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https://www.readbyqxmd.com/read/28341561/duvoglustat-hcl-increases-systemic-and-tissue-exposure-of-active-acid-%C3%AE-glucosidase-in-pompe-patients-co-administered-with-alglucosidase-%C3%AE
#1
Priya Kishnani, Mark Tarnopolsky, Mark Roberts, Kumarswamy Sivakumar, Majed Dasouki, Mazen M Dimachkie, Erika Finanger, Ozlem Goker-Alpan, Karl A Guter, Tahseen Mozaffar, Muhammad Ali Pervaiz, Pascal Laforet, Todd Levine, Matthews Adera, Richard Lazauskas, Sheela Sitaraman, Richie Khanna, Elfrida Benjamin, Jessie Feng, John J Flanagan, Jay Barth, Carrolee Barlow, David J Lockhart, Kenneth J Valenzano, Pol Boudes, Franklin K Johnson, Barry Byrne
Duvoglustat HCl (AT2220, 1-deoxynojirimycin) is an investigational pharmacological chaperone for the treatment of acid α-glucosidase (GAA) deficiency, which leads to the lysosomal storage disorder Pompe disease, which is characterized by progressive accumulation of lysosomal glycogen primarily in heart and skeletal muscles. The current standard of care is enzyme replacement therapy with recombinant human GAA (alglucosidase alfa [AA], Genzyme). Based on preclinical data, oral co-administration of duvoglustat HCl with AA increases exposure of active levels in plasma and skeletal muscles, leading to greater substrate reduction in muscle...
March 21, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28332088/simple-technique-of-repair-for-barlow-syndrome-with-posterior-resection-and-chordal-transfer-via-minimally-invasive-approach-primary-experience-in-a-consecutive-series-of-22-patients
#2
H Kamiya, Payam Akhyari, J-P Minol, A C Ites, T Weinreich, S Sixt, P Rellecke, U Boeken, A Albert, A Lichtenberg
OBJECTIVE: Current techniques for mitral valve repair (MVR) in Barlow's disease require high level of surgical expertise due to a complex anatomy. A novel and simple standardized technique that particularly considers the pathological changes of the mitral valve in Barlow's disease has been developed. METHODS: Between 2009 and 2013, 22 patients underwent minimally invasive MVR for Barlow's disease and severe mitral regurgitation (MR). A simple, standardized technique was applied, including resection of P2 segment of posterior mitral leaflet (PML) with preservation of the shortest chordae, transfer of the preserved chordae to A2, and implantation of a semi-rigid open ring...
March 22, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28328293/history-of-knowledge-and-evolution-of-occupational-health-and-regulatory-aspects-of-asbestos-exposure-science-1900-1975
#3
Christy A Barlow, Jennifer Sahmel, Dennis J Paustenbach, John L Henshaw
The understanding by industrial hygienists of the hazards of asbestos and appropriate ways to characterize and control exposure has evolved over the years. Here, a detailed analysis of the evolution of industrial hygiene practices regarding asbestos and its health risks, from the early 1900s until the advent of the national occupational health and safety regulatory structure currently in place in the US (early-to-mid 1970s) is presented. While industrial hygienists recognized in the early 1900s that chronic and high-level exposures to airborne concentrations of asbestos could pose a serious health hazard, it was not until the mid-1950s that the carcinogenic nature of asbestos began to be characterized and widespread concern followed...
March 22, 2017: Critical Reviews in Toxicology
https://www.readbyqxmd.com/read/28323949/plasma-arginine-asymmetric-dimethylarginine-ratio-and-incidence-of-cardiovascular-events-a-case-cohort-study
#4
Edward Yu, Miguel Ruiz-Canela, Frank B Hu, Clary B Clish, Dolores Corella, Jordi Salas-Salvadó, Adela Hruby, Montserrat Fitó, Liming Liang, Estefania Toledo, Emilio Ros, Ramón Estruch, Enrique Gómez-Gracia, Jose Lapetra, Fernando Arós, Dora Romaguera, Lluís Serra-Majem, Marta Guasch-Ferré, Dong D Wang, Miguel A Martínez-González
CONTEXT: Arginine, its methylated metabolites and other metabolites related to the urea cycle have been independently associated with cardiovascular risk, but the potential causal meaning of these associations (positive for some metabolites and negative for others) remains elusive due to a lack of studies measuring metabolite changes over time. OBJECTIVE: To examine the association between baseline and 1-year concentrations of urea cycle metabolites and cardiovascular disease in a case-cohort setting...
March 2, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28321329/utility-of-the-wireless-motility-capsule-and-lactulose-breath-testing-in-the-evaluation-of-patients-with-parkinson-s-disease-who-present-with-functional-gastrointestinal-symptoms
#5
Andrew Su, Rita Gandhy, Carrolee Barlow, George Triadafilopoulos
BACKGROUND: The aetiology and origin of gastrointestinal symptoms in Parkinson's disease (PD) remains poorly understood. Gastroparesis, small bowel transit delay and bacterial overgrowth may, individually or collectively, play a role. AIMS: In patients with PD and functional gastrointestinal symptoms, we aimed to determine the utility of the wireless motility capsule and lactulose breath tests in further defining their symptoms' aetiology. METHODS: In this retrospective cohort study, consecutive patients with PD and functional gastrointestinal symptoms underwent clinical assessment, as well as wireless motility capsule and lactulose breath testing using standard protocols...
2017: BMJ Open Gastroenterology
https://www.readbyqxmd.com/read/28319322/an-analysis-of-the-survival-outcomes-of-simultaneous-pancreas-and-kidney-transplantation-compared-to-live-donor-kidney-transplantation-in-patients-with-type-1-diabetes-a-uk-transplant-registry-study
#6
Adam D Barlow, Kourosh Saeb-Parsy, Christopher J E Watson
BACKGROUND: Transplant options for patients with type 1 diabetes and end-stage renal disease (ESRD) include deceased donor kidney, live donor kidney (LDK) and simultaneous pancreas-kidney (SPK) transplantation. The aim of this study was to compare outcomes between LDK and SPK for patients with type 1 diabetes and ESRD in the UK. METHODS: Data on all SPK (n=1739) and LDK (n=385) transplants performed between January 2001 and December 2014 were obtained from the UK Transplant Registry...
March 20, 2017: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/28302452/lactobacillus-rhamnosus-endocarditis-an-unusual-culprit-in-a-patient-with-barlow-s-disease
#7
Ioannis Felekos, George Lazaros, Athanasia Tsiriga, Maria Pirounaki, George Stavropoulos, Joseph Paraskevas, Marina Toutouza, Dimitrios Tousoulis
No abstract text is available yet for this article.
November 16, 2016: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/28290825/-myocardial-crypt-of-the-left-ventricle-combined-with-barlows-syndrome
#8
S L Dzemeshkevich, V E Sinitsyn, Yu V Frolova, S V Fedulova, E V Zaklyazminskaya, A A Bukaeva, D G Tarasov, M S Malikova, A V Dombrovskaya, M E Polyak
Myocardial crypts were initially described in patients with hypertrophic cardiomyopathy. Modern diagnostic data show that this structural abnormality can be found in patients with other diseases, or might represent the variant of normal heart development in healthy individuals. The prognostic significance of this finding is uncertain. In this publication we present a clinical case of the combination of myocardial crypt and Barlows syndrome.
December 2016: Kardiologiia
https://www.readbyqxmd.com/read/28274238/the-health-impact-of-trade-and-investment-agreements-a-quantitative-systematic-review-and-network-co-citation-analysis
#9
Pepita Barlow, Martin McKee, Sanjay Basu, David Stuckler
BACKGROUND: Regional trade agreements are major international policy instruments that shape macro-economic and political systems. There is widespread debate as to whether and how these agreements pose risks to public health. Here we perform a comprehensive systematic review of quantitative studies of the health impact of trade and investment agreements. We identified studies from searches in PubMed, Web of Science, EMBASE, and Global Health Online. Research articles were eligible for inclusion if they were quantitative studies of the health impacts of trade and investment agreements or policy...
March 8, 2017: Globalization and Health
https://www.readbyqxmd.com/read/28258927/a-practical-review-of-gastrointestinal-manifestations-in-parkinson-s-disease
#10
REVIEW
Andrew Su, Rita Gandhy, Carrolee Barlow, George Triadafilopoulos
Parkinson's disease (PD) is a chronic neurodegenerative disease with prominent motor and non-motor symptoms. Gastrointestinal (GI) dysfunction is among the most common and bothersome of non-motor symptoms that physicians will encounter while caring for their patients. Patients are subject to a wide variety of GI symptoms involving organs from the oropharynx to the anorectum. Our awareness and understanding of GI involvement in PD continues to evolve. In this review, we use a gastroenterologist's perspective to provide practical considerations for the diagnosis and symptom-based management of GI dysfunction seen in PD...
February 23, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28242319/white-paper-aga-power-practice-guide-on-obesity-and%C3%A2-weight-management-education-and-resources
#11
REVIEW
Andres Acosta, Sarah Streett, Mathew D Kroh, Lawrence J Cheskin, Katherine H Saunders, Marina Kurian, Marsha Schofield, Sarah E Barlow, Louis Aronne
The epidemic of obesity continues at alarming rates, with a high burden to our economy and society. The American Gastroenterological Association understands the importance of embracing obesity as a chronic, relapsing disease and supports a multidisciplinary approach to the management of obesity. Because gastrointestinal disorders resulting from obesity are more frequent and often present sooner than type 2 diabetes mellitus and cardiovascular disease, gastroenterologists have an opportunity to address obesity and provide an effective therapy early...
February 24, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28213528/leptospira-interrogans-serogroup-pomona-infections-in-the-uk-is-there-a-real-threat-for-farm-animals
#12
Z Arent, C Gilmore, A M Barlow, L Smith, W A Ellis
Current typing of Leptospira strains requires a combination of both serological and molecular methods. Study of the epidemiology of strains within the Pomona serogroup has proved problematic due to the limitations of serological typing methods. This is particularly true of the two main complexes within this group, namely the Pomona, Monjakov, Kennewicki cluster in Leptospira interrogans species and the Mozdok, Tsaratsovo, Kunming, Altodouro cluster in Leptospira kirschneri Strains from the south of England have been shown previously to belong to L kirschneri serovar Mozdok, strains of which are maintained by small rodents in western Europe...
February 17, 2017: Veterinary Record
https://www.readbyqxmd.com/read/28203545/robotic-mitral-repair-for-barlow-s-disease-with-bileaflet-prolapse-and-annular-calcification-using-pericardial-patch-technique
#13
REVIEW
Didier F Loulmet, Michael S Koeckert, Peter J Neuburger, Robert Nampiaparampil, Eugene A Grossi
No abstract text is available yet for this article.
January 2017: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28179525/differentiation-dependent-lmp1-expression-is-required-for-efficient-lytic-ebv-reactivation-in-epithelial-cells
#14
Dhananjay M Nawandar, Makoto Ohashi, Reza Djavadian, Elizabeth Barlow, Kathleen Makielski, Ahmed Ali, Denis Lee, Paul F Lambert, Eric Johannsen, Shannon C Kenney
Epstein-Barr virus (EBV)-associated diseases of epithelial cells, including tumors such as nasopharyngeal carcinoma (NPC) that have latent infection, and oral hairy leukoplakia (OHL) lesions that have lytic infection, frequently express the viral latent membrane protein 1 (LMP1). In lytically-infected cells, LMP1 expression is activated by the BRLF1 (R) immediate-early (IE) protein. However, the mechanisms by which LMP1 expression is normally regulated in epithelial cells remain poorly understood, and its potential roles in regulating lytic reactivation in epithelial cells are as yet unexplored...
February 8, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28165796/crohn-s-disease-for-the-general-physician-management
#15
J J Barlow, A B Hawthorne
Crohn's disease presents to general physicians in a variety of ways. The previous article outlined clinical features and initial investigations, and this article covers management of Crohn's disease, including monitoring and drug toxicity.
February 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/28165787/crohn-s-disease-for-the-general-physician-presentation-and-investigations
#16
J J Barlow, A B Hawthorne
Crohn's disease presents to general physicians in a variety of ways. This article outlines the clinical features and initial investigation of suspected Crohn's disease. The accompanying article reviews treatment strategies.
February 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/28154909/beyond-moulage-sign-and-ttg-levels-the-role-of-cross-sectional-imaging-in-celiac-sprue
#17
Shannon P Sheedy, John M Barlow, Joel G Fletcher, Thomas C Smyrk, Francis J Scholz, Don C Codipilly, Badr F Al Bawardy, Jeff L Fidler
Celiac disease is an autoimmune disorder that causes inflammation and destruction in the small intestine of genetically susceptible individuals following ingestion of gluten. Awareness of the disease has increased; however, it remains a challenge to diagnose. This review summarizes the intestinal and extraintestinal cross-sectional imaging findings of celiac disease. Small intestine fold abnormalities are the most specific imaging findings for celiac disease, whereas most other imaging findings reflect a more generalized pattern seen with malabsorptive processes...
February 2, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28107283/naspghan-clinical-practice-guideline-for-the-diagnosis-and-treatment-of-nonalcoholic-fatty-liver-disease-in-children-recommendations-from-the-expert-committee-on-nafld-econ-and-the-north-american-society-of-pediatric-gastroenterology-hepatology-and-nutrition
#18
Miriam B Vos, Stephanie H Abrams, Sarah E Barlow, Sonia Caprio, Stephen R Daniels, Rohit Kohli, Marialena Mouzaki, Pushpa Sathya, Jeffrey B Schwimmer, Shikha S Sundaram, Stavra A Xanthakos
Nonalcoholic fatty liver disease (NAFLD) is a highly prevalent chronic liver disease that occurs in the setting of insulin resistance and increased adiposity. It has rapidly evolved into the most common liver disease seen in the pediatric population and is a management challenge for general pediatric practitioners, subspecialists, and for health systems. In this guideline, the expert committee on NAFLD reviewed and summarized the available literature, formulating recommendations to guide screening and clinical care of children with NAFLD...
February 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28106961/optimal-surgical-mitral-valve-repair-in-barlow-s-disease-the-concept-of-functional-prolapse
#19
Rjm Klautz, A Tomi, M Palmen, T J van Brakel, Patrick Perier
Barlow's disease presents the most severe form of degenerative mitral valve disease as it normally affects various valvular structures and segments. We discuss the technical aspects of mitral valve repair in this setting. Furthermore, the concept of "functional prolapse" of the mitral valve is discussed as well as recommendations on when to proceed with surgical correction of anterior leaflet prolapse.
December 6, 2016: Multimedia Manual of Cardiothoracic Surgery: MMCTS
https://www.readbyqxmd.com/read/28103959/validation-of-a-case-definition-for-pediatric-brain-injury-using-administrative-data
#20
Jane McChesney-Corbeil, Karen Barlow, Hude Quan, Guanmin Chen, Samuel Wiebe, Nathalie Jette
BACKGROUND: Health administrative data are a common population-based data source for traumatic brain injury (TBI) surveillance and research; however, before using these data for surveillance, it is important to develop a validated case definition. The objective of this study was to identify the optimal International Classification of Disease , edition 10 (ICD-10), case definition to ascertain children with TBI in emergency room (ER) or hospital administrative data. We tested multiple case definitions...
March 2017: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
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