserine-exposure-and-circulating-microparticles-contribute-to-procoagulant-activity-after-carotid-artery-stenting
#19
Lu Zhao, Xiaoming Wu, Yu Si, Zhipeng Yao, Zengxiang Dong, Valerie A Novakovic, Li Guo, Dongxia Tong, He Chen, Yayan Bi, Junjie Kou, Huaizhang Shi, Ye Tian, Shaoshan Hu, Jin Zhou, Jialan Shi
OBJECTIVE Phosphatidylserine (PS) is a major component of the inner leaflet of membrane bilayers. During cell activation or apoptosis, PS is externalized to the outer membrane, providing an important physiological signal necessary for the release of the microparticles (MPs) that are generated through the budding of cellular membranes. MPs express PS and membrane antigens that reflect their cellular origin. PS exposure on the cell surface and the release of MPs provide binding sites for factor Xa and prothrombinase complexes that promote thrombin formation...
December 23, 2016: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28005334/design-of-uv-absorbing-polypropylene-films-with-polymeric-benzotriaziole-based-nano-and-microparticle-coatings
#20
Sarit Cohen, Hai Haham, Michal Pellach, Shlomo Margel
UV-absorbing nanoparticles (NPs) and microparticles (MPs) were prepared by emulsion and dispersion copolymerization of the vinylic monomer 2-(2'-hydroxy-5'-methacryloxyethylphenyl)-2H-benzotriazole (Norbloc (NB)) with the crosslinking monomer divinylbenzene. The effect of the initiator concentration on the size and size distribution of the polyNB (PNB) particles was elucidated. Thin coatings of the formed PNB NPs or MPs of 19 ± 2 and 200 ± 25 nm dry diameter, respectively, onto polypropylene (PP) films were then prepared and characterized...
December 22, 2016: ACS Applied Materials & Interfaces
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