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https://www.readbyqxmd.com/read/28220405/altered-cellular-homeostasis-in-murine-mps-i-fibroblasts-evidence-of-cell-specific-physiopathology
#1
Gustavo Monteiro Viana, Cinthia Castro do Nascimento, Edgar Julian Paredes-Gamero, Vânia D'Almeida
Mucopolysaccharidosis type I (MPS I), a rare autosomal recessive disease, is caused by a deficiency of the lysosomal enzyme alfa-L-iduronidase. Impaired enzyme activity promotes glycosaminoglycans accumulation in several tissues and organs, leading to complex multisystemic complications. Several studies using animal models indicated different intracellular pathways involving MPS I physiopathology; however, the exact mechanisms underlying this syndrome are still not understood. Previous results from our group showed alterations in ionic homeostasis and cell viability of splenocytes and macrophages in Idua-/- mice...
February 21, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28210640/muscle-weakness-a-misleading-presentation-in-children-with-distinctive-syndromic-entities-clinical-case-reports
#2
Ali Al Kaissi, Sergey Ryabykh, Polina Ochirova, Vladimir Kenis, Jochen G Hofstätter, Franz Grill, Rudolf Ganger, Susanne Gerit Kircher
Marked ligamentous hyperlaxity and muscle weakness/wasting associated with awkward gait are the main deficits confused with the diagnosis of myopathy. Seven children (6 boys and 1 girl with an average age of 8 years) were referred to our department because of diverse forms of skeletal abnormalities. No definitive diagnosis was made, and all underwent a series of sophisticated investigations in other institutes in favor of myopathy. We applied our methodology through the clinical and radiographic phenotypes followed by targeted genotypic confirmation...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28207887/platelet-monocyte-derived-and-tissue-factor-carrying-circulating-microparticles-are-related-to-acute-myocardial-infarction-severity
#3
Gemma Chiva-Blanch, Kristian Laake, Peder Myhre, Vibeke Bratseth, Harald Arnesen, Svein Solheim, Lina Badimon, Ingebjørg Seljeflot
OBJECTIVE: Circulating microparticles (cMPs) are phospholipid-rich vesicles released from cells when activated or injured, and contribute to the formation of intracoronary thrombi. Tissue factor (TF, CD142) is the main trigger of fibrin formation and TF-carrying cMPs are considered one of the most procoagulant cMPs. Similar types of atherosclerotic lesions may lead to different types of AMI, although the mechanisms behind are unresolved. Therefore, we aimed to investigate the phenotype of cMPs found in plasma of ACS patients and its relation to AMI severity and thrombotic burden...
2017: PloS One
https://www.readbyqxmd.com/read/28207863/identification-of-age-dependent-motor-and-neuropsychological-behavioural-abnormalities-in-a-mouse-model-of-mucopolysaccharidosis-type-ii
#4
Hélène F E Gleitz, Claire O'Leary, Rebecca J Holley, Brian W Bigger
Severe mucopolysaccharidosis type II (MPS II) is a progressive lysosomal storage disease caused by mutations in the IDS gene, leading to a deficiency in the iduronate-2-sulfatase enzyme that is involved in heparan sulphate and dermatan sulphate catabolism. In constitutive form, MPS II is a multi-system disease characterised by progressive neurocognitive decline, severe skeletal abnormalities and hepatosplenomegaly. Although enzyme replacement therapy has been approved for treatment of peripheral organs, no therapy effectively treats the cognitive symptoms of the disease and novel therapies are in development to remediate this...
2017: PloS One
https://www.readbyqxmd.com/read/28198536/prevalence-of-risk-factors-and-asymptomatic-carotid-atherosclerosis-in-diabetic-patients-screened-for-silent-myocardial-ischemia-by-spect-myocardial-imaging
#5
Irena Peovska Mitevska, Natalija Baneva, Marijan Bosevski, Elizabeta Srbinovska Kostovska
BACKGROUND: The aim of the study was to evaluate whether there is any association between myocardial ischemia, common risk factors and carotid artery ultrasound parameters in asymptomatic type 2 diabetic (DMT2) patients. MATERIAL AND METHODS: 60 asymptomatic DMT2 patients (pts) without known coronary artery disease (CAD) underwent one day rest Dypiridamole stress Tc-99m sestamibi single photon emission computed tomography myocardial perfusion scintigraphy (MPS)...
2017: Nuclear Medicine Review. Central & Eastern Europe
https://www.readbyqxmd.com/read/28193505/statistical-modelling-of-ion-pgm-hid-str-10-plex-mps-data
#6
Søren B Vilsen, Torben Tvedebrink, Helle Smidt Mogensen, Niels Morling
We investigated the results of short tandem repeat (STR) markers of dilution series experiments and reference profiles generated using the Ion PGM massively parallel sequencing platform utilising the HID STR 10-plex panel. The STR markers were identified by the marker specific flanking regions of the STR region. We investigated the following: (1) the usage of quality measures for identifying substitution errors, (2) the heterozygote balance and compared it to that of capillary electrophoresis (CE), (3) the stability of the coverage and the consequence of IonExpress Barcode adapter (IBA) sampling with decreasing amounts of template DNA, (4) the hypothesis that the parental longest uninterrupted stretch (LUS) is a better linear predictor of stutter ratio than the parent allele length, (5) the use of parental allele length as a predictor of shoulder ratio, and (6) the removal of non-systematic erroneous sequences using dynamic thresholds created by fitting the distribution of the non-systematic erroneous sequences...
February 3, 2017: Forensic Science International. Genetics
https://www.readbyqxmd.com/read/28186595/-analysis-of-ids-gene-mutation-in-a-family-affected-with-mucopolysaccharidosis-type%C3%A2
#7
Yiying Li, Shiyue Mei, Xiangdong Kong, Zhenhua Zhao, Xiaofan Zhu, Xinyu Yang, Zhi Qin, Han Wu
OBJECTIVE: To detect potential mutation of iduronate-2-sulfatase (IDS) gene in a family affected with mucopolysaccharidosis type Ⅱ (MPS Ⅱ). METHODS: For the proband and his unaffected mother, the whole coding sequence of the IDS gene was analyzed with PCR and bidirectional Sanger sequencing. RESULTS: A novel splicing mutation, c.709-1G>A, was detected in the proband, for which his mother was heterozygous. CONCLUSION: The c...
February 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28170539/objective-quantification-of-changes-in-corneal-clouding-over-time-in-patients-with-mucopolysaccharidosis
#8
Ahmed Javed, Tariq Aslam, Simon A Jones, Jane Ashworth
Purpose: We determine objective changes in corneal opacification levels over time in patients with mucopolysaccharidoses (MPS) treated with enzyme replacement therapy or hematopoietic stem cell transplant. A prospective cohort study was done of 9 patients with MPS I (Hurler) or VI (Maroteaux-Lamy). Methods: Quantification of corneal clouding using the Iris camera and full ophthalmic examination, including subjective assessment of corneal clouding, was done in 2011 and repeated in 2015/2016...
February 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28168351/circulating-microparticles-and-the-risk-of-thromboembolic-events-in-egyptian-beta-thalassemia-patients
#9
Ilham Youssry, Nohair Soliman, Mona Ghamrawy, Rania Mohamed Samy, Amal Nasr, Mohamed Abdel Mohsen, Mohamed ElShahaat, Rayan Bou Fakhredin, Ali Taher
The presence of elevated numbers of circulating microparticles (MPs) has been hypothesized to be responsible for the occurrence of thromboembolic events (TEEs) in thalassemic patients. Our aim is to evaluate the presence and the thrombotic risk of circulating MPs in thalassemia patients and to determine the difference in MPs between β-thalassemia major (β-TM) and thalassemia intermedia (TI). The percentage of the annexin-labeled MPs, platelet-derived MPs (PMPs), erythrocyte-derived MPs (RMPs), and endothelial-derived MPs (EMPs) was measured by flow cytometry, in 87 thalassemia patients (39 β-TM and 48 TI)...
February 7, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28164577/clinical-and-experimental-analysis-of-platelet-micro-particles-inducing-thrombosis-after-coronary-stenting
#10
Muyesai Nijiati, Abulijiang Saidaming, Adelijiang Tuohuti, Li Guoqing, Du Rong
BACKGROUND: A limited number of reports are analyzing the etiology and the mechanism of coronary heart disease by examining the source cells of micro particles (MPs) in coronary heart disease patients with percutaneous coronary intervention (PCI). This study aims to explore the circulating platelet micro particles (PMPs) content variation in the blood stream and the mechanism of MPs-inducing thrombosis in patients operated with coronary stenting, with the intent to analyze the impact of PMPs on thrombosis and in-stent restenosis...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28164307/how-to-quantify-microparticles-in-rbcs-a-validated-flow-cytometry-method-allows-the-detection-of-an-increase-in-microparticles-during-storage
#11
Clémentine Gamonet, Guillaume Mourey, Sophie Aupet, Sabéha Biichle, Régis Petitjean, Chrystelle Vidal, Aurore Pugin, Christian Naegelen, Pierre Tiberghien, Pascal Morel, Fanny Angelot-Delettre, Estelle Seilles, Philippe Saas, Laurent Bardiaux, Francine Garnache-Ottou
BACKGROUND: The procoagulant and proinflammatory microparticles (MPs) released during storage of packed red blood cells (pRBCs) can potentially modify transfusion benefits. A robust method to quantify MPs in pRBCs is needed to evaluate their impact in clinical trials. STUDY DESIGN AND METHODS: The objective was to validate the preanalytic conditions required to prepare pRBC supernatant as well as a method to quantify and evaluate MP variations over 42 days of pRBC storage...
February 5, 2017: Transfusion
https://www.readbyqxmd.com/read/28156669/a-novel-scoring-system-to-predict-survival-in-patients-with-advanced-pancreatic-adenocarcinoma-the-memorial-prognostic-score-mps
#12
Alison Wiesenthal, Andrew S Epstein, Junting Zheng, Jessica Goldberg, Jason Meadows, Eileen Mary O'Reilly, Paul A Glare
: 36 Background: A major limitation of prognostic tools such as the Eastern Cooperative Oncology Group (ECOG), Karnofsky, and Palliative Performance Scale is a reliance on subjective clinical assessment. An objective tool, the Glasgow Prognostic Score (GPS) is derived from C-reactive Protein (CRP) and albumin and has been validated in patients with operable and inoperable malignancies. One disadvantage of this tool is that CRP is not routinely measured in the United States. We examined if the Neutrophil-Lymphocyte Ratios (NLR) (Ahn, H...
October 9, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28156456/prevalence-of-myofascial-pain-syndrome-in-patients-with-incurable-cancer
#13
Junya Kinkawa, Aya Watanabe, Chie Watanabe, Tsukuru Chiba, Naoki Shimada, Keisuke Ariyoshi, Masanori Nojima, Satoru Iwase, Arinobu Tojo
: 97 Background: Myofascial pain syndrome (MPS) is a condition that involves skeletal muscles. It is caused by overload or disuse of muscles and is characterized by extreme tenderness in the muscles with taut bands. Treatment for MSP is different from that for cancer-related pain. Cancer patients have many factors that cause restriction of body movement and posture. Although cancer patients appear to demonstrate risk factors for MPS, its prevalence has not been reported in patients with incurable cancer...
October 9, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28155823/ultrasonography-in-diagnosis-of-myofascial-pain-syndrome-and-reliability-of-novel-ultrasonic-indexes-of-upper-trapezius-muscle
#14
Navid Taheri, Farshad Okhovatian, Asghar Rezasoltani, Mehdi Karami, Sayed Mohsen Hosseini, Hosein Kouhzad Mohammadi
BACKGROUND: Myofascial pain syndrome (MPS) is a common non-articular musculoskeletal disorder. It is characterized by local and referred pain due to the presence of myofascial trigger points (MTrPs). MTrPs most commonly involve the upper trapezius muscle and can be visualized using ultrasound imaging. This study was designed to determine the inter-rater reliability of some new ultrasonographic indices of the upper trapezius muscle and the sensitivity and specificity of 2D ultrasound imaging in the diagnosis of MPS...
March 23, 2016: Ortopedia, Traumatologia, Rehabilitacja
https://www.readbyqxmd.com/read/28151904/comparison-between-ultrasound-guided-interfascial-pulsed-radiofrequency-and-ultrasound-guided-interfascial-block-with-local-anesthetic-in-myofascial-pain-syndrome-of-trapezius-muscle
#15
RANDOMIZED CONTROLLED TRIAL
Ik Tae Cho, Yun Woo Cho, Sang Gyu Kwak, Min Cheol Chang
Myofascial pain syndrome (MPS) of the trapezius muscle (TM) is a frequently occurring musculoskeletal disorder. However, the treatment of MPS of the TM remains a challenge. We investigated the effects of ultrasound (US)-guided pulsed radiofrequency (PRF) stimulation on the interfascial area of the TM. In addition, we compared its effect with that of interfascial block (IFB) with 10 mL of 0.6% lidocaine on the interfascial area of the TM. Thirty-six patients with MPS of the TM were included and randomly assigned into 2 groups...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28150311/spherocytic-shift-of-red-blood-cells-during-storage-provides-a-quantitative-whole-cell-based-marker-of-the-storage-lesion
#16
Camille Roussel, Michaël Dussiot, Mickaël Marin, Alexandre Morel, Papa Alioune Ndour, Julien Duez, Caroline Le Van Kim, Olivier Hermine, Yves Colin, Pierre A Buffet, Pascal Amireault
BACKGROUND: Storage lesion may explain the rapid clearance of up to 25% of transfused red blood cells (RBCs) in recipients. Several alterations affect stored RBC but a quantitative, whole cell-based predictor of transfusion yield is lacking. Because RBCs with reduced surface area are retained by the spleen, we quantified changes in RBC dimensions during storage. STUDY DESIGN AND METHODS: Using imaging flow cytometry we observed the dimension and morphology of RBCs upon storage, along with that of conventional biochemical and mechanical markers of storage lesion...
February 1, 2017: Transfusion
https://www.readbyqxmd.com/read/28148395/tissue-factor-factor-viia-complex-triggers-protease-activated-receptor-2-dependent-growth-factor-release-and-migration-in-ovarian-cancer
#17
Alice Chanakira, Pamela R Westmark, Irene M Ong, John P Sheehan
OBJECTIVE: Enhanced tissue factor (TF) expression in epithelial ovarian cancer (EOC) is associated with aggressive disease. Our objective was to evaluate the role of the TF-factor VIIa-protease-activated receptor-2 (PAR-2) pathway in human EOC. METHODS: TCGA RNAseq data from EOC databases were analyzed for PAR expression. Cell and microparticle (MP) associated TF protein expression (Western blot) and MP-associated coagulant activity were determined in human EOC (SKOV-3, OVCAR-3 and CaOV-3) and control cell lines...
January 29, 2017: Gynecologic Oncology
https://www.readbyqxmd.com/read/28145222/protective-immunity-against-toxoplasmosis-in-mice-induced-by-single-dose-immunization-with-rsag1-2-protein-released-from-poly-lactide-co-glycolide-microparticles
#18
Shu-Chun Chuang, Yao-Chi Chung, Chung-Da Yang
Triphasic sustained release of tachyzoite chimeric protein, rSAG1/2, from poly(lactide-co-glycolide) (PLG)-encapsulated rSAG1/2 (PLG-rSAG1/2) microparticles (MPs) is a promising characteristic for developing a single-dose vaccine against Toxoplasma gondii in domestic animals. In the present study, we aimed to evaluate whether single immunization with PLG-rSAG1/2 MPs in BALB/c mice would achieve effective immunity and protection against T. gondii. Peritoneal immunization of mice with a single dose of PLG-rSAG1/2 MPs enhanced serum IgG titers and lymphocyte proliferation in a triphasic model over a long 12-week period...
2017: Parasite: Journal de la Société Française de Parasitologie
https://www.readbyqxmd.com/read/28143737/near-complete-correction-of-profound-metabolomic-impairments-corresponding-to-functional-benefit-in-mps-iiib-mice-after-iv-raav9-hnaglu-gene-delivery
#19
Haiyan Fu, Aaron S Meadows, Tierra Ware, Robert P Mohney, Douglas M McCarty
Mucopolysaccharidosis (MPS) IIIB is a lysosomal storage disease with complex CNS and somatic pathology due to a deficiency in α-N-acetylglucosaminidase (NAGLU). Using global metabolic profiling by mass spectrometry targeting 361 metabolites, this study detected significant decreases in 225 and increases in six metabolites in serum samples from 7-month-old MPS IIIB mice, compared to wild-type (WT) mice. The metabolic disturbances involve virtually all major pathways of amino acid, peptide (58/102), carbohydrate (18/28), lipid (111/139), nucleotide (12/24), energy (2/9), vitamin and cofactor (11/16), and xenobiotic (11/28) metabolism...
January 28, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28138910/spray-dried-thiolated-chitosan-coated-sodium-alginate-multilayer-microparticles-for-vaginal-hiv-microbicide-delivery
#20
Jianing Meng, Vivek Agrahari, Miezan J Ezoulin, Sudhaunshu S Purohit, Tao Zhang, Agostino Molteni, Daniel Dim, Nathan A Oyler, Bi-Botti C Youan
It is hypothesized that novel thiolated chitosan-coated multilayer microparticles (MPs) with enhanced drug loading are more mucoadhesive than uncoated MPs and safe in vivo for vaginal delivery of topical anti-HIV microbicide. Formulation optimization is achieved through a custom experimental design and the alginate (AG) MPs cores are prepared using the spray drying method. The optimal MPs are then coated with the thiolated chitosan (TCS) using a layer-by-layer method. The morphological analysis, in situ drug payload, in vitro drug release profile, and mucoadhesion potential of the MPs are carried out using scanning electron microscopy, solid-state (31)P NMR spectroscopy, UV spectroscopy, fluorescence imaging and periodic acid Schiff method, respectively...
January 30, 2017: AAPS Journal
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