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https://www.readbyqxmd.com/read/27916847/aortic-root-dilatation-in-mucopolysaccharidosis-i-vii
#1
Meena Bolourchi, Pierangelo Renella, Raymond Y Wang
The prevalence of aortic root dilatation (ARD) in mucopolysaccharidosis (MPS) is not well documented. We investigated aortic root measurements in 34 MPS patients at the Children's Hospital of Orange County (CHOC). The diagnosis, treatment status, age, gender, height, weight and aortic root parameters (aortic valve annulus (AVA), sinuses of Valsalva (SoV), and sinotubular junction (STJ)) were extracted by retrospective chart review and echocardiographic measurements. Descriptive statistics, ANOVA, and paired post-hoc t-tests were used to summarize the aortic dimensions...
November 29, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27910891/elevated-cerebral-spinal-fluid-biomarkers-in-children-with-mucopolysaccharidosis-i-h
#2
Gerald V Raymond, Marzia Pasquali, Lynda E Polgreen, Patricia I Dickson, Weston P Miller, Paul J Orchard, Troy C Lund
Mucopolysaccharidosis (MPS) type-IH is a lysosomal storage disease that results from mutations in the IDUA gene causing the accumulation of glycosaminoglycans (GAGs). Historically, children with the severe phenotype, MPS-IH (Hurler syndrome) develop progressive neurodegeneration with death in the first decade due to cardio-pulmonary complications. New data suggest that inflammation may play a role in MPS pathophysiology. To date there is almost no information on the pathophysiologic changes within the cerebral spinal fluid (CSF) of these patients...
December 2, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27904658/circulating-microparticles-enhanced-rat-vascular-wall-remodeling-following-endothelial-denudation
#3
Fan-Yen Lee, Hung-I Lu, Han-Tan Chai, Jiunn-Jye Sheu, Yi-Ling Chen, Tein-Hung Huang, Gour-Shenq Kao, Sheng-Yi Chen, Sheng-Ying Chung, Pei-Hsun Sung, Hsueh-Wen Chang, Mel S Lee, Hon-Kan Yip
This study tested the hypothesis that circulating microparticles (MPs) exacerbated vascular wall (VW) remodeling after endothelial denudation by 0.014 wire in a rat model. Adult male Sprague Dawley rats (n = 40) were equally categorized into group 1 [sham-control (SC); 3.0 mL saline intravenous injection], group 2 [SC + intravenous MPs (1.0 × 10(7)) derived from patients with carotid artery stenosis (CAS)], group 3 [femoral arterial endothelial denudation (FAED)], group 4 (FAED + MPs derived from healthy subjects), and group 5 (FAED + CAS-derived MPs)...
2016: American Journal of Translational Research
https://www.readbyqxmd.com/read/27903833/meal-distribution-of-dietary-protein-and-leucine-influences-long-term-muscle-mass-and-body-composition-in-adult-rats
#4
Layne E Norton, Gabriel J Wilson, Christopher J Moulton, Donald K Layman
BACKGROUND: Protein quantity and quality at a meal affect muscle protein synthesis (MPS); however, long-term effects of protein distribution at individual meals on adult muscle mass remain unknown. OBJECTIVE: We used a precise feeding protocol in adult rats to determine if optimizing postmeal MPS response by modifying the meal distribution of protein, and the amino acid leucine (Leu), would affect muscle mass. METHODS: Two studies were conducted with the use of male Sprague-Dawley rats (∼300 g) trained to consume 3 meals/d, then assigned to diet treatments with identical macronutrient contents (16% of energy from protein, 54% from carbohydrates, and 30% from fat) but differing in protein quality or meal distribution...
November 30, 2016: Journal of Nutrition
https://www.readbyqxmd.com/read/27903231/new-analogues-of-mycophenolic-acid
#5
Agnieszka Głowacka, Michal Prejs, Grzegorz Cholewinski, Krystyna Dzierzbicka
Mycophenolic acid (MPA) possesses antibacterial, antifungal, antiviral, immunosuppressive and anticancer properties. It is a non-competitive and reversible inhibitor of dehydrogenase inosine-5'-monophosphate (IMPDH) [1,2]. This compound belongs to the immunosuppressive drugs used for the prevention of both acute and chronic transplant rejection [3]. Until now clinically have been used two derivatives of MPA: mycophenolate mofetil (MMF, CellCept) and mycophenolate sodium (MPS, Myfortic). They cause, similar to MPA, although at lower degree, the side effects such as vomiting, abdominal pain, diarrhea, nausea, gastrointestinal, urogenital tract, blood or nervous system disorders...
November 29, 2016: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/27900452/microparticles-from-splenectomized-%C3%AE-thalassemia-hbe-patients-play-roles-on-procoagulant-activities-with-thrombotic-potential
#6
Phatchanat Klaihmon, Kunwadee Phongpao, Wasinee Kheansaard, Egarit Noulsri, Archrob Khuhapinant, Suthat Fucharoen, Noppawan Phumala Morales, Saovaros Svasti, Kovit Pattanapanyasat, Pornthip Chaichompoo
Thromboembolic events including cerebral thrombosis, deep vein thrombosis, and pulmonary embolism are major complications in β-thalassemia. Damaged red blood cells and chronic platelet activation in splenectomized β-thalassemia/HbE patients were associated with increased microparticles (MPs) releases into blood circulation. MPs are small membrane vesicles, which play important roles on coagulation. However, the role of MP in thalassemia is poorly understood. In this study, the effects of splenectomized-MPs on platelet activation and aggregation were investigated...
November 29, 2016: Annals of Hematology
https://www.readbyqxmd.com/read/27888093/randomized-trial-of-ratg-daclizumab-vs-ratg-alemtuzumab-as-dual-induction-therapy-in-renal-transplantation-results-at-8-years-of-follow-up
#7
Gaetano Ciancio, Jeffrey J Gaynor, Giselle Guerra, Junichiro Sageshima, David Roth, Linda Chen, Warren Kupin, Adela Mattiazzi, Lissett Tueros, Sandra Flores, Lois Hanson, Phillip Ruiz, Rodrigo Vianna, George W Burke
Our goal in using dual induction therapy is to bring the kidney transplant recipient closer (through more effectively timed lymphodepletion) to an optimally immunosuppressed state. Here, we report long-term results of a prospective randomized trial comparing (Group I,N=100) rATG/Dac (3 rATG, 2 Dac doses) vs. (Group II,N=100) rATG/Alemtuzumab(C1H) (1 dose each), using reduced tacrolimus dosing, EC-MPS, and early corticosteroid withdrawal. Lower EC-MPS dosing was targeted in Group II to avoid severe leukopenia...
November 22, 2016: Transplant Immunology
https://www.readbyqxmd.com/read/27882161/potential-diagnostic-role-of-diffusion-tensor-imaging-in-early-stage-osteonecrosis-of-the-femoral-head
#8
Hongwei Min, Feng Xu, Rui Gu, Xinzuo Han, Anqing Wang, Kemin Liu
The present study aimed to explore the potential diagnostic role of diffusion tensor magnetic resonance imaging (DTI) in the early stage of modified corticosteroid-induced osteonecrosis of the femoral head (ONFH). A total of 20 beagles were randomly classified (1:1) into either an experimental group (LM), which were intramuscularly injected with lipopolysaccharide (LPS) and methylprednisolone (MPS) on three consecutive days, or control (CON) group, which were injected with saline. Magnetic resonance imaging (MRI) and DTI were performed at pre-induction and 8 and 12 weeks post-induction...
November 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27876528/the-impact-of-lens-care-solutions-on-corneal-epithelial-changes-during-daily-silicone-hydrogel-contact-lens-wear-as-measured-by-in-vivo-confocal-microscopy
#9
Xiaolin Zhang, Christine Marchetti, Jessica Lee, Yan Sun, Sara Debanne, Ying Jiang, Jami Kern, Mark Harrod, Beth Ann Benetz, Eric Pearlman, Loretta Szczotka-Flynn
PURPOSE: To assess corneal epithelial microstructure via confocal microscopy and determine if cellular changes are associated with lens care solutions during daily wear of silicone hydrogel contact lenses. METHODS: Corneal in vivo confocal microscopy with the Nidek ConfoScan4 was performed at baseline and after 5 months of lotrafilcon A daily contact lens wear. Enrolled participants were randomized to use either a polyhexamethylene biguanide (PHMB) preserved multipurpose care solution (MPS) or a peroxide based solution system...
November 19, 2016: Contact Lens & Anterior Eye: the Journal of the British Contact Lens Association
https://www.readbyqxmd.com/read/27873516/cyclosporine-sparing-effect-of-enteric-coated-mycophenolate-sodium-in-de-novo-kidney-transplantation
#10
Su Hyung Lee, Jae Berm Park, Chang Kwon Oh, Myoung Soo Kim, Sung Joo Kim, Jongwon Ha
PURPOSE: The increased tolerability of enteric-coated mycophenolate sodium (EC-MPS), compared to mycophenolate mofetil, among kidney transplant recipients has the potential to facilitate cyclosporine (CsA) minimization. Therefore, a prospective trial to determine the optimum EC-MPS dose in CsA-based immunosuppression regimens is necessary. MATERIALS AND METHODS: A comparative, parallel, randomized, open-label study was performed for 140 patients from four centers to compare the efficacy and tolerability of low dose CsA with standard dose EC-MPS (the investigational group) versus standard dose CsA with low dose EC-MPS (the control group) for six months in de novo kidney transplant recipients...
January 2017: Yonsei Medical Journal
https://www.readbyqxmd.com/read/27810364/lack-of-the-p2x7-receptor-protects-against-amd-like-defects-and-microparticle-accumulation-in-a-chronic-oxidative-stress-induced-mouse-model-of-amd
#11
Kyle A Carver, C M Lin, Catherine Bowes Rickman, Dongli Yang
The P2X7 receptor (P2X7R) is an ATP-gated ion channel that is a key player in oxidative stress under pathological conditions. The P2X7R is expressed in the retinal pigmented epithelium (RPE) and neural retina. Chronic oxidative stress contributes to the pathogenesis of age-related macular degeneration (AMD). Mice lacking Cu, Zn superoxide dismutase (Sod1) developed chronic oxidative stress as well as AMD-like features, but whether the P2X7R plays a causative role in oxidative stress-induced AMD is unknown. Thus, the main purpose of this study was to test if concurrent knockout (KO) of P2X7R could block AMD-like defects seen in Sod1 KO mice...
October 31, 2016: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/27802066/analgesic-effect-of-botulinum-toxin-a-in-myofascial-pain-syndrome-patients-previously-treated-with-local-infiltration-of-anesthetic-and-steroids
#12
Joaquín Cartagena-Sevilla, María R García-Fernández, Juan P Vicente-Villena
The purpose of this study was to evaluate the analgesic effect of botulinum toxin A (BoNTA) injections in patients with myofascial pain syndrome (MPS) who were previously treated with the local infiltration of anesthetic and steroids (LIAS). The study included a retrospective phase and a longitudinal open-label prospective phase, which were conducted on consecutive patients with MPS previously treated with the local infiltration of anesthetic (levobupivacaíne 0.25%) and steroids (triamcinolone 40 mg). Eligible patients were treated with a single intramuscular injection of BoNTA (Botox; Allergan, Inc...
November 1, 2016: Journal of Pain & Palliative Care Pharmacotherapy
https://www.readbyqxmd.com/read/27797586/novel-mutations-including-a-large-deletion-in-the-arsb-gene-causing-mucopolysaccharidosis-type-vi
#13
Chupong Ittiwut, Sukanya Boonbuamas, Chalurmpon Srichomthong, Rungnapa Ittiwut, Kanya Suphapeetiporn, Vorasuk Shotelersuk
OBJECTIVE: Mucopolysaccharidosis type VI (MPS VI; Maroteaux-Lamy syndrome), a rare autosomal recessive lysosomal storage disease, is caused by mutations in the N-acetylgalactosamine-4-sulfatase (arylsulfatase B, or ARSB) gene, resulting in a deficiency of ARSB activity. This study aimed to characterize the clinical and molecular features of four unrelated Thai patients with MPS VI. Two were products of consanguineous marriages. MATERIALS AND METHODS: The diagnosis was confirmed by biochemical and genetic tests...
October 31, 2016: Genetic Testing and Molecular Biomarkers
https://www.readbyqxmd.com/read/27792124/erythrocyte-derived-microparticles-activate-pulmonary-endothelial-cells-in-a-murine-model-of-transfusion
#14
Alex L Chang, Young Kim, Aaron P Seitz, Rebecca M Schuster, Alex B Lentsch, Timothy A Pritts
Erythrocyte-derived microparticles (MPs) are sub-micrometer, biologically active vesicles shed by red blood cells as part of the biochemical changes that occur during storage. We hypothesized that MPs from stored red blood cells would activate endothelial cells. MPs from aged murine packed red blood cells (pRBCs) were isolated and used to treat confluent layers of cultured endothelial cells. Endothelial expression of leukocyte adhesion molecules, ELAM-1 and ICAM-1, and inflammatory mediator, IL-6, were evaluated at 0...
October 27, 2016: Shock
https://www.readbyqxmd.com/read/27789401/generation-of-human-induced-pluripotent-stem-cell-ipsc-line-from-an-unaffected-female-carrier-of-mucopolysaccharidosis-type-ii-mps-ii-disorder
#15
Eszter Varga, Csilla Nemes, Eszter Kovács, István Bock, Norbert Varga, Anita Fehér, András Dinnyés, Julianna Kobolák
Peripheral blood was collected from a 39-year-old unaffected female carrier of an X-linked recessive mutation of Iduronate 2-sulfatase gene (NM_000202.7(IDS):c.85C>T) causing MPS II (OMIM 309900). Peripheral blood mononuclear cells (PBMCs) were reprogrammed by lentiviral delivery of a self-silencing hOKSM polycistronic vector. The pluripotency of iPSC line was confirmed by the expression of pluripotency-associated markers and in vitro spontaneous differentiation towards the 3 germ layers. The iPSC showed normal karyotype...
October 3, 2016: Stem Cell Research
https://www.readbyqxmd.com/read/27789399/generation-of-mucopolysaccharidosis-type-ii-mps-ii-human-induced-pluripotent-stem-cell-ipsc-line-from-a-1-year-old-male-with-pathogenic-ids-mutation
#16
Eszter Varga, Csilla Nemes, István Bock, Norbert Varga, Anita Fehér, András Dinnyés, Julianna Kobolák
Peripheral blood was collected from a 1-year-old male patient with an X-linked recessive mutation of Iduronate 2-sulfatase (IDS) gene (NM_000202.7(IDS):c.85C>T) causing MPS II (OMIM 309900). Peripheral blood mononuclear cells (PBMCs) were reprogrammed by lentiviral delivery of a self-silencing hOKSM polycistronic vector. The pluripotency of the iPSC line was confirmed by the expression of pluripotency-associated markers and in vitro spontaneous differentiation towards the 3 germ layers. The iPSC line showed normal karyotype...
October 1, 2016: Stem Cell Research
https://www.readbyqxmd.com/read/27789398/generation-of-mucopolysaccharidosis-type-ii-mps-ii-human-induced-pluripotent-stem-cell-ipsc-line-from-a-3-year-old-male-with-pathogenic-ids-mutation
#17
Eszter Varga, Csilla Nemes, István Bock, Norbert Varga, Anita Fehér, Julianna Kobolák, András Dinnyés
Peripheral blood was collected from a 3-year-old male patient with an X-linked recessive mutation of Iduronate 2-sulfatase (IDS) gene (NM_000202.7(IDS):c.85C>T) causing MPS II (OMIM 309900). Peripheral blood mononuclear cells (PBMCs) were reprogrammed by lentiviral delivery of a self-silencing hOKSM polycistronic vector. The pluripotency of the iPSC line was confirmed by the expression of pluripotency-associated markers and in vitro spontaneous differentiation towards the 3 germ layers. The iPSC line showed normal karyotype...
October 1, 2016: Stem Cell Research
https://www.readbyqxmd.com/read/27789394/generation-of-mucopolysaccharidosis-type-ii-mps-ii-human-induced-pluripotent-stem-cell-ipsc-line-from-a-7-year-old-male-with-pathogenic-ids-mutation
#18
Eszter Varga, Csilla Nemes, István Bock, Norbert Varga, Anita Fehér, Julianna Kobolák, András Dinnyés
Peripheral blood was collected from a 7-year-old male patient with an X-linked recessive mutation of Iduronate 2-sulfatase (IDS) gene (NM_000202.7(IDS):c.182C>T) causing MPS II (OMIM 309900). Peripheral blood mononuclear cells (PBMCs) were reprogrammed by lentiviral delivery of a self-silencing hOKSM polycistronic vector. The pluripotency of the iPSC line was confirmed by the expression of pluripotency-associated markers and in vitro spontaneous differentiation towards the 3 germ layers. The iPSC line showed normal karyotype...
October 1, 2016: Stem Cell Research
https://www.readbyqxmd.com/read/27769462/green-synthesis-of-mesoporous-molecular-sieve-incorporated-monoliths-using-room-temperature-ionic-liquid-and-deep-eutectic-solvents
#19
Li-Shun Zhang, Qing-Li Zhao, Xin-Xin Li, Xi-Xi Li, Yan-Ping Huang, Zhao-Sheng Liu
A hybrid monolith incorporated with mesoporous molecular sieve MCM-41 of uniform pore structure and high surface area was prepared with binary green porogens in the first time. With a mixture of room temperature ionic liquids and deep eutectic solvents as porogens, MCM-41 was modified with 3-(trimethoxysilyl) propyl methacrylate (γ-MPS) and the resulting MCM-41-MPS was incorporated into poly (BMA-co-EDMA) monoliths covalently. Because of good dispersibility of MCM-41-MPS in the green solvent-based polymerization system, high permeability and homogeneity for the resultant hybrid monolithic columns was achieved...
December 1, 2016: Talanta
https://www.readbyqxmd.com/read/27765042/circulating-microparticles-in-severe-pulmonary-arterial-hypertension-increase-intercellular-adhesion-molecule-1-expression-selectively-in-pulmonary-artery-endothelium
#20
Leslie A Blair, April K Haven, Natalie N Bauer
BACKGROUND: Microparticles (MPs) stimulate inflammatory adhesion molecule expression in systemic vascular diseases, however it is unknown whether circulating MPs stimulate localized ICAM-1 expression in the heterogeneically distinct pulmonary endothelium during pulmonary arterial hypertension (PAH). Pulmonary vascular lesions with infiltrating inflammatory cells in PAH form in the pulmonary arteries and arterioles, but not the microcirculation. Therefore, we sought to determine whether circulating MPs from PAH stimulate pulmonary artery endothelial cell-selective ICAM-1 expression...
October 20, 2016: Respiratory Research
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