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barlow disease

Zahir H Alshehry, Piyushkumar A Mundra, Christopher K Barlow, Natalie A Mellett, Gerard Wong, Malcolm J McConville, John Simes, Andrew M Tonkin, David R Sullivan, Elizabeth H Barnes, Paul J Nestel, Bronwyn A Kingwell, Michel Marre, Bruce Neal, Neil R Poulter, Anthony Rodgers, Bryan Williams, Sophia Zoungas, Graham S Hillis, John Chalmers, Mark Woodward, Peter J Meikle
BACKGROUND: -Clinical lipid measurements do not show the full complexity of the altered lipid metabolism associated with diabetes or cardiovascular disease. Lipidomics enables the assessment of hundreds of lipid species as potential markers for disease risk. METHODS: -Plasma lipid species (310) were measured by a targeted lipidomic analysis with liquid chromatography electrospray ionisation-tandem mass spectrometry on a case-cohort (n=3,779) subset from the ADVANCE (Action in Diabetes and Vascular disease: preterAx and diamicroN-MR Controlled Evaluation) trial...
October 18, 2016: Circulation
Ryan W Himes, Sarah E Barlow, Kevin Bove, Norma M Quintanilla, Rachel Sheridan, Rohit Kohli
Lysosomal acid lipase deficiency (LAL-D) is a classic lysosomal storage disorder characterized by accumulation of cholesteryl ester and triglyceride. Although it is associated with progressive liver injury, fibrosis, and end-stage liver disease in children and adolescents, LAL-D frequently presents with nonspecific signs that overlap substantially with other, more common, chronic conditions like nonalcoholic fatty liver disease (NAFLD), metabolic syndrome, and certain inherited dyslipidemias. We present 2 children with NAFLD who achieved clinically significant weight reduction through healthy eating and exercise, but who failed to have the anticipated improvements in aminotransferases and γ-glutamyl transferase...
September 13, 2016: Pediatrics
Jeffrey B Schwimmer, Joel E Lavine, Laura A Wilson, Brent A Neuschwander-Tetri, Stavra A Xanthakos, Rohit Kohli, Sarah E Barlow, Miriam B Vos, Saul J Karpen, Jean P Molleston, Peter F Whitington, Philip Rosenthal, Ajay K Jain, Karen F Murray, Elizabeth M Brunt, David E Kleiner, Mark L Van Natta, Jeanne M Clark, James Tonascia, Edward Doo
BACKGROUND & AIMS: No treatment for nonalcoholic fatty liver disease (NAFLD) has been approved by regulatory agencies. We performed a randomized controlled trial to determine whether 52 weeks of cysteamine bitartrate delayed release (CBDR) reduces the severity of liver disease in children with NAFLD. METHODS: We performed a double-masked trial of 169 children with NAFLD Activity Scores ≥ 4 at 10 centers. From June 2012 to January 2014, the patients were randomly assigned to receive CBDR or placebo twice daily (300 mg for ≤65 kg, 375 mg for >65-80 kg, 450 mg for >80 kg) for 52 weeks...
August 25, 2016: Gastroenterology
Astrid Apor, Anikó Ilona Nagy, Attila Kovács, Aristomenis Manouras, Péter Andrássy, Béla Merkely
BACKGROUND: Real-time three-dimensional transesophageal echocardiography has increased our understanding of the distinct pathomechanisms underlying functional, ischaemic or degenerative mitral regurgitation. However, potential differences in dynamic morphology between the subtypes of degenerative mitral prolapse have scarcely been investigated. METHODS: In order to compare the dynamic behavior of the different phenotypes of degenerative mitral valve prolapse, real-time three-dimensional transesophageal echocardiography recordings of 77 subjects, 27 with Barlow disease (BD), 32 with Fibroelastic deficiency (FED) and 18 normal controls (NC) were analysed...
2016: Cardiovascular Ultrasound
Ziqing Yu, Bing Fan, Hongyi Wu, Xiangfei Wang, Chenguang Li, Rende Xu, Yangang Su, Junbo Ge
BACKGROUND: Systemic embolism, especially septic embolism, is a severe complication of infective endocarditis (IE). However, concurrent embolism to the brain, coronary arteries, and spleen is very rare. Because of the risk of hemorrhage or visceral rupture, anticoagulants are recommended only if an indication is present, e.g. prosthetic valve. Antiplatelet therapy in IE is controversial, but theoretically, this therapy has the potential to prevent and treat thrombosis and embolism in IE...
August 11, 2016: BMC Infectious Diseases
Dominique P Germain, Derralynn A Hughes, Kathleen Nicholls, Daniel G Bichet, Roberto Giugliani, William R Wilcox, Claudio Feliciani, Suma P Shankar, Fatih Ezgu, Hernan Amartino, Drago Bratkovic, Ulla Feldt-Rasmussen, Khan Nedd, Usama Sharaf El Din, Charles M Lourenco, Maryam Banikazemi, Joel Charrow, Majed Dasouki, David Finegold, Pilar Giraldo, Ozlem Goker-Alpan, Nicola Longo, C Ronald Scott, Roser Torra, Ahmad Tuffaha, Ana Jovanovic, Stephen Waldek, Seymour Packman, Elizabeth Ludington, Christopher Viereck, John Kirk, Julie Yu, Elfrida R Benjamin, Franklin Johnson, David J Lockhart, Nina Skuban, Jeff Castelli, Jay Barth, Carrolee Barlow, Raphael Schiffmann
BACKGROUND: Fabry's disease, an X-linked disorder of lysosomal α-galactosidase deficiency, leads to substrate accumulation in multiple organs. Migalastat, an oral pharmacologic chaperone, stabilizes specific mutant forms of α-galactosidase, increasing enzyme trafficking to lysosomes. METHODS: The initial assay of mutant α-galactosidase forms that we used to categorize 67 patients with Fabry's disease for randomization to 6 months of double-blind migalastat or placebo (stage 1), followed by open-label migalastat from 6 to 12 months (stage 2) plus an additional year, had certain limitations...
August 11, 2016: New England Journal of Medicine
R André, A Gabrielli, E Laffitte, O Kherad
INTRODUCTION: Scurvy, or "Barlow's disease", is a widely described disease involving cutaneous and mucosal lesions resulting from vitamin C deficiency. Herein, we report a case of scurvy in a 48-year-old woman that was unusual in its atypical cutaneous-mucosal presentation as well as its association with anorexia nervosa. PATIENTS AND METHODS: A 48-year-old woman treated for depression for several years was admitted to hospital for her impaired general state of health...
August 5, 2016: Annales de Dermatologie et de Vénéréologie
Dana Meaney-Delman, Titilope Oduyebo, Kara N D Polen, Jennifer L White, Andrea M Bingham, Sally A Slavinski, Lea Heberlein-Larson, Kirsten St George, Jennifer L Rakeman, Susan Hills, Christine K Olson, Alys Adamski, Lauren Culver Barlow, Ellen H Lee, Anna M Likos, Jorge L Muñoz, Emily E Petersen, Elizabeth M Dufort, Amy B Dean, Margaret M Cortese, Gilberto A Santiago, Julu Bhatnagar, Ann M Powers, Sherif Zaki, Lyle R Petersen, Denise J Jamieson, Margaret A Honein
OBJECTIVE: Zika virus infection during pregnancy is a cause of microcephaly and other fetal brain abnormalities. Reports indicate that the duration of detectable viral RNA in serum after symptom onset is brief. In a recent case report involving a severely affected fetus, Zika virus RNA was detected in maternal serum 10 weeks after symptom onset, longer than the duration of RNA detection in serum previously reported. This report summarizes the clinical and laboratory characteristics of pregnant women with prolonged detection of Zika virus RNA in serum that were reported to the U...
October 2016: Obstetrics and Gynecology
Kimberly P Newton, Jiayi Hou, Nancy A Crimmins, Joel E Lavine, Sarah E Barlow, Stavra A Xanthakos, Jonathan Africa, Cynthia Behling, Michele Donithan, Jeanne M Clark, Jeffrey B Schwimmer
Importance: Nonalcoholic fatty liver disease (NAFLD) is a major chronic liver disease in children in the United States and is associated with insulin resistance. In adults, NAFLD is also associated with type 2 diabetes. To our knowledge, the prevalence of type 2 diabetes in children with NAFLD is unknown. Objective: To determine the prevalence of type 2 diabetes and prediabetes in children with NAFLD and assess type 2 diabetes and prediabetes as risk factors for nonalcoholic steatohepatitis (NASH)...
October 3, 2016: JAMA Pediatrics
Anne F Schott, William E Barlow, Catherine H Van Poznak, Daniel F Hayes, Carol M Moinpour, Danika L Lew, Philip A Dy, Evan T Keller, Jill M Keller, Gabriel N Hortobagyi
Bone metastases from breast cancer are common, causing significant morbidity. Preclinical data of dasatinib, an oral small molecule inhibitor of multiple oncogenic tyrosine kinases, suggested efficacy in tumor control and palliation of bone metastases in metastatic breast cancer (MBC). This clinical trial aimed to determine whether treatment with either of 2 dose schedules of dasatinib results in a progression-free survival (PFS) >50 % at 24 weeks in bone metastasis predominant MBC, to evaluate the toxicity of the 2 dosing regimens, and explore whether treatment results in decreased serum bone turnover markers and patient-reported "worst pain...
August 2016: Breast Cancer Research and Treatment
Nadine A Kasparian, Shab Mireskandari, Phyllis N Butow, Mbathio Dieng, Anne E Cust, Bettina Meiser, Kristine Barlow-Stewart, Scott Menzies, Graham J Mann
PURPOSE: People with melanoma often report pervasive fears about cancer recurrence, unmet information needs, and difficulties accessing psychological care. Interventions addressing the supportive care needs of people with melanoma are rare, and needs are often overlooked. The study evaluated a newly developed, evidence-based, psycho-educational resource for people with melanoma. METHODS: The evaluation study comprised three groups: adults at high risk of new primary disease due to multiple previous melanomas or one melanoma and dysplastic nevus syndrome (DNS), adults at moderate risk due to one previous melanoma and no DNS, and health professionals involved in melanoma care...
July 27, 2016: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
Srivathsan Ravindran, Sarah Helen Hancox, Neil Barlow, Arthur Dunk, David Howlett
Aims. To identify the prevalence of colonic and extraenteric incidental findings in magnetic resonance enterography (MRE) and their clinical significance. Methods. We retrospectively analysed 470 MRE studies carried out between March 2012 and 2014. Incidental findings were defined as those not expected from or made apparent on the referral. MRE reports were reviewed for colonic and extraenteric findings, subcategorised into "clinically significant" and "insignificant." Follow-up was identified from the electronic patient record...
2016: Canadian Journal of Gastroenterology & Hepatology
T Lieu, E Savage, P Zhao, L Edgington-Mitchell, N Barlow, R Bron, D P Poole, P McLean, R-J Lohman, D P Fairlie, N W Bunnett
BACKGROUND AND PURPOSE: Diverse proteases cleave protease-activated receptor-2 (PAR2) on primary sensory neurons and epithelial cells to evoke pain and inflammation. Trypsin and tryptase activate PAR2 by a canonical mechanism that entails cleavage within the extracellular N-terminus revealing a tethered ligand that activates the cleaved receptor. Cathepsin-S and elastase are biased agonists that cleave PAR2 at different sites to activate distinct signalling pathways. Although PAR2 is a therapeutic target for inflammatory and painful diseases, the divergent mechanisms of proteolytic activation complicate the development of therapeutically useful antagonists...
September 2016: British Journal of Pharmacology
Z A Nahleh, W E Barlow, D F Hayes, A F Schott, J R Gralow, W M Sikov, E A Perez, S Chennuru, H R Mirshahidi, S W Corso, D L Lew, L Pusztai, R B Livingston, G N Hortobagyi
SWOG S0800, a randomized open-label Phase II clinical trial, compared the combination of weekly nab-paclitaxel and bevacizumab followed by dose-dense doxorubicin and cyclophosphamide (AC) with nab-paclitaxel followed or preceded by AC as neoadjuvant treatment for HER2-negative locally advanced breast cancer (LABC) or inflammatory breast cancer (IBC). Patients were randomly allocated (2:1:1) to three neoadjuvant chemotherapy arms: (1) nab-paclitaxel with concurrent bevacizumab followed by AC; (2) nab-paclitaxel followed by AC; or (3) AC followed by nab-paclitaxel...
August 2016: Breast Cancer Research and Treatment
Martin G Thomas, Davide Sartini, Monica Emanuelli, Matthijs J van Haren, Nathaniel I Martin, David M Mountford, David J Barlow, Fábio Klamt, David B Ramsden, Madeehah Reza, Richard B Parsons
Nicotinamide N-methyltransferase (NNMT) is responsible for the N-methylation of nicotinamide to 1-methylnicotinamide. Our recent studies have demonstrated that NNMT regulates cellular processes fundamental to the correct functioning and survival of the cell. It has been proposed that NNMT may possess β-carboline (BC) N-methyltransferase activity, endogenously and exogenously produced pyridine-containing compounds which, when N-methylated, are potent inhibitors of Complex I and have been proposed to have a role in the pathogenesis of Parkinson's disease...
October 1, 2016: Biochemical Journal
Kirsty M Hooper, Peter G Barlow, Craig Stevens, Paul Henderson
Inflammatory bowel disease [IBD] is characterized by chronic inflammation of the gastrointestinal tract. Medications such as corticosteroids, thiopurines, immunomodulators and biologic agents are used to induce and maintain remission; however, response to these drugs is variable and can diminish over time. Defective autophagy has been strongly linked to IBD pathogenesis, with evidence showing that enhancing autophagy may be therapeutically beneficial by regulating inflammation and clearing intestinal pathogens...
July 5, 2016: Journal of Crohn's & Colitis
Nina B Radford, Laura F DeFina, Carolyn E Barlow, Susan G Lakoski, David Leonard, Andre R M Paixao, Amit Khera, Benjamin D Levine
OBJECTIVES: The authors sought to determine the relative contributions of baseline coronary artery calcification (CAC), follow-up CAC, and CAC progression on incident cardiovascular disease (CVD). BACKGROUND: Repeat CAC scanning has been proposed as a method to track progression of total atherosclerotic burden. However, whether CAC progression is a useful predictor of future CVD events remains unclear. METHODS: This was a prospective observational study of 5,933 participants free of CVD who underwent 2 examinations, including CAC scores, and subsequent CVD event assessment...
June 23, 2016: JACC. Cardiovascular Imaging
Paul C Kirchberger, Fabini D Orata, E Jed Barlow, Kathryn M Kauffman, Rebecca J Case, Martin F Polz, Yan Boucher
UNLABELLED: Vibrio cholerae is a ubiquitous aquatic microbe in temperate and tropical coastal areas. It is a diverse species, with many isolates that are harmless to humans, while others are highly pathogenic. Most notable among them are strains belonging to the pandemic O1/O139 serogroup lineage, which contains the causative agents of cholera. The environmental selective regimes that led to this diversity are key to understanding how pathogens evolve in environmental reservoirs. A local population of V...
September 15, 2016: Applied and Environmental Microbiology
Dawn L Hershman, Cathee Till, Jason D Wright, Danielle Awad, Scott D Ramsey, William E Barlow, Lori M Minasian, Joseph Unger
BACKGROUND: Neuropathy is a debilitating toxicity associated with various chemotherapy agents. We evaluated the association between common comorbid conditions and the development of peripheral neuropathy in patients treated with taxane-based chemotherapy. METHODS: We examined the Southwest Oncology Group database to identify phase II and III trials that included taxane therapy from 1999 to 2011. We linked the Southwest Oncology Group clinical records to Medicare claims data according to Social Security number, sex, and date of birth...
September 1, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Fern Findlay, Lorna Proudfoot, Craig Stevens, Peter G Barlow
Cationic Host Defense Peptides (HDP, also known as antimicrobial peptides) are crucial components of the innate immune system and possess broad-spectrum antibacterial, antiviral, and immunomodulatory activities. They can contribute to the rapid clearance of biological agents through direct killing of the organisms, inhibition of pro-inflammatory mediators such as lipopolysaccharide, and by modulating the inflammatory response to infection. Category A biological agents and materials, as classified by the United States National Institutes for Health, the US Centers for Disease Control and Prevention, and the US Department of Homeland Security, carry the most severe threat in terms of human health, transmissibility, and preparedness...
June 2016: Pathogens and Global Health
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