tbuckley/cabot-search huggingface dataset:
Rapid and reproducible characterization of sickling during automated deoxygenation in sickle cell disease patients
Authors: Minke A.E. Rab, Brigitte A. van Oirschot, Jennifer Bos, Tesy H. Merkx, Annet C.W. van Wesel, Osheiza Abdulmalik, Martin K. Safo, Birgitta A. Versluijs, Maite E. Houwing, Marjon H. Cnossen, Jurgen Riedl, Roger E.G. Schutgens, Gerard Pasterkamp, Marije Bartels, Eduard J. van Beers, Richard van Wijk
Journal: American Journal of Hematology (2019)

In sickle cell disease (SCD), sickle hemoglobin (HbS) polymerizes upon deoxygenation, resulting in sickling of red blood cells (RBCs). These sickled RBCs have strongly reduced deformability, leading to vaso-occlusive crises and chronic hemolytic anemia. To date, there are no reliable laboratory parameters or assays capable of predicting disease severity or monitoring treatment effects. We here report on the oxygenscan, a newly developed method to measure RBC deformability (expressed as Elongation Index - EI) as a function of pO2 . Upon a standardized, 22 minute, automated cycle of deoxygenation (pO2 median 16 mmHg ± 0.17) and reoxygenation, a number of clinically relevant parameters are produced in a highly reproducible manner (coefficients of variation <5%). In particular, physiological modulators of oxygen affinity, such as, pH and 2,3-diphosphoglycerate showed a significant correlation (respectively R = -0.993 and R = 0.980) with Point of Sickling (PoS5% ), which is defined as the pO2 where a 5% decrease in EI is observed during deoxygenation. Furthermore, in vitro treatment with antisickling agents, including GBT440, which alter the oxygen affinity of hemoglobin, caused a reproducible left-shift of the PoS, indicating improved deformability at lower oxygen tensions. When RBCs from 21 SCD patients were analyzed, we observed a significantly higher PoS in untreated homozygous SCD patients compared to treated patients and other genotypes. We conclude that the oxygenscan is a state-of-the-art technique that allows for rapid analysis of sickling behavior in SCD patients. The method is promising for personalized treatment, development of new treatment strategies and could have potential in prediction of complications.
SP0116 How to Select the Most Appropriate Capillaroscopic Device: PROS and Cons
Authors: W. Hermann
Journal: Annals of the Rheumatic Diseases (2014)

<h3></h3> One of the most important indications for performing a capillaroscopy is the differentiation between primary and secondary Raynaud9s Syndrome. It is possible to examine with different kind of microscopes, which differ in different points like picture quality or price. Before buying a microscope there should be thought about different aspects of the examination: – The region of interest. The capillaries have a diameter of about 8 μm. For a assessment a magnificiation of 100-200x is needed, for the overview the magnification should be about 50x. – Measurement. Beside of quantitative statements for example about the architecture of the vessels there should be a possibility of performing measurements like the number of capillaries/mm or the diameter of the vessels. – Documentation. All parts of the examination have to be stored and assigned to the patient and the case. – Practical aspects. The more often the examanitaion is performed, the more important will become practical aspects. Three different kind of microscopes are: – Stereo microscope.Advantages: Very good image quality, zooming in without problems, relatively easy to use.Disadvantages: no mobile device, in patients with contractures difficult to perform examinations, relatively high costs – Videocapillaroscopes:Advantages: Very good image quality, easy to use, "gold standard" for capillaroscopy.Disadvantages: No overview, no direct zooming in possible, relatively high costs. – USB microscopes:Advantages: low costs, zooming in without problems, easy to use.Disadvantages: limited picture quality, documentation laborious The selection of a capillaroscopic device depends on different preconditions which should be cleared up before buying the device. The price range differs between 100 and about 10000€. <h3>Literature</h3> Cutolo M et al.: Raynaud9s phenomenon and the role of capillaroscopy. Arthritis Rheum 2003; 48: 3023-3030 Sander O et al.: Kapillarmikroskopie: Durchführung und Nomenklatur. Z Rheumatol 2010; 69: 253-262 Hermann W, Meier FMP, Müller-Ladner U: Reichen weniger als 100 Euro für ein Kapillarmikroskop? Z Rheumatol 2013.72:520-522 <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.6131
Type 2 Diabetes Mellitus in Patients with Sickle Cell Disease: A Population-Based Longitudinal Analysis of Three Cohorts
Authors: Jifang Zhou, Jin Han, Edith A. Nutescu, William Galanter, Surrey M. Walton, Victor R. Gordeuk, Santosh L. Saraf, Andrew Srisuwananukorn, Gregory Sampang Calip
Journal: Blood (2018)
GW25-e0061 Up-regulation of neuronal nitric oxide synthase modulates myofilament Ca 2+ sensitivity and controls left ventricular myocyte contractility in hypertensive rats
Authors: Jin Chun Zi, Cui Lan, Xu Wen Hu, Zhang Yin Hua, Zhao Guang Xian
Journal: Journal of the American College of Cardiology (2014)
A 2018 Horizon Scan of Emerging Issues for Global Conservation and Biological Diversity
Authors: William J. Sutherland, Stuart H.M. Butchart, Ben Connor, Caroline Culshaw, Lynn V. Dicks, Jason Dinsdale, Helen Doran, Abigail C. Entwistle, Erica Fleishman, David W. Gibbons, Zhigang Jiang, Brandon Keim, Xavier Le Roux, Fiona A. Lickorish, Paul Markillie, Kathryn A. Monk, Diana Mortimer, James W. Pearce-Higgins, Lloyd S. Peck, Jules Pretty, Colleen L. Seymour, Mark D. Spalding, Femke H. Tonneijck, Rosalind A. Gleave
Journal: Trends in Ecology & Evolution (2017)

This is the ninth such annual horizon scan. Twenty-four experts in conservation research and practice, ecology, economics, policy, and science communication identified 15 topics following a wide consultation. They followed a Delphi like process to score and identify the most important. The issues highlighted span a wide range of fields and include thiamine deficiency in wild animals, the geographic expansion of chronic wasting disease, genetic control of invasive mammal populations and the effect of culturomics on conservation science, policy and action. This is our ninth annual horizon scan to identify emerging issues that we believe could affect global biological diversity, natural capital and ecosystem services, and conservation efforts. Our diverse and international team, with expertise in horizon scanning, science communication, as well as conservation science, practice, and policy, reviewed 117 potential issues. We identified the 15 that may have the greatest positive or negative effects but are not yet well recognised by the global conservation community. Themes among these topics include new mechanisms driving the emergence and geographic expansion of diseases, innovative biotechnologies, reassessments of global change, and the development of strategic infrastructure to facilitate global economic priorities. This is our ninth annual horizon scan to identify emerging issues that we believe could affect global biological diversity, natural capital and ecosystem services, and conservation efforts. Our diverse and international team, with expertise in horizon scanning, science communication, as well as conservation science, practice, and policy, reviewed 117 potential issues. We identified the 15 that may have the greatest positive or negative effects but are not yet well recognised by the global conservation community. Themes among these topics include new mechanisms driving the emergence and geographic expansion of diseases, innovative biotechnologies, reassessments of global change, and the development of strategic infrastructure to facilitate global economic priorities.
Effects of Metformin Therapy on Coronary Endothelial Dysfunction in Patients With Prediabetes With Stable Angina and Nonobstructive Coronary Artery Stenosis: The CODYCE Multicenter Prospective Study
Authors: Celestino Sardu, Pasquale Paolisso, Cosimo Sacra, Ciro Mauro, Fabio Minicucci, Michele Portoghese, Maria Rosaria Rizzo, Michelangela Barbieri, Ferdinando Carlo Sasso, Nunzia D’Onofrio, Maria Luisa Balestrieri, Paolo Calabrò, Giuseppe Paolisso, Raffaele Marfella
Journal: Diabetes Care (2019)

OBJECTIVE To evaluate the effect of metformin therapy on coronary endothelial function and major adverse cardiac events (MACE) in patients with prediabetes with stable angina and nonobstructive coronary stenosis (NOCS). RESEARCH DESIGN AND METHODS Metformin therapy may be needed to reduce coronary heart disease risk in patients with prediabetes. A total of 258 propensity score–matched (PSM) patients with stable angina undergoing coronary angiography were enrolled in the study. Data from 86 PSM subjects with normoglycemia (NG), 86 PSM subjects with prediabetes (pre-DM), and 86 PSM subjects with prediabetes treated with metformin (pre-DM metformin) were analyzed. During coronary angiography, NOCS was categorized by luminal stenosis &amp;lt;40% and fractional flow reserve &amp;gt;0.80. In addition, we assessed the endothelial function, measuring coronary artery diameter of left anterior descending coronary (LAD) at baseline and after the infusion of acetylcholine, by means of an intracoronary Doppler guide wire. MACE, as cardiac death, myocardial infarction, and heart failure, was evaluated at 24 months of follow-up. RESULTS At baseline, NG patients had a lower percentage of LAD endothelial dysfunction compared with pre-DM patients (P &amp;lt; 0.05). The pre-DM patients had a higher percentage of endothelial LAD dysfunction as compared with the pre-DM metformin patients (P &amp;lt; 0.05). At the 24th month of follow-up, MACE was higher in pre-DM versus NG (P &amp;lt; 0.05). In pre-DM metformin patients, MACE was lower compared with pre-DM patients (P &amp;lt; 0.05). CONCLUSIONS Metformin therapy may reduce the high risk of cardiovascular events in pre-DM patients by reducing coronary endothelial dysfunction.
Deriving Core Patient-Reported Outcomes in Patients with Myelodysplastic Syndromes — a Delphi Survey from the European-MDS Registry
Authors: Igor Stojkov, Annette Conrads-Frank, Ursula Rochau, Karin A Koinig, Helena Borba, Marjan Arvandi, Pierre Fenaux, Argiris Symeonidis, David Bowen, Moshe Mittelman, Corine van Marrewijk, Theo de Witte, Fabio Efficace, Uwe Siebert, Reinhard Stauder
Journal: Blood (2018)
P045 Effect of specialized 6-month physical-occupational intervention with subsequent 6-month follow-up period in patients with scleroderma – preliminary data
Authors: M Spiritovic, H Smucrova, S Oreska, H Storkanova, B Hermankova, P Cesak, A Rathouska, O Ruzickova, K Pavelka, L Senolt, J Vencovsky, R Becvar, M Tomcik
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Career situation of first and presenting author</h3> Student for a master or a PhD. <h3>Introduction</h3> Systemic sclerosis (SSc) is accompanied by stiffness and contraction of tissue, predominantly of the skin, caused by fibrosis and resulting in reduced quality of life. The aim of our study was to determine the effect of specialized physical-occupational intervention (POI) on hand/face function and strength, and quality of life of SSc patients. <h3>Objectives</h3> The study included a total of 55 patients who fulfilled the EULAR/ACR criteria for SSc (2013) and had skin involvement leading to hand-function impairment and mouth handicap. 25 patients were recruited into the intervention group (IG) and 30 patients into the control group (CG). Both groups received educational material for home exercise, but only the IG underwent a 6 month intervention with a subsequent 6 month follow-up period. <h3>Methods</h3> Patients were assessed by a physician and a physiotherapist blinded to intervention at months 0, 3, 6, and 12, and parameters evaluating hand/face and quality of life were recorded. Patients also filled out PRO (patient reported outcomes) questionnaires and provided blood for routine laboratory analysis and bio-banking. Data analysis was done between groups and within the group. <h3>Results</h3> Compared to the observed statistically significant deterioration in the CG, we found a statistically significant improvement in the IG in objectively assessed function and strength of hand, distance between incisors and lips and functional ability (SHAQ). Only numerical improvements in the IG during the intervention compared to numerical deterioration in CG that did not reach statistical significance were observed in subjectively assessed hand function and mouth handicap, functional ability (HAQ) and in some domains of QoL (SF-36) and fatigue (FIS–in cognitive function). During the follow-up period, there was a significant deterioration or stagnation of the achieved positive results in the IG. <h3>Conclusions</h3> Our specialized POI intervention led to a significant improvement in the observed parameters that was clinically significant in a substantial proportion of patients, and prevention of the expected worsening of hand/face function and strength and quality of life. <h3>Acknowledgements</h3> Supported by AZV-16-33574A, SVV for FTVS UK 2019-260466, MHCR 023728. <h3>Disclosure of Interest</h3> None declared
All Traumas Are Not Created Equal
Authors: Sarah Gebauer, Rachel Moore, Joanne Salas
Journal: JAMA Pediatrics (2019)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Pediatrics HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Pediatrics journal
lncRNA SNHG6 regulates EZH2 expression by sponging miR-26a/b and miR-214 in colorectal cancer
Authors: Mu Xu, Xiaoxiang Chen, Kang Lin, Kaixuan Zeng, Xiangxiang Liu, Xueni Xu, Bei Pan, Tao Xu, Li Sun, Bangshun He, Yuqin Pan, Huiling Sun, Shukui Wang
Journal: Journal of Hematology & Oncology (2019)

Abnormal expression of long non-coding RNAs (lncRNAs) has been found in almost all human tumors, providing numerous potential diagnostic biomarkers, prognostic biomarkers, and therapeutic targets.
CRISPR-Based Functional Genomics Studies Reveal Distinct and Overlapping Genes Mediating Resistance to Different Classes of Heterobifunctional Degraders of Oncoproteins: Implications for Novel Therapeutics across Diverse Neoplasias
Authors: Ryosuke Shirasaki, Sara Gandolfi, Ricardo De Matos Simoes, Geoffrey Matthews, Dennis Buckley, Olga Dashevsky, Sondra L. Downey-Kopyscinski, Huihui Tang, Michal Sheffer, Eugen Dhimolea, Brian Glassner, Megan Bariteau, Jeffrey Sorrell, Johanna B Bruggenthies, Quinlan Sievers, Haley Poarch, Nicholas P Kwiatkowski, Tinghu Zhang, Jacob Laubach, Robert L. Schlossman, Paul Richardson, Aedin Culhane, Richard WJ Groen, Eric S. Fischer, Francisca Vazquez, Aviad Tsherniak, William C. Hahn, Joan Levy, Daniel Auclair, Jonathan D. Licht, Jonathan J Keats, Lawrence H. Boise, Benjamin L. Ebert, James Bradner, Nathanae Gray, Constantine S. Mitsiades
Journal: Blood (2018)
Ethical Guidelines for DNA Testing in Migrant Family Reunification
Authors: Nita Farahany, Saheel Chodavadia, Sara H. Katsanis
Journal: The American Journal of Bioethics (2019)

In the summer of 2018, U.S. Customs and Border Protection Officers removed more than 2600 migrant children from their families at the United States–Mexico border (Gonzalez, Republic, and Gomez 2018...
Hyperpolarised<sup>129</sup>Xe magnetic resonance imaging to monitor treatment response in children with cystic fibrosis
Authors: Jonathan H. Rayment, Marcus J. Couch, Nancy McDonald, Nikhil Kanhere, David Manson, Giles Santyr, Felix Ratjen
Journal: European Respiratory Journal (2019)

Pulmonary magnetic resonance imaging using hyperpolarised 129Xe gas (XeMRI) can quantify ventilation inhomogeneity by measuring the percentage of unventilated lung volume (ventilation defect per cent (VDP)). While previous studies have demonstrated its sensitivity for detecting early cystic fibrosis (CF) lung disease, the utility of XeMRI to monitor response to therapy in CF is unknown. The aim of this study was to assess the ability of XeMRI to capture treatment response in paediatric CF patients undergoing inpatient antibiotic treatment for a pulmonary exacerbation.15 CF patients aged 8-18 years underwent XeMRI, spirometry, plethysmography and multiple-breath nitrogen washout at the beginning and end of inpatient treatment of a pulmonary exacerbation. VDP was calculated from XeMRI images obtained during a static breath hold using semi-automated k-means clustering and linear binning approaches.XeMRI was well tolerated. VDP, lung clearance index and the forced expiratory volume in 1 s all improved with treatment; however, response was not uniform in individual patients. Of all outcome measures, VDP showed the largest relative improvement (-42.1%, 95% CI -52.1--31.9%, p<0.0001).These data support further investigation of XeMRI as a tool to capture treatment response in CF lung disease.
Dose-response associations of cardiorespiratory fitness with all-cause mortality and incidence and mortality of cancer and cardiovascular and respiratory diseases: the UK Biobank cohort study
Authors: Lewis Steell, Frederick K Ho, Anne Sillars, Fanny Petermann-Rocha, Hiu Li, Donald M Lyall, Stamatina Iliodromiti, Paul Welsh, Jana Anderson, Daniel F MacKay, Jill P Pell, Naveed Sattar, Jason MR Gill, Stuart Robert Gray, Carlos A Celis-Morales
Journal: British Journal of Sports Medicine (2019)

Objective To investigate the association of cardiorespiratory fitness with all-cause mortality, and cardiovascular disease (CVD), respiratory disease, chronic obstructive pulmonary disease (COPD) and cancer mortality and incidence. Design Prospective population-based study. Setting UK Biobank. Participants Of the 5 02 628 (5.5% response rate) participants recruited by UK Biobank, we included 73 259 (14.6%) participants with available data in this analysis. Of these, 1374 participants died and 4210 developed circulatory diseases, 1293 respiratory diseases and 4281 cancer, over a median of 5.0 years (IQR 4.3–5.7) follow-up. Main outcome measures All-cause mortality and circulatory disease, respiratory disease, COPD and cancer (such as colorectal, lung, breast and prostate) mortality/incidence. Fitness was estimated using a submaximal cycle ergometer test. Results The HR for all-cause mortality for each metabolic equivalent of task (MET) higher fitness was 0.96 (95% CI 0.93 to 0.98). Similar results were observed for incident circulatory disease (HR 0.96 [0.95 to 0.97]), respiratory disease (HR 0.96 [0.94 to 0.98]), COPD (HR 0.90 [0.86 to 0.95) and colorectal cancer (HR 0.96 [0.92 to 1.00]). Nonlinear analysis revealed that a high level of fitness (&gt;10METs) was associated with a greater incidence of atrial fibrillation (HR 1.24 [1.07 to 1.44]) and prostate cancer (HR 1.16 [1.02 to 1.32]) compared with average fitness. All results were adjusted for sociodemographic, lifestyle and dietary factors, body composition, and morbidity at baseline and excluded events in the first 2 years of follow-up. Conclusions Higher cardiorespiratory fitness was associated with lower risk of premature mortality and incidence of CVD, respiratory disease and colorectal cancer.
Depletion of Vascular Nitric Oxide Contributes to Poor Outcomes after Cardiac Arrest
Authors: Kei Hayashida, Yusuke Miyazaki, Binglan Yu, Michael G. Silverman, Riccardo Pinciroli, Lorenzo Berra, Rajeev Malhotra, Michael W. Donnino, Fumito Ichinose
Journal: American Journal of Respiratory and Critical Care Medicine (2019)
Preserved Ratio Impaired Spirometry and Interstitial Lung Abnormalities in Smokers
Authors: Almerico Marruchella
Journal: American Journal of Respiratory and Critical Care Medicine (2019)

I read with great interest the paper by Wan and coworkers evaluating the preserved ratio impaired spirometry (PRISm) functional pattern (FEV 1 /FVC > 0.7 and FEV 1 .80%) in their longitudinal study of a large cohort of current or ex-smokers (1).The prevalence of this heterogeneous condition is remarkable (12.4% and 12.5% at baseline and follow-up, respectively) and its association with increased mortality, comparable to that observed in Global Initiative for Chronic Obstructive Lung Disease (GOLD) 2 subjects, deserves special consideration.Individuals with PRISm are characterized by a significantly lower TLC% predicted and percent emphysema, as measured on quantitative computed tomography scans, in comparison with GOLD 0 and GOLD 1-4 groups.In the study cohort, the PRISm status was not stable at 5-year follow-up; in particular, the authors report a transition to GOLD 0 in 22% of the subjects with PRISm and a transition to PRISm in about one-third of baseline GOLD 0 individuals.The latter group was characterized by a lesser amount of emphysema and air trapping on computed tomography scans, an increase in body mass index, and decreased TLC% predicted and percent emphysema at baseline.Moreover, this group exhibited the largest functional decline (either FEV 1 or FVC) in comparison with all other groups, suggesting a restrictive physiologic impairment.Interstitial lung abnormalities have been found in a substantial minority of a cohort of smokers enrolled in the COPDGene (Genetic Epidemiology of Chronic Obstructive Pulmonary Disease) study (1 out of 12) and were associated with a reduced TLC and a lesser amount of emphysema.Smokers with interstitial lung abnormalities were more likely to have an "unclassified" spirometric pattern, analogous to PRISm (2).Under this definition are grouped various morphological pictures that may represent static imaging findings or the early stage of a progressive fibrosing disease (3).The evidence of many similarities between smokers identified as carriers of interstitial lung abnormalities and those functionally classified as having PRISm makes me think that at least a small subset of baseline GOLD 0 subjects who transitioned to the PRISm group could have developed a smoke-related interstitial disorder.