tbuckley/cabot-search huggingface dataset:
AB1065 Development of The First Skill Framework in Juvenile Idiopathic Arthritis
Authors: J.-D. Cohen, A. Belot, W. Fahy, P. Quartier, C. Sordet, S. Trope
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> In the “ list of healthcare treatments and interventions-JIA ” of 2009, HAS recalls that “ therapeutic education must ensure involvement of the patient and his parents: understandability of the disease, mastering of the technical skills, and adaptation of the lifestyle. The main aim of the ETP of the child, the adolescent, and his relatives is to take care of and prevent complications and to learn the skills involved in care. ” <h3>Objectives</h3> In this context, we have decided to develop a framework of the necessary skills needed for children suffering from JIA (REAJI) in order to improve their quality of life (knowledge, know-how, know-how-to-be). <h3>Methods</h3> The steering committee is composed of health professionals involved in paediatric rheumatology and patients9 associations. Different stages are scheduled: 1. Literature review 2. Setup of four focus groups (FG) with different facilitation methods: 2 patient FGs (8–12 year olds and adolescents), 1 parent FG, 1 health professional (HP) 3. Analysis by the steering committee et production of a first skills list. 4. Submission of the list to a panel of patients and to the national JIA competence centres for further information. 5. Submission of the completed list to the same participants for ranking (primary and secondary objectives). 6. Validation of the final list by the committee. <h3>Results</h3> The steering committee is made up of two rheumatologists, two paediatricians, and two patients associations, ANDAR (association of patients suffering from RA) and KOURIR (association of parents whose children suffer from JIA). The press review highlights a number of works, mainly on adolescents and outside the French territory. The HP FG is composed of 1 rehabilitation doctor, 1 rheumatologist, 1 paediatrician, 1 psychologist, 1 occupational therapist, 1 nurse and 1 pain nurse. The parent FG is composed of 10 people (6 mothers, 1 aunt, 3 fathers). The 8–12 year olds FG had 3 children and the adolescents FG 4 teenagers. The committee gathers to establish the initial skills list and then proposes to complete it via an online questionnaire to a selection of HPs from the 38 French centres listed by the Francophone Society of Rheumatology And Internal Paediatric Medicine (SOFREMIP) and an equal number of patients/ parents. This expanded list is sent back to participants using the same method in order to rank it and lastly to be validated in its final version by the committee. <h3>Conclusions</h3> The collectively-developed REAJI framework is a consensus-based support tool that anyone will be able to call upon: not only individual families but in particular teams working on educational projects. This mutidisciplinary participative, national effort is an example of health democracy, the results of which will be made available to all. <h3>Disclosure of Interest</h3> None declared
Effectiveness of Ursodeoxycholic Acid As Prophylactic Agent Against Hepatic Sinusoidal Obstruction Syndrome in Patients Undergoing Allogeneic Hematopoietic Cell Transplantation for Treatment of Hematological Malignancies
Authors: Hong Yen HY Ng, Mui Fong MF Chong, Jielin JL Song, William YK Hwang, Yeh Ching Linn, Yvonne SM Loh, Aloysius YL Ho, Yeow-Tee YT Goh
Journal: Blood (2012)
A Novel Recombinant Eklf-GATA1 Fusion Protein Reduces Erythrocytes Sickling in Human Erythroid Culture Model
Authors: Jianqiong Zhu, Kyung Chin, Wulin Aerbajinai,, Hongzhen Li, Chutima Kumkhaek, Matthew Hsieh, Griffin P Rodgers
Journal: Blood (2015)
SNP Associations with Event Free Survival in Myeloma from Two Phase III Clinical Trials Using the Bank On A Cure Chip.
Authors: Brian G. Van Ness, John C. Crowley, Christine Ramos, Suzanne M. Grindle, Antje Hoering, Jeff Haessler, Susanna Jacobus, Brian G. Durie, Michael Katz, Vincent S. Rajkumar, Bart Barlogie, David C. Johnson, Gareth J. Morgan
Journal: Blood (2006)
Evolution of Blast Crisis (BC) in Chronic Myeloid Leukemia (CML) in the Imatinib-Era: A Rare Event with High Proportions of Low Risk Patients and of Early Bc; Need for Rapid Detection. Results of the German CML Study IV.
Authors: Susanne Saussele, Michael Lauseker, Markus Pfirrmann, Armin Leitner, Nadine Pletsch, Frank Stegelmann, Anthony D Ho, Günter Schlimok, Albrecht Lindemann, Dietrich W Beelen, Alois Gratwohl, Mathias Hänel, Claudia Haferlach, Brigitte Schlegelberger, Martin C Müller, Philipp Erben, Susanne Schnittger, Susanne Jung-Munkwitz, Samina Shazi, Joerg Hasford, Andreas Hochhaus, Rüdiger Hehlmann
Journal: Blood (2009)
Genome-Wide Analysis of NOTCH1, ETS Family Factors, and RUNX1 Binding in Human T Lymphoblastic Leukemia Cells Reveals Distinct Regulatory Elements
Authors: Hongfang Wang, Chongzhi Zang, Len Taing, Hoifung Wong, Yumi Yashiro-Ohtani, Stephen Blacklow, Warren S. Pear, X. Shirley Liu, Jon C Aster
Journal: Blood (2012)
THU0612 Development and Validation of An Accurate and Precise Natural Language Processing System To Capture Rheumatoid Arthritis Disease Activity Measures: Table 1
Authors: G.W. Cannon, S. Mehortra, B.R. South, J. Leng, C.-C. Lu, B.C. Sauer
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Systems to electronically retrieve disease activity measures in medical records would significantly aid rheumatoid arthritis (RA) management and enhance epidemiologic research. As part of the national VA rheumatoid arthritis (VARA) registry our clinic measures 28 joint disease activity score (DAS28) by manual extraction (ManEx). <h3>Objectives</h3> 1. To develop a natural language processing (NLP) system to extract core measures from patient clinic note text and erythrocyte sedimentation rate (ESR) from the laboratory data to calculate DAS28. 2. To compare the accuracy and precision of the NLP DAS28 with ManEx DAS28. 3. To validate these findings in a second RA cohort. <h3>Methods</h3> An NLP application used clinical notes between January 1, 2015 and September 30, 2015 for RA patients in VARA (n=207) at our single site to extract tender joint count (TJC), swollen joint count (SJC), and patient global assessment (PtGA) from note text. The ESR value within 2 weeks closest to the clinic visit was combined with TJC, SJC, and PtGA to calculate DAS28. During the same observation period, ManEx of DAS28 observations were also identified. Discrepancies between ManEx and NLP were resolved by investigator review of the clinic notes. An analysis of all notes identified either by ManEx or NLP were evaluated for accuracy (% of values correctly detected in all notes) and precision (% of retrieved values by either ManEx or NLP correctly identified by that specific method). The NLP system was validated in a second cohort of RA patients in VARA (n=214) at our site between April 1, 2014 to December 31, 2014. <h3>Results</h3> The derivation set contained a total of 472 notes; 440 (93%) identified by both ManEx and NLP; 20 (4%) by ManEx alone and 12 (3%) notes by NLP alone. The accuracy and precision for TJC, SJC, PtGA, ESR and DAS28 are as noted in the table. Failure of NLP to detect core measures was because of use of incorrect note template (n=5, 1.0%) and template modification (n=18, 3.8%); however, when detected NLP precision was near 100%. Failure of ManEx was a result of missed notes (n=19, 4.0%) and data entry errors (n=13, 2.8%). Similar results were seen in the validations set of notes (n=484). <h3>Conclusions</h3> This accurate and precise NLP system can retrieve DAS28 from clinic notes to aid in clinical care and research activities. This method can be implemented at other VARA sites and other research networks to collect RA outcomes. <h3>Disclosure of Interest</h3> None declared
Unraveling the Efficacy and Safety Profiles of Anti-CD123 Chimeric Antigen Receptors (CARs) in a Model of Acute Myeloid Leukemia Immunotherapy By Investigating CAR Binding Affinity and Density Variables
Authors: Silvia Arcangeli, Marco Bardelli, Sarah Tettamanti, Maria Caterina Rotiroti, Luca Simonelli, Chiara F Magnani, Luca Varani, Andrea Biondi, Ettore Biagi
Journal: Blood (2015)
Phase II Study of R-CHOP Followed by 90Y-Ibritumomab Tiuxetan in Untreated Mantle Cell Lymphoma: Eastern Cooperative Oncology Group Study E1499.
Authors: Mitchell R. Smith, Lijun Zhang, Leo I. Gordon, James Foran, Brad Kahl, Randy D. Gascoyne, Ranjana Advani, Elisabeth Paietta, Edie Weller, Sandra J. Horning
Journal: Blood (2007)
Mesenchymal Stromal Cells From Myelodysplastic Syndrome Patients Show Lower DICER1 and DROSHA Expression, as Well as Decreased Mir-155 and Mir-181a MicroRNA Expression, When Compared with Healthy Controls
Authors: Carlos Santamaría, Olga López-VIllar, Sandra Muntión, Belén Blanco, Soraya Carrancio, Fermin M. Sanchez-Guijo, María Diez-Campelo, Stela Álvarez-Fernández, María E. Sarasquete, Marcos González, Jesús F. San Miguel, María C. Del Cañizo
Journal: Blood (2010)
Multiple Cytotoxic Factors and JAK/STAT Pathways Are Involved in the Enhanced Antitumor Function of CIK Cells Induced by IL-21
Authors: Sajin Rajbhandary, Mingfeng Zhao, Nan Zhao, Wenyi Lu, Haibo Zhu, Juanxia Meng, Xiao Chai, Xia Xiao, Qi Deng, Yuming Li, Yuliang Wang
Journal: Blood (2012)
SP0012 How To Assess The CVD Risk in The Individual Patient?
Authors: N. Sattar
Journal: Annals of the Rheumatic Diseases (2016)

This talk will overview the methods of assessing CVD risk in patients with Rheumatoid arthritis. It will give a rationale for why CVD risk screening in RA can be done using risk scores developed for the general population and also give two examples of scores which include RA as a risk stratifier. The talk will highlight that i) CVD risk scoring is easy to do in routine clinical practice, ii) requires simple additional of non-fasting lipids and blood pressure, iii) can be intergrated to existing RA clinics and iv) can be repeated at intervals of time which is pragmatic. The talk will further emphasise that consideration of usual CVD risk factors is critically important in determing who should be recommended statins and that having RA alone does not mean individuals are necessarily at high CVD risk. A case history will be used to highlight main points discussed in the talk. Finally, the talk will also discuss the pros and cons of additional tests and discuss the recommendations from recent guidelines which will fit in with the talks conclusions. <h3>Disclosure of Interest</h3> N. Sattar Consultant for: Roche, Astrazeneca, Amgen, Sanofi, Merck, Speakers bureau: Roche
THU0101 The Predictors of Fragility Fracture in Patients with Rheumatoid Arthritis: An Observational Study:
Authors: R. Sinha, M. Bukhari
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Rheumatoid arthritis (RA)is a well known risk factor for osteoporosis (OO) and is an independent risk factor in the FRAX ™ tool, additionally the femoral neck Bone mineral density (BMD) is included in the tool as a risk factor for fracture, but not the lumbar spine. <h3>Objectives</h3> To determine the predictors of fragility fractures in an observational cohort of patients with RA referred for BMD estimation, and whether using lumbar spine BMD would predit risk. <h3>Methods</h3> Patients referred for BMD estimation in a scanner in the North west of England between 2004 and 2014 with a history of RA were identified from a dual X-ray absorptiometry (DEXA) database. Demographics and other risk factors were also recorded. Fragility fractures were also recorded. Initially those who had sustained a fracture were compared to those who had not sustained a fracture using chi squared test for categorical variables and Student9s T test for continous variables. Subsequently univariate and multivariate logistic regression models were fitted. Variables included age at scan, gender, weight, height and body mass index (BMI),family history of fracture, alcohol, smoking, current steroid exposure, causes of secondary OP as per the FRAX™ definition, in addition to BMD in the lumbar spine (L1-L4) and the non-dominant femoral neck. <h3>Results</h3> 1527 patients were scanned in the referral period. Mean age at scan was 64.3 year (SD 11.6), 1207 (79.0%) were female, 406 (26.6%) had sustained a fracture. Patients who had sustained a fracture were more likely to be female 347/860 (28.8%) vs 59/320 (18.4%) (p&lt;0.001) and older 65.6 years (SD11.6) vs 63.9 years (SD11.5) (p=0.01). They also had lower BMD in the lumbar spine and the femoral neck. Results of the univariate analysis unadjusted and adjusted for age and gender are shown in table 1 with significant predictors marked with an asterisk (*). In the multivariate model, the variables that predicted fractures in this cohort the BMD in the lumbar spine (OR 0.33 95%CI 0.14,0.80), BMD in the femoral neck (OR 0.29 95%CI 0.09,0.94)and alcohol excess (OR 2.01 95%CI 1.12,3.61)family history (OR 1.49 95%CI 1.01,2.19)and secondary osteoporosis (OR 1.50 95%CI 1.03,2.18). Using the lumbar spine only gave an area under the curve (AUC) of the model of 0.640 whilst using the lumbar spine only gave an AUC of 0.633, using both gave an AUC of 0.646. <h3>Conclusions</h3> In univariate analysis, many risk factors are associated with fracture in this cohort with RA. Using the lumbar spine BMD improved the prediction of fractures, this is not included in the FRAX™ tool and would underestimate the fracture risk in this cohort. Further models including the L1l4 BMD should be developed to more accurately predict fractures in patients with RA. <h3>Disclosure of Interest</h3> None declared
The Experience on Multiparametric Molecular Diagnostic Approach of Pediatric Acute Lymphoblastic Leukemia (ALL) in Greece.
Authors: Agapi Parcharidou, Constantina Sambani, Christina Stavropoulou, George Paterakis, Chrysoula Belesi, Stefanos Papadhimitriou, Constantinos Tsitsikas, Vassilios Georgakakos, Vassilios Papadakis, Sophia Polychronopoulou
Journal: Blood (2006)
THU0235 Adjuvite: A Double-Blind, Randomized, Placebo-Controlled Trial of Adalimumab in Juvenile Idiopathic Arthritis Associated Uveitis: Table 1.
Authors: P. Quartier, V. Despert, S. Poignant, C. Elie, I. Kone-Paut, A. Belot, L. Kodjikian, D. Monnet, M. Weber, B. Bodaghi, A. Baptiste
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Juvenile idiopathic arthritis (JIA) patients may develop chronic, anterior uveitis. Topical steroids and methotrexate (MTX) often lack of efficacy. <h3>Objectives</h3> To asses safety and efficacty of adalimumab in patients with JIA-associated uveitis. <h3>Methods</h3> Elligible patients had chronic, "white eyes" uveitis with inadequate response to topical steroids and MTX, no previous anti-TNF antibody therapy and at least one assessable eye with inflammation quantified by laser flare photometry ≥30 photons/ms. Double-blind randomization at Day 1 (D1) into 2 equal groups, one treated with placebo and one with adalimumab (24 mg/m<sup>2</sup> in patients aged 4 to less than 13 years, 40 mg in patients ≥13), every other week subcutaneous injections. Primary objective: to demonstrate a higher response rate at Month 2 (M2) in the adalimumab arm versus the placebo arm. Response was defined as a 30% reduction of inflammation on laser flare photometry in the eye with the highest flare value at D1 and improvement or a stable appearance on slit lamp examination. From M2 to M12, all patients were allowed to receive adalimumab (open phase) (NCT01385826). <h3>Results</h3> 34 patients were screened and 31 received at least one injection of study treatment. At M2, in intention-to-treat (Primary objective), there were 9/16 responders in the adalimumab group and 3/15 in the placebo group (<i>p</i>=0.038, Chi-squared test; RR=2.81, CI95%=[0.94–8.45] with log-binomial model estimation). One patient stopped the trial at D14 (adalimumab group, uveitis worsening) and one at M9 (ocular hypertony). 29 patients reached M12 on adalimumab. There were 5 serious adverse events in 4 patients, all in the placebo group, 4 during the open-label phase, none related to study treatment (investigator assessment). <h3>Conclusions</h3> Adalimumab was effective in reducing ocular inflammation within 2 months and well tolerated over 12 months in patients with JIA-associated chronic uveitis and an inadequate response to topical steroids and MTX. Laser flare photometry is a valuable tool to assess early improvement. <h3>Disclosure of Interest</h3> P. Quartier Grant/research support from: Abbvie, Novartis, Pfizer, Roche, Consultant for: Abbvie, Novartis, SOBI, Speakers bureau: Abbvie, BMS, Novartis, PfiZer, Roche, SOBI, V. Despert: None declared, S. Poignant: None declared, C. Elie: None declared, I. Kone-Paut Grant/research support from: Roche, SOBI, Consultant for: Abbvie, Chugai, Novartis, Pfizer, Roche, SOBI, A. Belot: None declared, L. Kodjikian Consultant for: Alcom, Alimera, Allergan, Bayer, Bausch&amp;Lomb, Novartis, Thea, Speakers bureau: Alcom, Alimera, Allergan, Bayer, Bausch&amp;Lomb, Novartis, Thea, D. Monnet: None declared, M. Weber: None declared, B. Bodaghi Consultant for: Abbvie, A. Baptiste: None declared
OP0033 European Scleroderma Study Group Activity Index Captures Variations in Disease Activity over Time and Predicts Organ Damage in Early Systemic Sclerosis: Data from The Canadian Scleroderma Research Group: Table 1.
Authors: T. Nevskaya, M. Baron, J.E. Pope
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Endpoints in systemic sclerosis (SSc), such as modified Rodnan skin score (mRSS) and pulmonary function tests represent only parts of a multisystem disease. The European Scleroderma Study Group (EScSG) developed an SSc activity index, which captures involvement of multiple organs. The predictive validity of the SSc activity index is unclear. <h3>Objectives</h3> To estimate the effect of disease activity as measured by EScSG activity index, on the risk of subsequent organ damage in the Canadian Scleroderma Research Group (CSRG) cohort. <h3>Methods</h3> Of 421 SSc patients from the CSRG database with disease duration of ≤3 years, 197 who had no evidence of endstage organ damage initially and 3-year follow-up were included. Disease activity was assessed by EScSG activity index with two variability measures: 1. adjusted mean (am) EScSG activity score (the area under the curve of EScSG activity index over observation period), and 2. persistently active disease (PAD)/flare (EScSG score of ≥3 at follow-up visit). Internal organ involvement assessed over follow-up was based on Medsger severity scale and included accrual of a new severity score overall and by specific organ systems. Adjusted odds ratios (OR) for association between disease activity, severity and progression were calculated using regression analysis. <h3>Results</h3> After adjustment for covariates, amEScSG activity score was the most consistent predictor of risk at 3 year follow-up: disease progression as accrual of a new severity grade (Δ≥1) in any of the major internal organs (OR 1.42, 95%CI 1.14–2.25, p=0.007), lung disease (OR 1.86, 95%CI 1.31–2.64, p&lt;0.0001), decline in FVC (OR 3.05, 95%CI 1.47–6.31, p=0.003) and DLCO (OR 1.61, 95%CI 1.06–2.46, p=0.026), ΔHAQ (OR 1.729, 95%CI 1.250–2.391, p&lt;0.001), severity of visceral disease (Table). amEScSG predicted accrual of organ damage only in diffuse cutaneous SSc (dcSSc). In the multivariable analysis, progression of lung disease in dcSSc was predicted by amEScSG activity score (p=0.006), and severity of lung disease by amEScSG (p&lt;0.0001), older age (p=0.023), mRSS (p&lt;0.001) and initial severity of lung disease (p&lt;0.0001). The best predictor of renal disease severity at 3-year was initial severity of kidney involvement (p&lt;0.0001). <h3>Conclusions</h3> The mean adjusted EScSG reliably captured disease activity over time in individual patients and predicted the risk of deterioration in health status, disease progression and development of severe internal organ involvement in diffuse SSc, after adjustment for key covariates. <h3>Disclosure of Interest</h3> None declared
Comparison Between FISH and Immunostimulatory Oligonucleotide-Induced Metaphase Cytogenetics in Previously Untreated CLL: Impact on Prognosis
Authors: Alessandra Tedeschi, Mariangela Mura, Eleonora Vismara, Silvia Soriani, Francesca Ricci, Elisa Roncoroni, Gabriella De Canal, Michele Nichelatti, Enrica Morra, Marco Montillo
Journal: Blood (2011)
Updated Results of a Phase 2 Study of Bendamustine in Combination with Dexamethasone (Ben/Dex) in Patients with Previously-Treated Systemic Light-Chain (AL) Amyloidosis
Authors: Suzanne Lentzsch, Raymond L. Comenzo, Jeffrey A Zonder, Keren Osman, Miao Susanna, Daniel Backenroth, Daniel Otap, Wei-Yann Tsai, Siva Pregja, Shahzad Raza, Vaishali Sanchorawala, Heather J Landau
Journal: Blood (2015)
PTU-113 Acute Gastrointestinal Bleeding in Patients with an Implanted Left Ventricular Assist Device; A Single Centre Experience from The United Kingdom
Authors: A Marley, P Harvey, C Dobson, L Marley, S Lim, N Bhala, R Boulton
Journal: Gut (2016)

<h3>Introduction</h3> Left ventricular assist devices (LVAD) are used in patients with advanced heart failure, generally as a bridge to cardiac transplantation. One complication is gastrointestinal bleeding (GIB) often related to the development of angiodysplasia (AD), platelet dysfunction and the requirement for patients to be on anticoagulation as well as an antiplatelet agent. Achieving definitive haemostasis is challenging, often requiring multiple endoscopic and radiological interventions. We report a single centre experience with one of the largest UK LVAD populations. <h3>Methods</h3> All LVAD patients with episodes of GIB were assessed. GIB episode was defined as GIB requiring GI consultation. Data was collected from electronic patient record and review of patient notes. Interventions to achieve haemostasis were subdivided by those stopping a bleeding episode and interventions with no subsequent GIB episodes. <h3>Results</h3> Forty-one patients have had an LVAD implanted of which 8 (20%) had GIB, including 13 separate episodes. Three patients had recurrent GIB episodes. All patients were routinely anticoagulated including 7 on warfarin, 1 unfractionated heparin. Concomitant medications included; 7 aspirin and 1 clopidogrel. Investigation comprised; 16 gastroscopies (OGD), 3 flexible sigmoidoscopies (FS) and 6 CT angiograms (CTA). OGDs included findings of; 7 D2 oozing/angiodysplasia, 2 gastric oozing/angiodysplasia, 1 gastritis and 6 were normal. None of the FS provided either diagnosis or therapeutic opportunity. 5 CTAs were non-diagnostic, although one delineated a significant bleeding point amenable to embolisation. CTA was noted to suffer artefact from the LVAD likely reducing diagnostic accuracy Interventions included; one radiologically guided embolisation, 2 monotherapy argon plasma coagulation (APC), 1 monotherapy haemospray, 5 dual therapy (including combinations of APC, heater probe, adrenaline injection and haemospray). There was no intervention in 8 OGDs. Interventions preventing further episodes included one of; embolisation, dual therapy of APC and haemospray or adrenaline injection. In 5 patients haemostasis was spontaneous. <h3>Conclusion</h3> GIB was common in those with an LVAD in-situ, in three cases this was recurrent. In such cases haemostasis was challenging, requiring multiple interventions. All patients were anticoagulated and most used antiplatelet agents, which could not be safely stopped. Further investigation is required to establish the optimal approach to such high risk patients, however dual therapy including APC or, if possible, CTA with embolisation appear to be the strategies of choice. <h3>Disclosure of Interest</h3> None Declared
Clinical and Pathological Features of Non-Hodgkin Lymphomas Harboring Concurrent t(14;18) and 8q24 Anomalies
Authors: Ash A. Alizadeh, Matthew Anderson, Holbrook E Kohrt, Ragini M Shyam, Charles D. Bangs, Athena M Cherry, Ranjana Advani, Yasodha Natkunam, Ronald Levy
Journal: Blood (2010)