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https://www.readbyqxmd.com/read/27906803/naspghan-clinical-practice-guideline-for-the-diagnosis-and-treatment-of-nonalcoholic-fatty-liver-disease-in-children
#1
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 (ECON) reviewed and summarized the available literature, formulating recommendations to guide screening and clinical care of children with NAFLD...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27843572/utility-of-high-resolution-anorectal-manometry-and-wireless-motility-capsule-in-the-evaluation-of-patients-with-parkinson-s-disease-and-chronic-constipation
#2
Andrew Su, Rita Gandhy, Carrolee Barlow, George Triadafilopoulos
BACKGROUND: The aetiology of constipation in Parkinson's disease remains poorly understood. Defaecatory dyssynergia, anal sphincter spasticity and slow transit constipation may, individually or collectively, play a role. AIMS: In this retrospective cohort analysis of patients with Parkinson's disease and chronic constipation, we determined the utility of high-resolution anorectal manometry, balloon expulsion and wireless motility capsule testing in defining the underlying aetiology for constipation...
2016: BMJ Open Gastroenterology
https://www.readbyqxmd.com/read/27834756/oral-pharmacological-chaperone-migalastat-compared-with-enzyme-replacement-therapy-in-fabry-disease-18-month-results-from-the-randomised-phase-iii-attract-study
#3
Derralynn A Hughes, Kathleen Nicholls, Suma P Shankar, Gere Sunder-Plassmann, David Koeller, Khan Nedd, Gerard Vockley, Takashi Hamazaki, Robin Lachmann, Toya Ohashi, Iacopo Olivotto, Norio Sakai, Patrick Deegan, David Dimmock, François Eyskens, Dominique P Germain, Ozlem Goker-Alpan, Eric Hachulla, Ana Jovanovic, Charles M Lourenco, Ichiei Narita, Mark Thomas, William R Wilcox, Daniel G Bichet, Raphael Schiffmann, Elizabeth Ludington, Christopher Viereck, John Kirk, Julie Yu, Franklin Johnson, Pol Boudes, Elfrida R Benjamin, David J Lockhart, Carrolee Barlow, Nina Skuban, Jeffrey P Castelli, Jay Barth, Ulla Feldt-Rasmussen
BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder caused by GLA mutations, resulting in α-galactosidase (α-Gal) deficiency and accumulation of lysosomal substrates. Migalastat, an oral pharmacological chaperone being developed as an alternative to intravenous enzyme replacement therapy (ERT), stabilises specific mutant (amenable) forms of α-Gal to facilitate normal lysosomal trafficking. METHODS: The main objective of the 18-month, randomised, active-controlled ATTRACT study was to assess the effects of migalastat on renal function in patients with Fabry disease previously treated with ERT...
November 10, 2016: Journal of Medical Genetics
https://www.readbyqxmd.com/read/27791090/parasite-stress-and-pathogen-avoidance-relate-to-distinct-dimensions-of-political-ideology-across-30-nations
#4
Joshua M Tybur, Yoel Inbar, Lene Aarøe, Pat Barclay, Fiona Kate Barlow, Mícheál de Barra, D Vaughn Becker, Leah Borovoi, Incheol Choi, Jong An Choi, Nathan S Consedine, Alan Conway, Jane Rebecca Conway, Paul Conway, Vera Cubela Adoric, Dilara Ekin Demirci, Ana María Fernández, Diogo Conque Seco Ferreira, Keiko Ishii, Ivana Jakšić, Tingting Ji, Florian van Leeuwen, David M G Lewis, Norman P Li, Jason C McIntyre, Sumitava Mukherjee, Justin H Park, Boguslaw Pawlowski, Michael Bang Petersen, David Pizarro, Gerasimos Prodromitis, Pavol Prokop, Markus J Rantala, Lisa M Reynolds, Bonifacio Sandin, Bariş Sevi, Delphine De Smet, Narayanan Srinivasan, Shruti Tewari, Cameron Wilson, Jose C Yong, Iris Žeželj
People who are more avoidant of pathogens are more politically conservative, as are nations with greater parasite stress. In the current research, we test two prominent hypotheses that have been proposed as explanations for these relationships. The first, which is an intragroup account, holds that these relationships between pathogens and politics are based on motivations to adhere to local norms, which are sometimes shaped by cultural evolution to have pathogen-neutralizing properties. The second, which is an intergroup account, holds that these same relationships are based on motivations to avoid contact with outgroups, who might pose greater infectious disease threats than ingroup members...
October 17, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27756783/plasma-lipidomic-profiles-improve-upon-traditional-risk-factors-for-the-prediction-of-cardiovascular-events-in-type-2-diabetes
#5
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
https://www.readbyqxmd.com/read/27624512/lysosomal-acid-lipase-deficiency-unmasked-in-two-children-with-nonalcoholic-fatty-liver-disease
#6
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
https://www.readbyqxmd.com/read/27569726/in-children-with-nonalcoholic-fatty-liver-disease-cysteamine-bitartrate-delayed-release-improves-liver-enzymes-but-does-not-reduce-disease-activity-scores
#7
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 of 4 or higher at 10 centers. From June 2012 to January 2014, the patients were assigned randomly to receive CBDR or placebo twice daily (300 mg for patients weighing ≤65 kg, 375 mg for patients weighing >65 to 80 kg, and 450 mg for patients weighing >80 kg) for 52 weeks...
December 2016: Gastroenterology
https://www.readbyqxmd.com/read/27528216/three-dimensional-dynamic-morphology-of-the-mitral-valve-in-different-forms-of-mitral-valve-prolapse-potential-implications-for-annuloplasty-ring-selection
#8
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
https://www.readbyqxmd.com/read/27514369/multiple-systemic-embolism-in-infective-endocarditis-underlying-in-barlow-s-disease
#9
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
https://www.readbyqxmd.com/read/27509102/treatment-of-fabry-s-disease-with-the-pharmacologic-chaperone-migalastat
#10
RANDOMIZED CONTROLLED TRIAL
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
https://www.readbyqxmd.com/read/27502755/-atypical-scurvy-associated-with-anorexia-nervosa
#11
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
https://www.readbyqxmd.com/read/27479770/prolonged-detection-of-zika-virus-rna-in-pregnant-women
#12
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
https://www.readbyqxmd.com/read/27478956/prevalence-of-prediabetes-and-type-2-diabetes-in-children-with-nonalcoholic-fatty-liver-disease
#13
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
https://www.readbyqxmd.com/read/27475087/phase-ii-studies-of-two-different-schedules-of-dasatinib-in-bone-metastasis-predominant-metastatic-breast-cancer-swog-s0622
#14
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
https://www.readbyqxmd.com/read/27465047/-melanoma-questions-and-answers-development-and-evaluation-of-a-psycho-educational-resource-for-people-with-a-history-of-melanoma
#15
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
https://www.readbyqxmd.com/read/27446837/unexpected-findings-in-magnetic-resonance-enterography-and-their-clinical-significance
#16
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
https://www.readbyqxmd.com/read/27423137/antagonism-of-the-proinflammatory-and-pronociceptive-actions-of-canonical-and-biased-agonists-of-protease-activated-receptor-2
#17
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
https://www.readbyqxmd.com/read/27393622/swog-s0800-nci-cdr0000636131-addition-of-bevacizumab-to-neoadjuvant-nab-paclitaxel-with-dose-dense-doxorubicin-and-cyclophosphamide-improves-pathologic-complete-response-pcr-rates-in-inflammatory-or-locally-advanced-breast-cancer
#18
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
https://www.readbyqxmd.com/read/27389312/nicotinamide-n-methyltransferase-catalyses-the-n-methylation-of-the-endogenous-%C3%AE-carboline-norharman-evidence-for-a-novel-detoxification-pathway
#19
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
https://www.readbyqxmd.com/read/27381462/inflammatory-bowel-disease-drugs-a-focus-on-autophagy
#20
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
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