tbuckley/cabot-search huggingface dataset:
MicroRNA Is Prognostic Indicator in Patients with Diffuse Large B Cell Lymphoma
Authors: Meir Lahav, Ron Ram, Michal Kushnir, Rosetta Genomics Israel, Ofer Spilberg, Gila Lithwick Yanai, Osnat Beeri, Moshe Hoshen, Maora Feinmesser
Journal: Blood (2008)
Tobacco 21–An Important Public Policy to Protect our Youth
Authors: Harold J Farber, Enid Neptune, Smita Pakhale
Journal: American Journal of Respiratory and Critical Care Medicine (2016)

Why is it important to protect youth from tobacco?Tobacco causes disease and death.It is very easy for teens to get addicted.Ninety percent of adults who use tobacco started as in their teen years.Ninetynine percent started to use tobacco before turning 26 years old.
A novel p.Ser282Pro<i>CPA1</i>variant is associated with autosomal dominant hereditary pancreatitis
Authors: Aleksandra A Kujko, Dorottya M. Berki, Grzegorz Oracz, Karolina Wejnarska, Justyna Antoniuk, Katarzyna Wertheim-Tysarowska, Elwira Kołodziejczyk, Jerzy Bal, Miklós Sahin-Tóth, Agnieszka M Rygiel
Journal: Gut (2017)

We read with great interest the recent publication by Nemeth et al .1 in which the authors demonstrate that a misfolding human cationic trypsinogen (serine protease 1, PRSS1) variant causes autosomal dominant hereditary chronic pancreatitis (CP) by inducing endoplasmic reticulum (ER) stress. Previously, the same mechanism was proposed for carboxypeptidase A1 ( CPA1 ) gene variants strongly associated with early onset sporadic CP;2 however, the association of misfolding CPA1 variants with familial or hereditary CP has not been demonstrated so far.

Here, we report two Polish families with hereditary CP carrying a novel heterozygous c.844T>C (p.Ser282Pro) CPA1 variant. In Family 1, the index patient, her mother and uncle (half-brother of the mother) developed CP (figure 1). The age of diagnosis was 17 years for the index patient and 51 and 31 years (with earlier abdominal pain) for the mother and the uncle, respectively. In Family 2, three members were affected by CP (figure 1): the index patient, her mother and her father with ages of onset of 12, 32 and 34 years, respectively. In both families, the diagnosis of CP was confirmed by …
A Retrospective Single-Centre Cohort Study Comparing Lower-Dose Intravenous Immune Globulin (IVIg) (1 g/kg) to Higher-Dose Ivig (2 g/kg) in Adult Immune Thrombocytopenia
Authors: Mikhyla J Richards, Sophie Yu Wang, Ribeka Kim, Kate M Chipperfield, David Pi, Luke Y. C. Chen
Journal: Blood (2012)
Authors’ reply to Doherty and Hawkins
Authors: Øyvind Holme, Magnus Løberg, Michael Bretthauer, Mette Kalager
Journal: BMJ (2017)

We thank Doherty and Hawkins for their responses to our article.123 Our paper is a meta-analysis, so we adhered to the PRISMA guidelines.4 The CONSORT guidelines, as mentioned by Doherty, are for individual randomised trials and do not apply to our paper.

The …
Long‐term future risk of severe exacerbations: Distinct 5‐year trajectories of problematic asthma
Authors: A. C. A. Yii, J. H. Y. Tan, T. S. Lapperre, A. K. W. Chan, S. Y. Low, T. H. Ong, K. L. Tan, S. H. Chotirmall, P. J. Sterk, M. S. Koh
Journal: Allergy (2017)

Abstract Background Assessing future risk of exacerbations is an important component of asthma management. Existing studies have investigated short‐ but not long‐term risk. Problematic asthma patients with unfavorable long‐term disease trajectory and persistently frequent severe exacerbations need to be identified early to guide treatment. Aim To identify distinct trajectories of severe exacerbation rates among “problematic asthma” patients and develop a risk score to predict the most unfavorable trajectory. Methods Severe exacerbation rates over five years for 177 “problematic asthma” patients presenting to a specialist asthma clinic were tracked. Distinct trajectories of severe exacerbation rates were identified using group‐based trajectory modeling. Baseline predictors of trajectory were identified and used to develop a clinical risk score for predicting the most unfavorable trajectory. Results Three distinct trajectories were found: 58.5% had rare intermittent severe exacerbations (“infrequent”), 32.0% had frequent severe exacerbations at baseline but improved subsequently (“nonpersistently frequent”), and 9.5% exhibited persistently frequent severe exacerbations, with the highest incidence of near‐fatal asthma (“persistently frequent”). A clinical risk score composed of ≥2 severe exacerbations in the past year (+2 points), history of near‐fatal asthma (+1 point), body mass index ≥25kg/m 2 (+1 point), obstructive sleep apnea (+1 point), gastroesophageal reflux (+1 point), and depression (+1 point) was predictive of the “persistently frequent” trajectory (area under the receiver operating characteristic curve: 0.84, sensitivity 72.2%, specificity 81.1% using cutoff ≥3 points). The trajectories and clinical risk score had excellent performance in an independent validation cohort. Conclusions Patients with problematic asthma follow distinct illness trajectories over a period of five years. We have derived and validated a clinical risk score that accurately identifies patients who will have persistently frequent severe exacerbations in the future.
Early Results of a Phase Ib/II Dose-Escalation Trial of Romidepsin in Association with CHOP in Patients with Peripheral T-Cell Lymphomas (PTCL)
Authors: Jehan Dupuis, Rene-Olivier Casasnovas, Franck Morschhauser, Herve Ghesquieres, Catherine Thieblemont, Vincent Ribrag, Herve Tilly, B. Coiffier
Journal: Blood (2011)
EZH2 Inhibitor GSK126 Exhibits Osteo-Anabolic Properties in MM Bone Disease and Synergizes with Bortezomib to Inhibit MM Cell Viability
Authors: Juraj Adamik, Rebecca Silbermann, Konstantinos Lontos, Peng Zhang, Quanhong Sun, Deborah Lynn Galson, G. David Roodman
Journal: Blood (2016)
Efficacy and Safety of a First Line Combined Therapeutic Approach for Young CLL Patients with Advanced or Progressive Disease Stratified According to the Biologic Features: First Analysis of the GIMEMA Multicenter Study LLC0405
Authors: Francesca Romana Mauro, Stefano Molica, Agostino Cortelezzi, Alfonso Piciocchi, Luca Laurenti, Maura Brugiatelli, Rita Rizzi, Rosanna Mirabelli, Francesco Zaia, Fabio Stagno, Stefania Ciolli, Francesco Forconi, Vincenzo Callea, Francesco Angrilli, Musolino Caterina, Francesco Buccisano, Massimo Massaia, Giovanni Del Poeta, Simona Santangelo, Marilisa Marinelli, Mauro Nanni, Maria Stefania De Propriis, Ilaria Del Giudice, Marco Vignetti, Fortunato Morabito, Giuseppe Torelli, Sergio Storti, Alessandro Levis, A. M. Michele Carella, Francesco Di Raimondo, Giuseppe Leone, Francesco Lauria, Giorgina Specchia, Antonio Cuneo, Giovanna Meloni, Anna Guarini, Robin Foá
Journal: Blood (2010)
Identification of Developmental and Behavioral Markers Associated With Genetic Abnormalities in Autism Spectrum Disorder
Authors: Somer L. Bishop, Cristan Farmer, Vanessa Bal, Elise B. Robinson, A. Jeremy Willsey, Donna M. Werling, Karoline Alexandra Havdahl, Stephan J. Sanders, Audrey Thurm
Journal: American Journal of Psychiatry (2017)

Aside from features associated with risk of neurogenetic syndromes in general (e.g., cognitive impairment), limited progress has been made in identifying phenotype-genotype relationships in autism spectrum disorder (ASD). The objective of this study was to extend work in the Simons Simplex Collection by comparing the phenotypic profiles of ASD probands with or without identified de novo loss of function mutations or copy number variants in high-confidence ASD-associated genes or loci.Analyses preemptively accounted for documented differences in sex and IQ in affected individuals with de novo mutations by matching probands with and without these genetic events on sex, IQ, and age before comparing them on multiple behavioral domains.Children with de novo mutations (N=112) had a greater likelihood of motor delay during early development (later age at walking), but they were less impaired on certain measures of ASD core symptoms (parent-rated social communication abnormalities and clinician-rated diagnostic certainty about ASD) in later childhood. These children also showed relative strengths in verbal and language abilities, including a smaller discrepancy between nonverbal and verbal IQ and a greater likelihood of having achieved fluent language (i.e., regular use of complex sentences).Children with ASD with de novo mutations may exhibit a "muted" symptom profile with respect to social communication and language deficits relative to those with ASD with no identified genetic abnormalities. Such findings suggest that examining early milestone differences and standardized testing results may be helpful in etiologic efforts, and potentially in clinical differentiation of various subtypes of ASD, but only if developmental and demographic variables are properly accounted for first.
Timing of the decline in physical activity in childhood and adolescence: Gateshead Millennium Cohort Study
Authors: Mohammed Abdulaziz Farooq, Kathryn N Parkinson, Ashley J Adamson, Mark S Pearce, Jessica K Reilly, Adrienne R Hughes, Xanne Janssen, Laura Basterfield, John J Reilly
Journal: British Journal of Sports Medicine (2017)

Background and aim There is a widely held and influential view that physical activity begins to decline at adolescence. This study aimed to identify the timing of changes in physical activity during childhood and adolescence. Methods Longitudinal cohort study (Gateshead Millennium Study) with 8 years of follow-up, from North-East England. Cohort members comprise a socioeconomically representative sample studied at ages 7, 9, 12 and 15 years; 545 individuals provided physical activity data at two or more time points. Habitual total volume of physical activity and moderate-to-vigorous intensity physical activity (MVPA) were quantified objectively using the Actigraph accelerometer over 5–7 days at the four time points. Linear mixed models identified the timing of changes in physical activity across the 8-year period, and trajectory analysis was used to identify subgroups with distinct patterns of age-related changes. Results Four trajectories of change in total volume of physical activity were identified representing 100% of all participants: all trajectories declined from age 7 years. There was no evidence that physical activity decline began at adolescence, or that adolescent declines in physical activity were substantially greater than the declines during childhood, or greater in girls than boys. One group (19% of boys) had relatively high MVPA which remained stable between ages 7 and15 years. Conclusions Future policy and research efforts to promote physical activity should begin well before adolescence, and should include both boys and girls.
Subject Index Vol. 79, 2010
Authors: Franz Caspar, P. Bech, Graeme J. Taylor, Felicity L. Bishop, Fiona Barlow, Jan Walker, Clare McDermott, George T. Lewith, Alexander Heinzel, Ralf Schäfer, Hans-Wilhelm Müller, Andre Schieffer, Ariane Ingenhag, Simon B. Eickhoff, Georg Northoff, Matthias Franz, Hubertus Hautzel, A. Lok, T.L.S. Visscher, M.W.J. Koeter, J. Assies, C.L.H. Bockting, W.M.M. Verschuren, A. Gill, A.H. Schene, Harald Baumeister, Gordon Parker, K.M.L. Huijbregts, H.W.J. van Marwijk, F.J. de Jong, B. Schreuders, A.T.F. Beekman, C.M. van der Feltz-Cornelis, William Y. Shi, Andrew G. Stewart, David L. Hare, Paola Rocca, Mario Aimetti, Michela Giugiario, Enrico Pigella, Federica Romano, Barbara Crivelli, Giuseppe Rocca, Filippo Bogetto, Jaap Lancee, Victor I. Spoormaker, Jan van den Bout, Marleen De Bolle, Jeffrey G. Johnson, Filip De Fruyt
Journal: Psychotherapy and Psychosomatics (2010)
Fellowtalk: Communication skills training (CST) curriculum for physicians in a hematology/oncology (HO) fellowship.
Authors: Fay J. Hlubocky, Anthony Back, James Alfred Wallace, Olwen Mary Hahn, Christopher Daugherty
Journal: Journal of Clinical Oncology (2014)

e17691 Background: CST is found to be effective in enhancing communication skills, knowledge, and professional fulfillment for HO fellows. Despite these data and ACGME requirements, the implementation of an evidence-based CST curriculum in a HO fellowship program has not been described. Methods: We designed Fellowtalk, an Oncotalk-adapted CST lecture series for HO fellows at the University of Chicago. Fellowtalk includes multiple training components: lectures-didactics, large group exercises, and one-on-one role play with communications faculty. Pre and post-CST was assessed utilizing a quantitative questionnaire with demographic information. Results: To date, from 2010-2013, a total of 34 fellows underwent Fellowtalk CST: median age 32y (29-46y); 50% male; 57% first year fellowship; 67% Ca; 29% Christian; 12% Jewish, 12% Hindu; median of 35 patients/week (12-56) in clinical practice. In comparison with pre-CST, analyses revealed fellows utilized twenty core communication skills in clinical discussions including: Breaking Bad News (3.5±.9 v 4.2 ±.5, p<.00); Conduct Family Conf (3.7±.8 v 4.1 ±.6 p<.00); Elicit Pt Emot (3.4 ± .9 v 4.2 ± .6 p<.00); Express Empathy (3.6±.7 v 4.1± .7, p=.02); Respond to Why Me? (3.3±.6 v 3.9 ±.7, p <.00); Pall Care (3.5±.9 v 4.3±.7, p= .03); Respond to Pt Denial (2.9 ±.6 v 3.6 ±.8, p=.03); D/c Pall Chemo (3.1±.8 v 4.0 ± .5,p<.00); Resp tx not indicated (3.3 ±.6 v 3.9 ±.5, p<.00); Disc Maintain Hope (3.3 ± .8 v 4.2 ± .7, p<.00); Code status (DNR)-Pt (4.0 ±.7 v 4.3 ± .5, p=.03); Code status (DNR)-Family(3.9 ± .7 v 4.2 ± .6, p= .03); Disc hospice (3.8 ±.9 v 4.3±.6, p= .01); Religiosity/Spirituality (3.2± .9 v 4.0 ± .8, p<.00); Pt Req Hasten Death (2.7±.9 v 3.3 ± 1.0, p=.01); Elicit Pt EOL Hopes (3.1±.7v3.9±.7, p <.00); Elicit Pt EOL Fears (3.1±.7v 4.1 ± .7, p <.00); Manage Pain (3.8±.7 v 4.1 ± .5, p=.03); Select Best Chemo (3.5±.9 v 4.0±.6 p= .04); Disc Clinical Trials (3.7±.9 v 4.1±.6 p= .03). Qualitative responses re CST revealed salient themes: value of the didactic; preparation to use skills; satisfaction; impact, and targeted communications skills for change. Conclusions: Fellowtalk represents a successful educational CST for improving communication skills for HO fellows.
Two Cycles of PAD Combination (PS-341/Bortezomib, Adriamycin and Dexamethasone) Followed by Autologous Hematopoietic Cell Transplantation (AHCT) in Newly Diagnosed Multiple Myeloma (MM) Patients.
Authors: Jung-Hee Lee, Je-Hwan Lee, Dae-Young Kim, Sung-nam Lim, Sung-Doo Kim, Young-Ah Kang, Miee Seol, Kyoo-Hyung Lee
Journal: Blood (2009)
Neutrophil Extracelluar Traps Contributes to the Hypercoagulable State and Liver Damage in Liver Cirrhosis
Authors: Xiaoming Wu, Lixiu Wang, Lu Zhao, Yan Zhang, Zhipeng Yao, Tao Li, Muhua Cao, Ruishuang Ma, Yayan Bi, Junjie Kou, Jin Zhou, Jialan Shi
Journal: Blood (2016)
Clinical Safety and Activity in a Phase 1 Trial of IPI-145, a Potent Inhibitor of Phosphoinositide-3-Kinase-δ,γ, in Patients with Advanced Hematologic Malignancies
Authors: Ian W. Flinn, Steven M. Horwitz, Manish Patel, Anas Younes, James R. Porter, Jennifer Sweeney, Kerstin Allen, Patrick Kelly, Brad S. Kahl
Journal: Blood (2012)
Loss of DDRGK1 modulates SOX9 ubiquitination in spondyloepimetaphyseal dysplasia
Authors: Adetutu T. Egunsola, Yangjin Bae, Ming-Ming Jiang, David S. Liu, Yuqing Chen-Evenson, Terry Bertin, Shan Chen, James T. Lu, Lisette Nevarez, Nurit Magal, Annick Raas-Rothschild, Eric C. Swindell, Daniel H. Cohn, Richard A. Gibbs, Philippe M. Campeau, Mordechai Shohat, Brendan H. Lee
Journal: Journal of Clinical Investigation (2017)

Shohat-type spondyloepimetaphyseal dysplasia (SEMD) is a skeletal dysplasia that affects cartilage development. Similar skeletal disorders, such as spondyloepiphyseal dysplasias, are linked to mutations in type II collagen (COL2A1), but the causative gene in SEMD is not known. Here, we have performed whole-exome sequencing to identify a recurrent homozygous c.408+1G>A donor splice site loss-of-function mutation in DDRGK domain containing 1 (DDRGK1) in 4 families affected by SEMD. In zebrafish, ddrgk1 deficiency disrupted craniofacial cartilage development and led to decreased levels of the chondrogenic master transcription factor sox9 and its downstream target, col2a1. Overexpression of sox9 rescued the zebrafish chondrogenic and craniofacial phenotype generated by ddrgk1 knockdown, thus identifying DDRGK1 as a regulator of SOX9. Consistent with these results, Ddrgk1-/- mice displayed delayed limb bud chondrogenic condensation, decreased SOX9 protein expression and Col2a1 transcript levels, and increased apoptosis. Furthermore, we determined that DDRGK1 can directly bind to SOX9 to inhibit its ubiquitination and proteasomal degradation. Taken together, these data indicate that loss of DDRGK1 decreases SOX9 expression and causes a human skeletal dysplasia, identifying a mechanism that regulates chondrogenesis via modulation of SOX9 ubiquitination.
Investigation of the Morphology of Recombinant Von Willebrand Factor by Two High-Resolution Microscopic Techniques
Authors: Hanspeter Rottensteiner, Birgit K Seyfried, Gernot Friedbacher, Guenter Allmaier, Nicola Ilk, Uwe B Sleytr, Hartmut J. Ehrlich, Peter L Turecek
Journal: Blood (2010)
The Biomarkers of Lupus Disease Study: A Bold Approach May Mitigate Interference of Background Immunosuppressants in Clinical Trials
Authors: Joan T. Merrill, Fred Immermann, Maryann Whitley, Tianhui Zhou, Andrew Hill, Margot O'Toole, Padmalatha Reddy, Marek Honczarenko, Aikaterini Thanou, Joe Rawdon, Joel M. Guthridge, Judith A. James, Sudhakar Sridharan
Journal: Arthritis & Rheumatology (2017)

Objective Molecular medicine raised expectations for strategically targeted biologic agents in systemic lupus erythematosus (SLE), but clinical trial results have been disappointing and difficult to interpret. Most studies add investigational agents to various, often effective, standard therapy immunosuppressants used at baseline, with unknown treatment interactions. Eliminating polypharmacy in trials of active lupus remains controversial. We undertook the Biomarkers of Lupus Disease study to test withdrawal of immunosuppressants as a novel approach to rendering SLE trials interpretable. Methods In 41 patients with active, non–organ‐threatening SLE flare (group A), temporary steroids were given while background immunosuppressants were withdrawn. Time to loss of disease suppression (time to disease flare) and safety were evaluated; standard therapy was immediately resumed when symptoms recurred. Immunologic impacts of standard therapy were studied at baseline by multiplex assay, enzyme‐linked immunosorbent assay, and messenger RNA array in group A patients plus 62 additional patients donating a single sample (group B). Results Patients with lower or higher baseline disease activity had median times to flare of 71 or 45 days, respectively; 40 of 41 patients (98%) had disease flares by 6 months. All flares were treated and resolved within 6 weeks. No serious adverse events occurred from flare or infection. Type I interferon (IFN), Th17, and B lymphocyte stimulator pathways tracked together. Baseline immunosuppressants had distinct impacts on Th17 and B lymphocyte stimulator, depending on IFN signature. Conclusion Trials in active, non–organ‐threatening SLE can safely withdraw background treatments if patients who have disease flares are designated nonresponders and returned to standard therapy. Immunologic effects of standard therapy vary between IFN‐defined subsets. These findings provide a strategy for minimizing or optimizing treatment combinations in lupus trials and clinical care.
Sun Safety Practices—Progress Made, More to Go
Authors: Henry W. Lim, Samantha L. Schneider
Journal: JAMA Dermatology (2017)

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 Dermatology 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 Dermatology journal
Contents, Vol. 5, 1957
Authors: G. Benedetti, P.C. Racamier, R. Schindler, W.Th. Winkler, Martti Siirala, A. Fuchs-Kamp, A. Storch, F.W. Beese, F. Meerwein, H. Bach, M. Sechehaye, K. Ernst, N. Elrod, Gerdhild von Staabs, Torsten Herner, Kauko Kaila, Georg Schwöbel, M. Boss
Journal: Psychotherapy and Psychosomatics (1957)
Effects of HLA-C and HLA-DQB1 Mismatching on Outcome of Unrelated Donor Marrow Transplantation for Patients with Severe Aplastic Anemia.
Authors: Seiji Kojima, Hiromasa Yabe, Koji Kato, Hisato Kigasawa, Hisashi Sakamaki, Masahiro Tsuchida, Shunichi Kato, Yasuo Morishima, Yoshihisa Kodera
Journal: Blood (2005)
Medicare Part D Plans’ Coverage and Cost-Sharing for Acute Rescue and Preventive Inhalers for Chronic Obstructive Pulmonary Disease
Authors: Chien-Wen Tseng, Jinoos Yazdany, R. Adams Dudley, Colette DeJong, Dhruv S. Kazi, Randi Chen, Grace A. Lin
Journal: JAMA Internal Medicine (2017)
Sepsis should be treated within one hour, says NICE
Authors: Jacqui Wise
Journal: BMJ (2017)

People with life threatening symptoms of sepsis should be reviewed and treated within an hour, the National Institute for Health and Care Excellence (NICE) has recommended.

In its draft quality standard NICE said that GPs, paramedics, and emergency department staff should check people with suspected sepsis for specific signs that indicate they are at high risk of severe illness or death. These signs include mottled or ashen appearance; cyanosis of the skin, lips, or tongue; non-blanching rash of the skin; …
대한간학회 제 8 차 춘계학술대회 초록집 / 포스터 전시 : Budd - Chiari 증후군에서 발생한 간세포암 1 예
Authors: 황준영, 정우진, 박경식, 조광범, 황재석, 안성훈, 박승국
Journal: Clinical and Molecular Hepatology (2002)