tbuckley/cabot-search huggingface dataset:
Mid-Term Valve-Related Outcomes After Transcatheter Tricuspid Valve-in-Valve or Valve-in-Ring Replacement
Authors: Doff B. McElhinney, Jamil A. Aboulhosn, Danny Dvir, Brian Whisenant, Yulin Zhang, Andreas Eicken, Flavio Ribichini, Aphrodite Tzifa, Michael R. Hainstock, Mary H. Martin, Ran Kornowski, Stephan Schubert, Azeem Latib, John D.R. Thomson, Alejandro J. Torres, Jeffery Meadows, Jeffrey W. Delaney, Mayra E. Guerrero, Stefano Salizzoni, Howaida El-Said, Ariel Finkelstein, Isaac George, Marc Gewillig, Maria Alvarez-Fuente, Luke Lamers, Asim N. Cheema, Jacqueline N. Kreutzer, Tanja Rudolph, David Hildick-Smith, Allison K. Cabalka, Younes Boudjemline, Guiti Milani, Martin L. Bocks, Jeremy D. Asnes, Vaikom Mahadevan, Dominique Himbert, Bryan H. Goldstein, Thomas E. Fagan, John P. Cheatham, Tarek S. Momenah, Dennis W. Kim, Antonio Colombo, Marco Ancona, Gianfranco Butera, Thomas J. Forbes, Eric Horlick, Carlos Pedra, Jacopo Alfonsi, Thomas K. Jones, Susan Foerster, Shabana Shahanavaz, Ivory Crittendon, Dietmar Schranz, Athar Qureshi, Martyn Thomas, Damien P. Kenny, Mark Hoyer, Sabine Bleiziffer, Joelle Kefer, Luca Testa, Matthew Gillespie, Danyal Khan, Robert H. Pass, Mohamed Abdel-Wahab, Harindra Wijeysundera, Filip Casselman, Tabitha Moe, Nicholas Hayes, Oluseun Alli, Keshav R. Nayak, Priti Patel, Nicolo Piazza, Cameron Seaman, Stephan Windecker, James Kuo, Frank F. Ing, Raj R. Makkar, Martin Greif, Alfredo G. Cerillio, Didier Champagnac, Fabian Nietlispach, Francesco Maisano, Hendrik Treede, Moritz Seiffert, Rui Campante Teles, Gudrun Feuchtner, Nikolaos Bonaros, Giuseppe Bruschi, Gabriele Pesarini
Journal: Journal of the American College of Cardiology (2019)
Persistence and effectiveness of nonbiologic systemic therapies for moderate‐to‐severe psoriasis in adults: a systematic review
Authors: K.J. Mason, S. Williams, Z.Z.N. Yiu, K. McElhone, D.M. Ashcroft, C.E. Kleyn, Z.K. Jabbar‐Lopez, C.M. Owen, N.J. Reynolds, C.H. Smith, N. Wilson, R.B. Warren, C.E.M. Griffiths
Journal: British Journal of Dermatology (2019)

The persistence and effectiveness of systemic therapies for moderate-to-severe psoriasis in current clinical practice are poorly characterized.To systematically review observational studies investigating the persistence and effectiveness of acitretin, ciclosporin, fumaric acid esters (FAE) and methotrexate, involving at least 100 adult patients with moderate-to-severe psoriasis, exposed to therapy for ≥ 3 months.MEDLINE, Embase, the Cochrane Library and PubMed were searched from 1 January 2007 to 1 November 2017 for observational studies reporting on persistence (therapy duration or the proportion of patients discontinuing therapy during follow-up) or effectiveness [improvements in Psoriasis Area and Severity Index (PASI) or Physician's Global Assessment (PGA)]. This review was registered with PROSPERO, number CRD42018099771.Of 411 identified studies, eight involving 4624 patients with psoriasis were included. Variations in the definitions and analyses of persistence and effectiveness outcomes prevented a meta-analysis from being conducted. One prospective multicentre study reported drug survival probabilities of 23% (ciclosporin), 42% (acitretin) and 50% (methotrexate) at 1 year. Effectiveness outcomes were not reported for either acitretin or ciclosporin. The persistence and effectiveness of FAE and methotrexate were better characterized, but mean discontinuation times ranged from 28 to 50 months for FAE and 7·7 to 22·3 months for methotrexate. At 12 months of follow-up, three studies reported that 76% (FAE), 53% (methotrexate) and 59% (methotrexate) of patients achieved ≥ 75% reduction in PASI, and one reported that 76% of FAE-exposed patients achieved a markedly improved or clear PGA.The comparative persistence and effectiveness of acitretin, ciclosporin, FAE and methotrexate in real-world clinical practice in the past decade cannot be well described due to the inconsistency of the methods used.
Acute maternal confusion and neonatal seizure postpartum
Authors: Adam D Jakes, Jillian Lloyd, Catherine Nelson-Piercy
Journal: BMJ (2019)

A 32 year old nulliparous woman underwent induction of labour with dinoprostone at 41+2 weeks’ gestation. Her membranes were artificially ruptured after 24 hours and she requested an epidural before augmentation of labour. A litre of Hartmann’s solution was administered simultaneously with the epidural to correct any epidural-induced hypotension. A synthetic oxytocin (syntocinon) infusion (10 units/50 mL in sodium chloride 0.9%) was started at 2 cm cervical dilatation. She remained on intravenous fluid throughout her labour, receiving a further litre of fluid six hours later. At 8 cm cervical dilatation, a persistent fetal bradycardia warranted an emergency caesarean section. Estimated blood loss was 830 mL and she received 2 L of fluid intra-operatively.

She gave birth to a 3975 g baby girl with Apgar scores of 9 at one minute and 10 at five minutes. The mother received 10 units of oxytocin to achieve adequate uterine tone. A continuous infusion of 40 units of syntocinon in a litre of Hartmann’s solution was also administered over four hours as per hospital protocol.

At 4 hours of age, the baby had a tonic-clonic seizure lasting 40 seconds and the mother became acutely confused. The mother was clinically euvolaemic and computed tomography of the head was normal. Blood results are shown in table 1.

View this table:

Table 1 
Neonatal and maternal blood tests



It later transpired the mother had drunk more than 4 L of fluid during labour, in addition to the 4 L given intravenously.

1. What is the diagnosis?

2. What factors might contribute …
THU0363 Association of depression with socioeconomic status, anticardiolipin anti-bodies, and organ damage in patients with systemic lupus erythematosus: results from the kornet registry
Authors: K.-E. Lee, J.-H. Kang, D.-J. Park, S.-S. Lee
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Objectives</h3> Depression is more common in patients with systemic lupus erythematosus (SLE) compared to the general population. However, few studies have investigated risk factors of depression in SLE patients, and the results are inconsistent. This study evaluated the prevalence of, and risk factors for, depression in ethnically homogeneous Korean SLE patients. <h3>Methods</h3> In this study, 505 consecutive SLE patients were enrolled from the Korean Lupus Network registry. Demographic variables, clinical manifestations, laboratory findings, physician global assessment, and SLEDAI-2000 and SLICC damage index were recorded at enrollment. Patients were identified as having depressive symptoms using the Korean version of the Beck Depression Inventory (BDI) with a cut-off ≥16, and categorised into four groups. Multivariable logistic regression analyses were performed to identify independent risk factors for depression defined as a BDI score ≥16. <h3>Results</h3> Of the 505 patients, 97 (19.2%) were diagnosed with depression. Patients with a higher BDI score were older, more likely to be a current smoker, and had a SLICC score &gt;1. Conversely, they had lower income and educational levels. Regarding the serologic findings, patients with a higher BDI score had lower anti-double-stranded DNA positivity and higher anticardiolipin (aCL) positivity. On multivariate analysis, the following factors were associated with depression: current smoking status (OR 2.533, p=0.049), aCL-positivity (OR 2.009, p=0.035), and a SLICC damage index score &gt;1 (OR 2.781, p=0.039). On the other hand, high-level education (OR 0.253, p=0.024) and a high income (OR 0.228, p=0.008) were negatively associated with depression. <h3>Conclusions</h3> Our results show that depression is prevalent in patients with SLE and multiple factors are associated with depression in SLE. These data could help guide target programs for those at high risk of depression in SLE. <h3>Disclosure of Interest</h3> None declared
SAT0599 Idiopathic granulomatous mastitis may response well to combination of immunosupresives and glucocorticoids
Authors: A. Sari, A. Konan, M. Akpinar, B. Armagan, A. Erden, L. Kilic, O. Karadag, A. Akdogan, S. Apras Bilgen, S. Kiraz, I. Ertenli, U. Kalyoncu
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Idiopathic granulomatous mastitis (IGM) is a rare inflammatory disease of breast. Corticosteroids (CS) and immunosuppressive agents constitute treatment alternatives other than surgery. <h3>Objectives</h3> To evaluate the clinical characteristics and treatment responses of IGM patients followed up in our clinic. <h3>Methods</h3> The medical records of 70 IGM patients who were referred to Hacettepe University Rheumatology Clinic were examined. Forty-four patients who had at least one visit in the last 2 years were included in the analysis. Demographic, clinical and laboratory characteristics of the patients, treatments, clinical and/or ultrasonographically measured lesion sizes at the time of diagnosis and at the last follow-up were recorded. Complete and partial response in the last control visit were defined as ≥50% and&lt;50% decrease in the lesion size, respectively. Lesions which are stable or increasing in size was accepted as unresponsive. Increase in the drug dose by the clinician or increasing of the lesion size during follow-up were considered as relapse. <h3>Results</h3> Median age was 35.7 (24.3–57.2) years and median symptom duration was 2.5 (0.2–54.1) months at baseline. Palpable mass (90.9%) and breast pain (88.1%) were the most common symptoms. Skin fistules and axillary lymphadenopathy were present in 16 (37.2%) and 15 (34.1%) patients, respectively. Erythema nodosum was seen in 5 (11.4%) patients during follow-up. The median follow-up duration was 10.5 (1.05–99.6) months. CS monotherapy and combination of CS and immunosuppressive were used in 3 (7.8%) and 38 (86.4%) patients, respectively. Three patients were followed up without treatment. The first immunosuppressive agent was methotrexate (MTX) in 32 patients (84.2%) and azathiopurine (AZA) in 6 patients (15.7%). In one patient, MTX was switched to AZA due to hepatotoxicity. Treatment regimens and responses of the patients are summarised in table 1. IGM lesions disappeared in 24 patients at the end of follow-up. In two patients who were on MTX and KS, surgical excision was required because of unresponsiveness. Among relapsed patients (n=5), 2 patients were on MTX and KS, 1 patients was on AZA and KS and 2 patients were on KS at the time of relapse. In one patient, relapse was observed 1 year after cessation of MTX and KS. <h3>Conclusions</h3> In a case series previously published by our study group, the efficacy of the combined use of KS and immunosuppressant's in IGM treatment was retrospectively studied.<sup>1</sup> In this study, the efficacy of immunosuppressive therapy has been demonstrated with prospective approach. <h3>Reference</h3> [1] Konan A, et al. Breast Care2012;7:297–301. <h3>Disclosure of Interest</h3> None declared
Chimeric Antigen Receptor T-Cell Therapy Shapes T Cell Repertoire in Chinese Patients with B-Cell Acute Lymphocyte Leukemia
Authors: Xiujian Wang, Tao Sun, Yongxian Hu, Xiao Liu, Jian Yu, Pengfei Xu, Guoqing Wei, Chao Jin, Wenjun Wu, Fu Huarui, Lijuan Ding, Fang Ni, Hao Zhang, Zuyu Liang, Binsheng Wang, Xiaoqing Li, Cong Wei, Yunyun Deng, Jimin Shi, Lei Xiao, Zhao Wu, He Huang
Journal: Blood (2018)
Derivation and validation of the SLE Disease Activity Score (SLE-DAS): a new SLE continuous measure with high sensitivity for changes in disease activity
Authors: Diogo Jesus, Ana Matos, Carla Henriques, Margherita Zen, Maddalena Larosa, Luca Iaccarino, José António Pereira Da Silva, Andrea Doria, Luís Sousa Inês
Journal: Annals of the Rheumatic Diseases (2019)

To derive and validate a new disease activity measure for systemic lupus erythematosus (SLE), the SLE Disease Activity Score (SLE-DAS), with improved sensitivity to change as compared with SLE Disease Activity Index (SLEDAI), while maintaining high specificity and easiness of use.We studied 520 patients with SLE from two tertiary care centres (derivation and validation cohorts). At each visit, disease activity was scored using the Physician Global Assessment (PGA) and SLEDAI 2000 (SLEDAI-2K). To construct the SLE-DAS, we applied multivariate linear regression analysis in the derivation cohort, with PGA as dependent variable. The formula was validated in a different cohort through the study of: (1) correlations between SLE-DAS, PGA and SLEDAI-2K; (2) performance of SLEDAI-2K and SLE-DAS in identifying a clinically meaningful change in disease activity (ΔPGA≥0.3); and (3) accuracy of SLEDAI-2K and SLE-DAS time-adjusted means in predicting damage accrual.The final SLE-DAS instrument included 17 items. SLE-DAS was highly correlated with PGA (r=0.875, p<0.0005) and SLEDAI-2K (r=0.943, p<0.0005) in the validation cohort. The optimal discriminative ΔSLE-DAS cut-off to detect a clinically meaningful change was 1.72. In the validation cohort, SLE-DAS showed a higher sensitivity than SLEDAI-2K (change ≥4) to detect a clinically meaningful improvement (89.5% vs 47.4%, p=0.008) or worsening (95.5% vs 59.1%, p=0.008), while maintaining similar specificities. SLE-DAS performed better in predicting damage accrual than SLEDAI-2K.SLE-DAS has a good construct validity and has better performance than SLEDAI-2K in identifying clinically significant changes in disease activity and in predicting damage accrual.
Abstract P301: The Association of Physical Activity and Inflammation is Independent of Central Obesity in the Multi-Ethnic Study of Atherosclerosis
Authors: Chantal A Vella, Matthew A Allison, Mary Cushman, Nancy S Jenny, Mary P Miles, Britta Larsen, Susan G Lakoski, Erin D Michos, Michael J Blaha
Journal: Circulation (2017)

Purpose: Physical activity is associated with decreased adiposity-related inflammation in adults. Whether this association is independent of central obesity is unknown but important for understanding the mechanisms associated with reducing cardiometabolic disease risk through physical activity. This study examined whether associations of physical activity and obesity-related inflammatory markers were independent of central adiposity. Methods: Between 2002 and 2005, 1970 participants from the Multi-Ethnic Study of Atherosclerosis completed detailed health history and physical activity questionnaires, underwent physical measurements including computed tomography to quantify abdominal visceral and subcutaneous fat, and measurements of adiponectin, leptin, interleukin-6, tumor necrosis factor-alpha, and resistin. Statistical analyses included analysis of covariance and multivariable-adjusted regression. Results: The mean (range) age of participants was 64.7 (55-84) years and 50% were female. After adjustment for age and sex, and compared to the lowest quartile, inflammatory markers in the highest quartile of moderate-to-vigorous physical activity were 16% higher for adiponectin and 30%, 26% and 9% lower for leptin, interleukin-6, and resistin, respectively (p&lt;0.05 for all). In linear regression adjusted for demographics, dyslipidemia, hypertension, diabetes, smoking, glomerular filtration rate, renin and aldosterone, each standard deviation increment of moderate-to-vigorous physical activity was associated with significantly higher levels of adiponectin (β=0.04) and lower levels of leptin (β=-0.06), interleukin-6 (β=-0.08), and resistin (β=-0.05, p&lt;0.05 for all). The associations with leptin, interleukin-6, and resistin were independent of total and central adiposity (p&lt;0.05), whereas the association between moderate-to-vigorous physical activity and adiponectin was attenuated by central adiposity (p&gt;0.05). There were no significant interactions by race/ethnicity or sex. Conclusions: Moderate-to-vigorous physical activity was associated with a more favorable profile of inflammatory markers, independent of relevant cardiometabolic disease risk factors including central obesity.
Cellular therapy against public neoantigens
Authors: Paul M. Armistead
Journal: Journal of Clinical Investigation (2019)

Neoantigen-targeted therapies have typically been based upon personalized neoantigen-specific vaccines; however, in this issue of JCI, van der Lee et al. describe the development of a potential cellular immunotherapy targeting a "public" neoantigen derived from nucleophosmin 1 (NPM1), which is mutated in approximately 30% of acute myeloid leukemias (AMLs). The authors use reverse immunology to predict, and biochemically confirm, NPM1-derived neoepitopes (ΔNPM1) and then generate high-avidity T cell clones and retrovirally transduced T cell populations that kill NPM1-mutated AML. This study provides a general approach to adoptive cellular therapy that can be applied to targeting other tumors with public neoantigens.
Abstract 20597: Transcriptome Profiling and a Novel in vitro Model of Single Ventricle Congenital Heart Disease
Authors: Anastacia M Garcia, Stephanie J Nakano, Anis Karimpour-Fard, Brian L Stauffer, Carmen C Sucharov, Shelley D Miyamoto
Journal: Circulation (2017)

Introduction: The molecular mechanisms underlying heart failure (HF) in the single ventricle (SV) population are poorly understood, and further investigation is limited by the lack of a model syste...
A Novel Prognostic Model Including Cytogenetic and Molecular Data in Patients with Primary and Post-Essential Thrombocythemia (ET)/Polycythemia Vera (PV) Myelofibrosis (MF)
Authors: Fabio PS Santos, Bartlomiej Getta, Lucia Masarova, Christopher Famulare, Jessica Schulman, Tarcila Santos Datoguia, Giulliana AR Goncalves, Nelson Hamerschlak, Hagop M. Kantarjian, Ross L. Levine, Paulo Vidal Campregher, Raajit K. Rampal, Srdan Verstovsek
Journal: Blood (2018)
P049 Age-associated B cells in early drug-naÏve rheumatoid arthritis patients
Authors: G Vidal Pedrola, A Pratt, A Mellor, D Scheel-Toellner, J Isaacs, A Anderson
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> Rheumatoid arthritis (RA) is a chronic autoimmune disorder characterised by joint inflammation and bone destruction. The presence of autoantibodies, years before the clinical onset of disease, and the efficacy of Rituximab, a B-cell depleting therapy, highlight a pathogenic role for B cells. Different groups have recently identified a novel subset of B cells named age-associated B cells (ABCs). Studies in mice autoimmune models and patients suffering from autoimmune diseases described these cells as CD19<sup>high</sup> CD21<sup>-</sup> CD11c<sup>+</sup>. Moreover, a subset of synovial fluid B cells with low levels of CD21, expresses FcRL4 and produces the cytokine RANKL, which stimulates the differentiation and activation of osteoclasts. The ABCs found in peripheral blood could therefore be the precursors of this FcRL4 positive subset found in synovia. <h3>Objectives</h3> We aimed to investigate the proportion and phenotype of peripheral blood ABCs in patients suffering from early drug naïve RA. <h3>Methods</h3> Newly presenting patients, naïve to immunomodulatory treatment, were recruited from the Newcastle Early Arthritis Clinic, and followed until diagnoses were confirmed. B-cell subsets in peripheral blood were detected and phenotyped using flow cytometry. NanoString nCounter Immunlogy v2 Panel (NanoString Technologies) was used to detect mRNA from cell lysates of sorted ABCs, naïve, memory and CD5+ B cells from RA patients, age-matched healthy controls and disease controls (Psoriatic Arthritis patients). <h3>Results</h3> Our work showed that there are no significant differences in the frequency of ABCs between RA patients, disease controls and age-matched healthy controls. There is a possible trend for increased frequencies of ABCs with age as well as those who have a high disease activity. Our results also show that ABCs resemble a memory B cells phenotype with regard to class-switch immunoglobulins expression, with a significant percentage of them being positive for IgG and IgA. Interestingly, the FcRL4<sup>+</sup>, the proliferating Ki67<sup>+</sup> and the T-bet expressing B cells were enriched in the ABC population compared to the other B cell subsets. Furthermore, ABCs expressed high levels of MHC class II and co-stimulatory molecules, as well as the activation marker, CD69. In addition, gene expression analysis using NanoString showed differentially expressed genes between ABCs and memory B cells. <h3>Conclusions</h3> These data supports an activated phenotype of the ABCs, which supports the idea that ABCs have a pathogenic role in RA, potentially via autoantibody and T cell stimulatory ability. However, further characterisation of this subset and functional studies are needed. <h3>Disclosure of Interest</h3> None declared.
AB0585 A lupus low disease activity state is associated with reduced flare, lower organ damage accrual, and better quality of life in patients with systemic lupus erythematosus
Authors: J.-H. Kang, K.-E. Lee, D.-J. Park, S.-S. Lee
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Objectives</h3> To identify the potential predictors of a lupus low disease activity state (LLDAS), and the relationship between LLDAS and disease flare, organ damage, and quality of life in Korean patients with systemic lupus erythematosus (SLE). <h3>Methods</h3> The study followed 181 SLE patients from a single centre for three years. LLDAS was defined as follows:<sup>1</sup> SLE Disease Activity Index (SLEDAI)−2K≤4, with no activity in major organ systems;<sup>2</sup> no new lupus disease activity compared with the previous assessment;<sup>3</sup> SLEDAI Physician Global Assessment≤1;<sup>4</sup> a current prednisolone (or equivalent) dose ≤7.5 mg daily; and<sup>5</sup> well-tolerated standard maintenance doses of immunosuppressive drugs. We assessed data annually and divided 4 groups according to the number of LLDAS; LLDAS=0, 1, 2, and 3. Univariate and multivariate analyses were performed to identify predictors of LLDAS. <h3>Results</h3> Of the 181 patients, 16.0% attained LLDAS on three consecutive years. Each group shows as follows; no LLDAS (n=30), LLDAS=1 (n=60), LLDAS=2 (n=62), and LLDAS=3 (n=29). The patients who had higher number of LLDAS had shorter duration of symptoms, lower anti-histone antibody positivity, lower cumulative prescribed dose of prednisolone at baseline, lower mean PGA, lower mean SLEDAI, lower mean Mental Component Summary in SF-36, lower change in SLICC/ACR damage index, and a lower frequency of flare. In the multivariate analysis, LLDAS was significantly associated with lower mean PGA (OR=0.671, 95% CI: 0.112–0.989, p=0.019) and a reduced risk of flare after adjusting for confounders (OR=0.012, 95% CI: 0.001–0.448, p=0.017). <h3>Conclusions</h3> Attaining LLDAS was associated with an improved outcome, as represented by a decreased rate of disease flare, lower organ damage accrual, and better quality of life in Korean patients with SLE. <h3>Disclosure of Interest</h3> None declared
Members of the microRNA-106a-363 Cluster Associate with Unfavorable Outcome in Adult Acute Myeloid Leukemia Patients and Promote Leukemogenesis invivo through Increased Metabolic Activity
Authors: Nadine Sperb, Kathrin Krowiorz, Sarah Grasedieck, Christoph Rueß, Nicole Pochert, Edith Schneider, Pegah Johansson, Erik Delsing Malmberg, Laleh Arabanian, Tobias Maetzig, Linda Fogelstrand, Hartmut Döhner, Lars Palmqvist, Reinhild Rösler, Sebastian Wiese, Enrico Calzia, Arefeh Rouhi, Florian Kuchenbauer
Journal: Blood (2018)
Lenalidomide Plus Hypomethylating Agent for Acute Myeloid Leukemia (AML) with Recurrent Genetic Abnormalities -AML with Inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, Mecom
Authors: Lauren E. Merz, Anthony J. Perissinotti, Bernard Lawrence Marini, Ashley Crouch, Kristen Pettit, Patrick W. Burke, Harry P. Erba, Dale L. Bixby
Journal: Blood (2018)