tbuckley/cabot-search huggingface dataset:
AB0748 Poliautoimmunity in scleroderma: data from de spanish scleroderma registry
Authors: D. Sanchez-Cano, M. Trigo-Rodríguez, A. Guillén del Castillo, R. Ríos-FernándeZ, C. Tolosa-Vilella, M. Rubio-Rivas, M. Freire, J. A. Todolí-Parra, M. Rodríguez-Carballeira, A. Marín-Ballvé, V. Fonollosa-Plá, C.P. Simeón-Aznar
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Systemic sclerosis (SSc) is a disorder with a well-known autoimmune base. There is evidence suggesting a common background with other connective tissue diseases, thus predisposing to the development of other autoimmne disorders in these patients, that is, poliautoimmunity (PAI). <h3>Objectives</h3> To assess PAI in a cohort of SSc patients. <h3>Methods</h3> A nationwide, cross-sectional study was performed including the 1928 patients enrolled in the Spanish SSc Registry (RESCLE). Ethics Committee approval was obtained by all participating centres. <h3>Results</h3> Prevalence of PAI was 45%, with 32% having more than one association. Most of these patients were women (93%, p&lt;0.001), with older age at diagnosis (53,7±14,7 years, p=0.027). Regarding subtypes, PAI was singnificantly more frequent in limited cutaneous SSc (48%, p=0.004) and pre-Ssc (47%, p&lt;0.001). Sjögren’s syndrome was by far the most common association (55%), followed by global autoimmune thyroid disorders (31%), autoimmune liver disorders (17%) and inflammatory myopathies (12%). Clinical features are shown on table 1. We found no significant differences concerning capillaroscopy patterns, or causes of death, neither SSc-related nor non-Ssc-related. Finally, a multivariate analysis with logistic regression was performed to evaluate the risk factors for PAI, which are shown on the table 2. <h3>Conclusions</h3> PAI in SSc is a frequent condition that special attention in these patients. We found a rather higher prevalence compared with those published in the literature, even though the distribution of those associated disorders was similar. In our cohort, there were no remarkable differences between both groups concerning clinical manifestations (although not always statistically significant). Finally, certain circumstances should make us aware of a possible associated condition to SSc. <h3>Reference</h3> [1] Elhai M, Avouac J, Kahan A, Allanore Y. Systemic sclerosis at the crossroad of polyautoimmunity. Autoimmun Rev. 2013;12:1052–7. <h3>Disclosure of Interest</h3> None declared
Personal Health Records
Authors: Christian Dameff, Brian Clay, Christopher A. Longhurst
Journal: JAMA (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 HomeNew OnlineCurrent IssueFor Authors Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More 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 journal
Incidence, Risk Factors, and Outcomes Associated With In-Hospital Acute Myocardial Infarction
Authors: Steven M. Bradley, Joleen A. Borgerding, G. Blake Wood, Charles Maynard, Stephan D. Fihn
Journal: JAMA Network Open (2019)

<h3>Importance</h3> Studies of acute myocardial infarction (AMI) occurring outside the hospital have informed approaches to addressing risk, treatment, and patient outcomes. Similar insights for in-hospital AMI are lacking. <h3>Objective</h3> To determine the incidence, risk factors, and outcomes associated with in-hospital AMI. <h3>Design, Setting, and Participants</h3> Cohort, nested case-control, and matched cohort study of patients hospitalized in US Veterans Health Administration facilities between July 2007 and September 2009. The incidence of in-hospital AMI was determined from a complete cohort of in-hospital AMI relative to the total number of inpatient admissions. From the in-hospital AMI cohort, detailed medical record review was performed on 687 cases and 687 individually matched controls. Risk factors and outcomes associated with in-hospital AMI were determined from matched comparison of in-hospital AMI cases to hospitalized controls. <h3>Exposures</h3> Candidate risk factors for in-hospital AMI included characteristics at the time of admission and in-hospital variables prior to the index date. <h3>Main Outcomes and Measures</h3> In the determination of the incidence and risk factors associated with in-hospital AMI, the outcome of interest was in-hospital AMI. All-cause mortality was the main outcome of interest following in-hospital AMI. <h3>Results</h3> A total of 5556 patients with in-hospital AMI (mean [SD] age, 73 [10] years; 5456 [98.2%] male) were identified among 1.3 million admissions, with an incidence of 4.27 in-hospital AMI events per 1000 admissions. Independent risk factors associated with in-hospital AMI included intensive care unit setting, history of coronary artery disease, heart rate greater than 100 beats/min, hemoglobin level less than 8 g/dL, and white blood cell count 14 000/μL or greater. Compared with the matched control group, mortality was significantly higher for patients with in-hospital AMI (in-hospital mortality, 26.4% vs 4.2%; 30-day mortality, 33.0% vs 10.0%; 1-year mortality, 59.2% vs 34.4%). <h3>Conclusions and Relevance</h3> In-hospital AMI was common and associated with common cardiovascular risk factors and markers of acute illness. Patient outcomes following in-hospital AMI were poor, with 1-year mortality approaching 60%. Further study of in-hospital AMI may yield opportunities to reduce in-hospital AMI risk and improve patient outcomes.
Relationship between Bone Marrow PD-1 and PD-L1 Expression and the Presence of Osteolytic Bone Disease in Multiple Myeloma Patients
Authors: Federica Costa, Marina Bolzoni, Rosanna Vescovini, Fabrizio Accardi, Anna Benedetta Dalla Palma, Federica De Luca, Valentina Marchica, Denise Toscani, Emanuela Vicario, Paola Storti, Franco Aversa, Nicola Giuliani
Journal: Blood (2018)
TP53 mutation status divides myelodysplastic syndromes with complex karyotypes into distinct prognostic subgroups
Authors: Detlef Haase, Kristen E. Stevenson, Donna Neuberg, Jaroslaw P. Maciejewski, Aziz Nazha, Mikkael A. Sekeres, Benjamin L. Ebert, Guillermo Garcia-Manero, Claudia Haferlach, Torsten Haferlach, Wolfgang Kern, Seishi Ogawa, Yasunobu Nagata, Kenichi Yoshida, Timothy A. Graubert, Matthew J. Walter, Alan F. List, Rami S. Komrokji, Eric Padron, David Sallman, Elli Papaemmanuil, Peter J. Campbell, Michael R. Savona, Adam Seegmiller, Lionel Adès, Pierre Fenaux, Lee-Yung Shih, David Bowen, Michael J. Groves, Sudhir Tauro, Michaela Fontenay, Olivier Kosmider, Michal Bar-Natan, David Steensma, Richard Stone, Michael Heuser, Felicitas Thol, Mario Cazzola, Luca Malcovati, Aly Karsan, Christina Ganster, Eva Hellström-Lindberg, Jacqueline Boultwood, Andrea Pellagatti, Valeria Santini, Lynn Quek, Paresh Vyas, Heinz Tüchler, Peter L. Greenberg, Rafael Bejar
Journal: Leukemia (2019)

Risk stratification is critical in the care of patients with myelodysplastic syndromes (MDS). Approximately 10% have a complex karyotype (CK), defined as more than two cytogenetic abnormalities, which is a highly adverse prognostic marker. However, CK-MDS can carry a wide range of chromosomal abnormalities and somatic mutations. To refine risk stratification of CK-MDS patients, we examined data from 359 CK-MDS patients shared by the International Working Group for MDS. Mutations were underrepresented with the exception of TP53 mutations, identified in 55% of patients. TP53 mutated patients had even fewer co-mutated genes but were enriched for the del(5q) chromosomal abnormality (p < 0.005), monosomal karyotype (p < 0.001), and high complexity, defined as more than 4 cytogenetic abnormalities (p < 0.001). Monosomal karyotype, high complexity, and TP53 mutation were individually associated with shorter overall survival, but monosomal status was not significant in a multivariable model. Multivariable survival modeling identified severe anemia (hemoglobin < 8.0 g/dL), NRAS mutation, SF3B1 mutation, TP53 mutation, elevated blast percentage (>10%), abnormal 3q, abnormal 9, and monosomy 7 as having the greatest survival risk. The poor risk associated with CK-MDS is driven by its association with prognostically adverse TP53 mutations and can be refined by considering clinical and karyotype features.
FRI0351 Clinical characteristics and outcomes of early-onset and late-onset lupus nephritis and its risk factors
Authors: C.-B. Choi, S. Won, S.-C. Bae
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> The kidney is one of the most commonly involved major organ in systemic lupus erythematosus (SLE). It may occur as an initial presentation at the onset of SLE and it can also present later in the course of the disease. <h3>Objectives</h3> To compare clinical characteristics, management, and outcomes after 12 months in patients with early-onset lupus nephritis (LN) and late-onset LN and to assess the risk factors for late-onset LN. <h3>Methods</h3> Patients with lupus nephritis enrolled in the Hanyang BAE Lupus Cohort were retrospectively assessed. Patients who developed LN within one year of the diagnosis of SLE (early-onset) were compared with those who developed LN more than a year later from the diagnosis of SLE (late-onset). Clinical characteristics including the features of SLE, management, and outcomes including renal responses and SLE disease activity were assessed. <h3>Results</h3> From 1,294 SLE patients in the Hanyang BAE Lupus Cohort, 641 (49.5%) patients had LN. Early-onset LN was observed in 469 (73.2%) and late-onset LN in 172 (26.8%). Hypertension was more frequent in early-onset LN while malar rash, discoid rash, photosensitivity, oral ulcer, arthritis, leukopenia, anti-Sm Ab, and anti-RNP Ab were more frequent in late-onset LN. Late-onset LN patients also showed lower C3 and higher activity index in renal biopsy. There was no significant difference in ISN/RPS classification and in induction therapy. SLEDAI score at onset of LN and after 12 months was similar in the two groups. Complete and partial response rates at six months and twelve months were also similar and there were no differences in progression to end-stage renal disease or death between the two groups. Multivariate analysis identified younger age at onset, malar rash, arthritis, serositis, anti-dsDNA Ab, and anti-Sm Ab as independent risk factors for late-onset LN. <h3>Conclusions</h3> Late-onset LN patients showed more mucocutaneous symptoms, autoantibodies, and higher activity index in renal biopsy compared to early-onset. However, there were no differences in outcomes after 12 months. Younger age at onset, malar rash, arthritis, serositis, anti-dsDNA Ab, and anti-Sm Ab were risk factors for late-onset LN. <h3>Disclosure of Interest</h3> None declared
FRI0586 Longitudinal assessment of cortical interruptions and vbmd in ra and healthy subjects using hr-pqct
Authors: M. Peters, J. van den Bergh, P. Geusens, A. Scharmga, D. Loeffen, R. Weijers, B. van Rietbergen, A. van Tubergen
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Preservation, repair and progression of cortical interruptions in finger joints has been observed in RA using conventional radiography, MRI, ultrasound and high-resolution peripheral quantitative computed tomography (HR-pQCT). Accurate and quantitative information about such changes over time, however, is still lacking. <h3>Objectives</h3> To evaluate changes over one year in cortical interruptions (number, surface area and volume), bone density (vBMD) and micro-structural parameters from HR-pQCT scans of finger joints in RA and healthy subjects (HS) using a recently developed semi-automatic algorithm for cortical interruptions detection.<sup>1</sup> <h3>Methods</h3> Baseline and 1 year follow up HR-pQCT scans of finger joints of 32 patients with RA (221 joints, 53% on biologic DMARDSs (bDMARDs)) and 32 HS (117 joints) were analysed for changes in cortical interruption, density and morphological parameters. Mean changes (at the group level) and proportions of joints (at the joint level) with changes beyond least significant changes were calculated after correction for baseline damage. <h3>Results</h3> At baseline, 530 interruptions (3.25/joint) were identified in RA, and 136 (1.45/joint) in HS. The mean of the interruption parameters did not change in either group, except for a significant increase in number of interruptions in PIP joints in RA. However, the proportion of joints showing repair in interruption volume was higher in RA than in HS (6.6% versus 1.7%, p=0.041). Changes in cortical interruption parameters were negatively correlated with changes in cortical BMD and thickness (p&lt;0.01). Mean vBMD decreased more and more joints showed loss of vBMD in RA than in HS (−4.4 versus −1.1 mgHA/cm<sup>3</sup> and 26.7% versus 12.9%, respectively, both p&lt;0.01). Proportionally more joints showed interruptions and loss of vBMD in patients with synthetic DMARDs (sDMARDS) compared to bDMARDs (6.1% versus 1.8% and 31.3% versus 17.2%, respectively, both p&lt;0.05). <h3>Conclusions</h3> Using HR-pQCT based semi-automatic cortical interruption analysis a large number of interruptions was identified in RA and HS. Cortical interruptions, vBMD and micro-structural parameters were more impaired in RA, of which vBMD and micro-structural parameters further deteriorated. Patients on sDMARDs had more joints with progression in number of interruptions and more loss of vBMD compared to bDMARDs. <h3>Reference</h3> [1] Peters M, Scharmga A, de Jong J, van Tubergen A, Geusens P, Arts JJ, Loeffen D, Weijers R, van Rietbergen B, van den Bergh J. An automated algorithm for the detection of cortical interruptions on high resolution peripheral quantitative computed tomography images of finger joints. PLoS One2017Apr 20;12(4):e0175829. <h3>Disclosure of Interest</h3> None declared
Clinical and Biological Indicators of Duvelisib Efficacy in CLL from the Phase 3 DUOTM Study
Authors: Jennifer R. Brown, Ian W. Flinn, Matthew S. Davids, Peter Hillmen, Marco Montillo, Zsolt Nagy, Julio Delgado, Bryone J. Kuss, Constantine S. Tam, Ulrich Jaeger, Paolo Ghia, Stephan Stilgenbauer, Carol Moreno, NgocDiep Le, Stephanie Lustgarten, Kam Sprott, Jonathan A. Pachter, David T. Weaver, Florence Cymbalista
Journal: Blood (2018)
Families Like This
Authors: Amy Haddad
Journal: Annals of Internal Medicine (2019)

Ad Libitum15 January 2019Families Like ThisAmy Haddad, PhDAmy Haddad, PhDCreighton University, Omaha, Nebraska (A.H.)Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/M18-1880 Audio Reading - “Families Like This” Audio. Michael A. LaCombe, MD, Annals Associate Editor, reads "Families Like This," by A. Haddad. Your browser does not support the audio element. Audio player progress bar Step backward in current audio track Play current audio trackPause current audio track Step forward in current audio track Mute current audio trackUnmute current audio track 00:00/ SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Speak too loudly, stink from sweatand beer. Thrown togetherlike strings of Christmas lightsjammed in a binwhere some work and some don't.Jessamyn's mine, the mother explains.Lonnie's ours.Families like this carry pocketknives in their overalls. Use oneto cut their boy down from the swing set,where he hung in the rain.Lay his body on the slide,wait for the squad. It was rainingthat day or we wouldn't neverhad looked out the window, neverwould've found him at all. It wasraining. They repeat the storyto the flock in the waiting room.Families like this are waryto every test and treatment.... Author, Article, and Disclosure InformationAffiliations: Creighton University, Omaha, Nebraska (A.H.)Current Author Address: Amy Haddad, PhD; e-mail, [email protected]edu. PreviousarticleNextarticle Advertisement Audio Reading - “Families Like This” Audio. Michael A. LaCombe, MD, Annals Associate Editor, reads "Families Like This," by A. Haddad. Your browser does not support the audio element. Audio player progress bar Step backward in current audio track Play current audio trackPause current audio track Step forward in current audio track Mute current audio trackUnmute current audio track 00:00/ FiguresReferencesRelatedDetails Metrics 15 January 2019Volume 170, Issue 2Page: 137KeywordsBeerDustHeart rateIntensive care unitsMusclesMyocardiumSweatVentilators ePublished: 15 January 2019 Issue Published: 15 January 2019 Copyright & PermissionsCopyright © 2019 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...
A pediatric regimen for older adolescents and young adults with acute lymphoblastic leukemia: results of CALGB 10403
Authors: Wendy Stock, Selina M. Luger, Anjali S. Advani, Jun Yin, Richard C. Harvey, Charles G. Mullighan, Cheryl L. Willman, Noreen Fulton, Kristina M. Laumann, Greg Malnassy, Elisabeth Paietta, Edy Parker, Susan Geyer, Krzysztof Mrózek, Clara D. Bloomfield, Ben Sanford, Guido Marcucci, Michaela Liedtke, David F. Claxton, Matthew C. Foster, Jeffrey A. Bogart, John C. Grecula, Frederick R. Appelbaum, Harry Erba, Mark R. Litzow, Martin S. Tallman, Richard M. Stone, Richard A. Larson
Journal: Blood (2019)
Understanding Perceived Barriers of Minority Medical Students Pursuing a Career in Dermatology
Authors: Yssra S. Soliman, Alexandra K. Rzepecki, Anthony K. Guzman, Ramone F. Williams, Steven R. Cohen, David Ciocon, Caroline Halverstam
Journal: JAMA Dermatology (2019)

Discussion | Although the number of cases of NUC in the study is small, the condition affected a high percentage of women receiving anticoagulation therapy, adding further support to the body of evidence that sex and anticoagulant use are 2 important risk factors for NUC.Warfarin, in particular, has been hypothesized to promote NUC by inhibiting vitamin K-dependent carboxylation of matrix Gla protein, decreasing inhibition of local calcification. 4he estimated 1-year survival rate for all patients with calciphylaxis has previously been reported as 45.8% 5 ; and patients with ulceration fare worse, with an estimated 80% mortality. 6atients in the present study had a high survival rate (75%) despite the fact that all patients had ulceration.One possible reason for this improved survival rate is the frequent use of sodium thiosulfate, with 63% of patients (n = 10) in our analysis receiving this form of therapy.This finding contrasts with the 48% survival rate previously reported among NUC patients 2 ; notably, none of these patients received sodium thiosulfate, suggesting a possible benefit associated with sodium thiosulfate therapy for NUC.A limitation of the study is that only patients who had a skin biopsy were included.The diagnosis of calciphylaxis is often made on clinical grounds, particularly in the setting of end-stage renal disease, and a skin biopsy is not always performed.
The ADAMTS13–VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension
Authors: Michael Newnham, Kieron South, Marta Bleda, William R. Auger, Joan A. Barberà, Harm Bogaard, Katherine Bunclark, John E. Cannon, Marion Delcroix, Charaka Hadinnapola, Luke S. Howard, David Jenkins, Eckhard Mayer, Choo Ng, Christopher J. Rhodes, Nicholas Screaton, Karen Sheares, Michael A. Simpson, Mark Southwood, Li Su, Dolores Taboada, Matthew Traylor, Richard C. Trembath, Sofia S. Villar, Martin R. Wilkins, John Wharton, Stefan Gräf, Joanna Pepke-Zaba, Michael Laffan, David A. Lane, Nicholas W. Morrell, Mark Toshner
Journal: European Respiratory Journal (2019)

Chronic thromboembolic pulmonary hypertension (CTEPH) is an important consequence of pulmonary embolism that is associated with abnormalities in haemostasis. We investigated the ADAMTS13-von Willebrand factor (VWF) axis in CTEPH, including its relationship with disease severity, inflammation, ABO groups and ADAMTS13 genetic variants.ADAMTS13 and VWF plasma antigen levels were measured in patients with CTEPH (n=208), chronic thromboembolic disease without pulmonary hypertension (CTED) (n=35), resolved pulmonary embolism (n=28), idiopathic pulmonary arterial hypertension (n=30) and healthy controls (n=68). CTEPH genetic ABO associations and protein quantitative trait loci were investigated. ADAMTS13-VWF axis abnormalities were assessed in CTEPH and healthy control subsets by measuring ADAMTS13 activity, D-dimers and VWF multimeric size.Patients with CTEPH had decreased ADAMTS13 (adjusted β -23.4%, 95% CI -30.9- -15.1%, p<0.001) and increased VWF levels (β +75.5%, 95% CI 44.8-113%, p<0.001) compared to healthy controls. ADAMTS13 levels remained low after reversal of pulmonary hypertension by pulmonary endarterectomy surgery and were equally reduced in CTED. We identified a genetic variant near the ADAMTS13 gene associated with ADAMTS13 protein that accounted for ∼8% of the variation in levels.The ADAMTS13-VWF axis is dysregulated in CTEPH. This is unrelated to pulmonary hypertension, disease severity or markers of systemic inflammation and implicates the ADAMTS13-VWF axis in CTEPH pathobiology.