tbuckley/cabot-search huggingface dataset:
AB0058 Eosinophil Cation Protein (ECP) – The Topping Attractor of The Disease Activity in Rheumatic Artritis (RA)
Authors: V.I. Shishkin, G.V. Kudriavtseva, V.V. Shishkin, Y.A. Malenkov
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> RA is systemic autoimmune disease characterized by the production of auto-reactive antibodies, formation of immune complexes causing tissue and organ damage, that reflects the degree of cellular destruction and the depth of the membrane pathological changes. ECP hinder the development of these negative phenomena. <h3>Objectives</h3> To assess serum levels of ECP, interleukins (IL-5, IL-10, IL-18) levels and immunoglobulins (IGA, IGE) levels in a population of patients with RA. <h3>Methods</h3> 15 patients with RA and 20 healthy controls were investigated in this study. Their ages changed from 35 to 60 years (female/male=3:1). The serum ECP, IL-5, IL-10, IL-18 and IGA, IGE levels were determined using (ELISA), and their correlations with the disease activity were measured using the DAS 28 and laboratory parameters including RF, antiCCP, erythrocyte sedimentation rate, complement (C3, C4), anti-double stranded – DNA antibody levels were analyzed. The clinically relevant correlation reported in the study parameter. <h3>Results</h3> The serum ECP, IG and IL levels were significantly higher (mean values ECP: 2500±134,7 ng/ml; IGA: 6970,5±357,9 mg/ml; IGE: 4350,9±210,7 ng/ml; IL-5: 632,5±19,9 pg/ml; IL-10: 956,4±14,7 pg/ml; IL-18: 2307,8±325,4 pg/ml) in RA patients compared to the control (mean values ECP: 21,7±0,9 ng/ml; IGA: 516,7±39,4 mg/ml; IGE: 37,0±9,4 ng/ml; IL-5: 53,7±12,6 pg/ml;IL-10: 93,4±17,8 pg/ml; IL-18: 187,3±25,4 pg/ml), (p&lt;0,001). Furthermore, such in increase in the levels of these analyzed parameters showed a highly significant positive correlation with the disease activity and laboratory parameters. <h3>Conclusions</h3> The production of pro-and anti-inflammation mediators and ECP in blood from patients with active RA shows a high degree of correlation with clinical disease activity measures. The level of ECP reflects the intensity of RA-inflammatory processes. It is proved expediency of application of the ECP-test in dynamics, status and forecast of the RA. <h3>Disclosure of Interest</h3> None declared
Integrating Newer Prognostic Factors in Evaluation of Previously Treated Patients with CLL Receiving Salvage Treatment.
Authors: Jerry D Henderson, Susan M O’Brien, Xuemei Wang, Stefan H Faderl, Alessandra Ferrajoli, Guillermo Garcia-Manero, Constantine S. Tam, Jan A Burger, Lynne V Abruzzo, Ellen Schlette, Susan Lerner, Michael J Keating, William G Wierda
Journal: Blood (2008)
PTH-064 Closing The Audit Loop Without Solving the Problem: Inappropriate Faecal Calprotectin Requests Continue Despite Agreed Guidelines: Abstract PTH-064 Table 1
Authors: H Parr, D Rampton, D Macdonald, C Scott
Journal: Gut (2016)

<h3>Introduction</h3> As a non-invasive marker of intestinal inflammation, faecal calprotectin (FCP) is widely used to minimise colonoscopy requests in patients presenting with diarrhoea and to monitor patients with established inflammatory bowel disease (IBD). We performed two successive audits of how well gastroenterologists in a specialist IBD unit adhere to defined agreed guidelines about the indications for FCP. <h3>Methods</h3> In mid 2014, we devised and circulated 3 criteria for requesting FCP. In early 2015, we audited the adherence of 45 successive requests for FCP to these criteria. On the basis of the results (below), we revised and recirculated the guidelines in early 2015, including 2 further criteria. In late 2015, we completed the audit loop by reviewing 80 recent FCP requests. <h3>Results</h3> The table shows the numbers of appropriately requested FCPs, as a percentage of all requests for each indication. The guidelines in italics are those added following the initial audit. There was a slight improvement in quality of requests for FCP between the first and the second audit. The most frequent and serious issue in FCP ordering was the requesting of colonoscopy simultaneously (15/80 in 2015) and/or ignoring of recent colonoscopy results (5/80 in 2015). There were no abnormal colonoscopies reported when FCP was normal. Consultants, SpRs and IBD nurses were equally prone to making inappropriate FCP requests. <h3>Conclusion</h3> Despite being issued with guidelines agreed and revised as a result of an initial audit, specialist clinicians continue to request FCP inappropriately. This results in avoidable colonoscopy and consequent unnecessary unpleasantness for patients and expense for health-care providers. Guidelines about when to request FCP need more effective dissemination and requests for colonoscopy should be vetted more stringently. <h3>Disclosure of Interest</h3> None Declared
Jumping Translocations of the Long Arms of Chromosome 1 (1qJT) in Myeloproliferative Neoplasms (MPNs) and Myelodysplastic Syndromes (MDS) Are Associated with High Risk of Transformation to Acute Myelogenous Leukemia (AML).
Authors: Vesna Najfeld, Joseph Tripodi, Steven Fruchtman, Lewis R. Silverman, Richard T. Silver, Angela Scalise, Ronald Hoffman
Journal: Blood (2009)
Reduction of cardiovascular disease inequalities in the USA through dietary policy
Authors: Jonathan Pearson-Stuttard, Piotr Bandosz, Colin Rehm, Jose Penalvo, Laurie Whitsel, Tom Gaziano, Zach Conrad, Parke Wilde, Renata Micha, Ffion Lloyd-Williams, Simon Capewell, Dariush Mozaffarian, Martin O'Flaherty
Journal: The Lancet (2016)
A trial of unrelated donor marrow transplantation for children with severe sickle cell disease
Authors: Shalini Shenoy, Mary Eapen, Julie A. Panepinto, Brent R. Logan, Juan Wu, Allistair Abraham, Joel Brochstein, Sonali Chaudhury, Kamar Godder, Ann E. Haight, Kimberly A. Kasow, Kathryn Leung, Martin Andreansky, Monica Bhatia, Jignesh Dalal, Hilary Haines, Jennifer Jaroscak, Hillard M. Lazarus, John E. Levine, Lakshmanan Krishnamurti, David Margolis, Gail C. Megason, Lolie C. Yu, Michael A. Pulsipher, Iris Gersten, Nancy DiFronzo, Mary M. Horowitz, Mark C. Walters, Naynesh Kamani
Journal: Blood (2016)
Central Nervous System Relapse in Patients with Acute Promyelocytic Leukemia Treated with All-Trans Retinoic Acid and Reinforced Anthracycline Monochemotherapy.
Authors: Pau Montesinos, Jose D. Gonzalez, Edo Vellenga, Chelo Rayon, Ricardo Parody, Angel Leon, Jordi Esteve, Juan Bergua, Gustavo Milone, Miguel A. Sanz
Journal: Blood (2007)
Outpatient Haploidentical Peripheral Blood Stem-Cell Transplantation with Post-Transplant Cyclophosphamide in Children and Adolescents
Authors: Oscar Gonzalez-Llano, Elias Eugenio Gonzalez-Lopez, Ana Carolina Ramirez-Cazares, Edson Rene Marcos-Ramirez, Guillermo J. Ruiz-Arguelles, David Gomez-Almaguer
Journal: Blood (2015)
AB0866 Cross-Sectional Evaluation of Vitamin D Levels in A Large Cohort of Patients with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS)
Authors: F. Falcini, G. Lepri, S. Stagi, E. Casalini, M. Matucci Cerinic
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Vitamin D (vitD) is known for its essential role in calcium homeostasis and bone health. It is now considered as a potent neurosteroid hormone, with a pivotal role on the brain development and normal brain function. VitD ligand-receptor, a receptor that mediates the majority of vitamin D biological actions, has been found throughout the body including the central nervous system (CNS). Vitamin D deficiency is commonly observed in patients with severe mental illness such as schizophrenia. PANDAS is an autoimmune disorder characterized by obsessive-compulsive symptoms and/or tics triggered by group-A beta-hemolytic Streptococcus infections with relapsing/remitting course due to recurrence of infections itself. Despite the action of 25-hydroxyvitamin D [25(OH)D] on immune and CNS system, to our knowledge, no studies have been performed in patients with PANDAS. <h3>Objectives</h3> To evaluate 25(OH)D levels in a large cohort of children and adolescents with PANDAS 2. To compare the results with a healthy control group. <h3>Methods</h3> We measured plasma 25(OH)D levels in one hundred seventy-nine patients with PANDAS (49 females, 132 males, mean age at diagnosis 101.4 ±30.1 months) and an age, sex, and body mass index-matched control group of 179 subjects. <h3>Results</h3> Patients with PANDAS showed a higher percentage of reduced 25(OH)-vitamin D levels (&lt;30 ng/mL) in comparison with controls (94.6% <i>vs.</i> 82.5%; p&lt;0.005). In addition, PANDAS patients have lower levels of 25(OH) D than controls (20.4±6.9 <i>vs.</i> 24.8±7.3 ng/mL, p&lt;0.0001). This difference has been confirmed also considering the winter (13.7±3.25 vs. 21.4±5.9) and summer (21.8±6.5 <i>vs.</i> 32.5±8.7) period. Yet, serum 25(OH)-vitamin D levels correlated with the number of streptococcal infections before the diagnosis (p&lt;0.005) and with their recurrences (p&lt;0.005). <h3>Conclusions</h3> PANDAS patients have reduced 25(OH)D values that seem to be associated with streptococcal infections, and the probability of its recurrences. So, reduced 25(OH)D levels may be a cause significantly important in PANDAS etiopathogenesis. A regular supplementation of VitD in PANDAS patients is recommended as it should improve the course of the disease and prevent or reduce the recurrences of infections and the relapses of neurological symptoms. This hypothesis should be further explored in future studies. <h3>Disclosure of Interest</h3> None declared
Modulating the Antithrombin-Mediated in Vivo Clearance of Coagulation Factor VIIa
Authors: Henrik Østergaard, Lene Hansen, Hermann Pelzer, Henrik Agersø, Anette A. Pedersen, Tina H. Glue, Laust B. Johnsen, Gorm Andersen, Jais R. Bjelke, Jens Breinholt, Henning Stennicke, Prafull S. Gandhi, Ole H. Olsen, Mette B. Hermit
Journal: Blood (2014)
Phase II Study of Clofarabine and Cytosine Arabinoside in Adult Patients with Relapsed AML and in Elderly Patients with Untreated AML Who Are at High Risk of Anthracycline Toxicity.
Authors: Edward Agura, R. Brian Berryman, Laura Brougher, Barry Cooper, Andrew De Leon, Joseph Fay, Houston Holmes, Christopher Maisel, Arturo Molina, Luis Pineiro, Giovanna Saracino, Mirjana Tadic-Ovcina, Estil Vance
Journal: Blood (2006)
SAT0188 Skeletal Muscle Fibers in Myositis Actively Upregulate Immunoproteasome Subunits
Authors: S. Bhattarai, K. Ghannam, S. Krause, L. Martinez-Gamboa, A. Marg, S. Spuler, O. Benveniste, W. Stenzel, E. Feist
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Idiopathic inflammatory myopathies (IIMs) are characterized by enhanced sarcolemmal expression of MHC class I molecules and infiltration of immune cells including CD8<sup>+</sup> T cells into skeletal muscle tissue. Immunoproteasome is a proteolytic complex that can efficiently produce peptides for antigen presentation via major histocompatability complex class I (MHC class I) molecules and is highly expressed in immune cells. Previously, we described upregulated expression of immunoproteasome subunits (β1i and β5i) within myositis muscle biopsies at the mRNA level suggesting its possible involvement in diseases pathogenesis <sup>[1]</sup>. <h3>Objectives</h3> The aim of this study was to clarify, whether immunoproteasomes are expressed within the muscle fibers of patients with IIMs and therefore, could be associated with the increased MHC class I surface expression. <h3>Methods</h3> Cryosections of muscle biopsies from sporadic Inclusion body myositis (sIBM), Immune-mediate necrotizing myopathy (IMNM), Dermatomyositis (DM) patients and healthy controls were examined for expression of proteasome subunits and cellular infiltrates by western blot and double-immunofluorescence. Proteasome activity was measured and compared between the different groups using a proteolytic assay <i>in vitro</i>. <h3>Results</h3> Western blot analyses of muscle biopsies from IBM (n=9), IMNM (n=9), DM (n=9) patients showed a strong upregulation of β1i and β5i subunits. Of note, double immunofluorescence provided clear evidence for an expression of immunosubunits β1i and β5i especially in the infiltrated muscle fibers in all studied disease conditions, whereas healthy muscle (n=4) fibers showed no staining for β1i and β5i. Interestingly, expression of proteasome immunosubunits was accompanied by increased MHC class I expression on the same muscle fibers. Both CD68<sup>+</sup> and CD14<sup>+</sup> macrophages showed strong staining of β1i and β5i in all disease group. In IBM, among the infiltrating cells about 50% of CD8+ T cells stained positive for β1i and β5i. In agreement with these results, significant increase in proteasomal chymotrypsin-like (CTL) activity was observed. <h3>Conclusions</h3> These results suggest direct involvement of immunoproteasome subunits β1i and β5i in the pathogenesis of myositis through enhanced upregulation of MHC class I. <h3>References</h3> Ghannam K, Martinez-Gamboa L, Spengler L, Krause S, Smiljanovic B, Bonin M, et al. (2014) Upregulation of Immunoproteasome Subunits in Myositis Indicates Active Inflammation with Involvement of Antigen Presenting Cells, CD8 T-Cells and IFNγ. PLoS ONE 9(8): e104048. doi:10.1371/journal.pone.0104048 <h3>Disclosure of Interest</h3> None declared
Infectious Events In Patients with Chronic Myeloid Leukemia Treated with Nilotinib as a Front Line Therapy and After Imatinib Failure
Authors: Ali M Al-Ameri, Jorge E. Cortes, Hagop Kantarjian, Elizabeth Burton, Alfonso Quintas-Cardama, Elias Jabbour, Xavier Badoux, Susan O'Brien, Gloria Mattiuzzi
Journal: Blood (2010)
The Risk of Sinusoidal Obstruction Syndrome (SOS) in Allogeneic Hematopoietic Stem Cell Transplantation after Prior Gemtuzumab Ozogamicin Treatment: A Retrospective Study on Behalf of the Acute Leukemia Working Party of the European Group for Blood and Marrow Transplantation
Authors: Giorgia Battipaglia, Myriam Labopin, Anna Candoni, Renato Fanin, Jean El Cheikh, Didier Blaise, Mauricette Michallet, Annalisa Ruggeri, Natalie Contentin, Josep Ribera, Michael Stadler, Jorge Sierra, Peter A. Von Dem Borne, Adrian Bloor, Gerard Socie, Mohamad Mohty, Arnon Nagler
Journal: Blood (2015)
Inter-Site Validation of a Single Breath Hold T2* MR Technique for Heart and Liver Iron Measurement.
Authors: Paul Kirk, Lisa J. Anderson, Mark A. Tanner, Renzo Galanello, Gildo Matta, Ru S. Tan, Ivg Ng, Angie Lai, Kevin Chen, David N. Firmin, Dudley J. Pennell
Journal: Blood (2005)
Tabes Dorsalis and Argyll Robertson Pupils
Authors: Chinar Osman, Tristan W Clark
Journal: New England Journal of Medicine (2016)

Images in Clinical MedicineA 47-year-old man with a history of human immunodeficiency virus (HIV) infection presented with an 8-month history of severe paroxysmal shooting pains in his legs, progressive difficulty in walking, tinnitus, and urinary incontinence.He had a fully suppressed HIV viral load and a CD4+ count of 400 cells per cubic millimeter while receiving antiretroviral therapy.The physical examination showed Argyll Robertson pupils, which are nonreactive to bright light but briskly constrict when focusing on a near object (see video).Magnetic resonance imaging showed high signal changes in the dorsal columns of the thoracic spine, and samples of blood and cerebrospinal fluid were positive for syphilis on Venereal Disease Research Laboratory (VDRL) testing and Treponema pallidum particle agglutination assay.The patient was treated with intravenous penicillin for 14 days, and gabapentin was started for the neuropathic leg pains.Tabes dorsalis is a form of neurosyphilis that is characterized by degeneration of the nerves in the dorsal columns of the spinal cord.Along with Argyll Robertson pupils, the condition is associated with ataxia and loss of proprioception.After treatment, the patient's symptoms and mobility slowly improved, although the shooting pains in his legs have continued despite pharmacotherapy.The serum VDRL level fell appropriately, and the result on VDRL testing of the cerebrospinal fluid was negative.
Synthesis, characterization and pharmacological evaluation of pyrazolyl urea derivatives as potential anti-inflammatory agents
Authors: Kanagasabai Somakala, Mohammad Amir
Journal: Acta Pharmaceutica Sinica B (2016)

p38α mitogen activated protein kinase (MAPK) inhibitors provide a novel approach for the treatment of inflammatory disorders. A series of fifteen pyrazolyl urea derivatives (3ao) were synthesized and evaluated for their p38α MAPK inhibition and antioxidant potential. Compounds 3ae, 3g and 3h showed low micromolar range potency (IC50 values ranging from 0.037 ± 1.56 to 0.069 ± 0.07 µmol/L) compared to the standard inhibitor SB 203580 (IC50 = 0.043 ± 3.62 µmol/L) when evaluated for p38α MAPK inhibition by an immunosorbent-based assay. Antioxidant activity was measured by a 2,2′-diphenyl-1-picryl hydrazyl radical (DPPH) free radical scavenging method and one of the compounds, 3c, showed better percentage antioxidant activity (75.06%) compared to butylated hydroxy anisole (71.53%) at 1 mmol/L concentration. Compounds 3ae, 3g and 3h showed promising in vivo anti-inflammatory activity (ranging from 62.25% to 80.93%) in comparison to diclofenac sodium (81.62%). The ulcerogenic liability and lipid peroxidation activity of these compounds were observed to be less in comparison to diclofenac sodium. These compounds also potently inhibited the lipopolysaccharide (LPS)-induced TNF-α release in mice (ID50 of 3ac = 19.98, 11.32 and 9.67 mg/kg, respectively). Among the screened compounds, derivative 3c was found to be the most potent and its binding mode within the p38α MAPK is also reported.
AB0728 Renal Disorders in Patients with Psoriatic Arthritis
Authors: E. Koraboev
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Objectives</h3> To study clinical and laboratory changes in kidney patients with psoriatic arthritis (PA). <h3>Methods</h3> The study included 60 patients. The age of patients ranged from 22 to 60 years. All patients underwent clinical and laboratory study: functional kidney tests, general analysis of blood, Zimnitsky test, Reberg test, creatinine and urea levels. <h3>Results</h3> Kidney disease was diagnosed in 25 (41,7%) patients. Nephropathy is directly related to the underlying disease, was detected in 18 (30.0%) patients, including chronic glomerulonephritis in 10 cases and renal amyloidosis in 6 cases. We failed to verify the nature of kidney impairment in 2 patients. There was the prevalence of men among patients with psoriatic nephropathy (in 17). Renal disease is seen in patients with other systemic manifestations of the PA, including the expressed trophic disorders (in 9), prolonged fever (in 5), lymphadenopathy (in 2), heart disease (in 6) and liver (in 3). Urinary syndrome was characterized by transient proteinuria and small haematuria (in 19 cases), a significant and persistent haematuria (red blood cells up to 30–40–50 in sight with episodes of gross haematuria) at extremely low values of proteinuria (in 5), regularly or occasionally high proteinuria above 3g/day (in 5). Extrarenal symptoms was presented with persistent oedema (in 4), hypertension (in 5). Nephritic syndrome was diagnosed in 12 patients. Reduced concentration ability of the kidneys was observed in 17 patients, and expressed presentation of chronic renal failure in 5 cases. Bladder syndrome was usually observed at the beginning of the PA, in exacerbations of joint syndrome with generalization process or the development of an advanced stage of dermatosis. In some cases, slight proteinuria and haematuria accompanied by acute syndromes was a comorbidity of main PA for many years without any development of kidney damage or azotaemia. Renal amyloidosis was characteristic for being accompanied by the nephritic syndrome. Daily protein loss reached 6–8g, and the amount of protein in a disposable examination of urine - 10,5 g/l. The total protein content in the serum was reduced to 49 g/l. Changes in urine, except for proteinuria, characterized microhaematuria (in 5 patients), small leukocytes (in 6), granular and wax cylinders. <h3>Conclusions</h3> Patients with PA in 41,7% cases had a variety of manifestations of the urinary syndrome. This represents the role of immediate diagnosis and correction of renal dysfunction; thus, chronic renal impairment may adversely affect prognosis of the disease. <h3>Disclosure of Interest</h3> None declared
SP0101 When To Use Objective and Subjective Assessment of Physical Activity? Issues of Reliability, Validity and Usability
Authors: T.W. Swinnen
Journal: Annals of the Rheumatic Diseases (2016)

Over the last decade, physical activity has become a key outcome domain in the assessment of rheumatic and musculoskeletal diseases (RMDs). Physical activity can be defined as any bodily movement produced by skeletal muscle that results in energy expenditure. Both physical and mental aspects of health in RMDs have been associated with optimal levels and patterns of physical activity. Also, intervention studies promoting physical activity in people with RMDs have largely confirmed these health benefits. Although there is consensus on its importance, the assessment of physical activity is complex and research in this area is rapidly growing. This lecture will therefore give a narrative overview of the state-of-the-art assessment of physical activity in RMDs considering the perspective of a researcher, a clinician, a patient and (to a lesser extent) the general population. In a first part, this lecture will discuss the relationship between physical activity and energy expenditure. More specifically, we will explain how total energy expenditure can be partitioned in obligatory energy expenditure to support basal metabolism (basal/resting metabolic rate), diet-induced energy expenditure and activity-induced energy expenditure (physical activity). A quick overview of gold standard tools to assess each component will be discussed in non-technical way. We will focus on the assessment of activity-induced energy expenditure (physical activity) using patient-reported (e-)questionnaires and (e-)diaries, observer-based and technology-based outcomes in RMDs. Often used single-sensor devices e.g. pedometer but also trends in more complex-sensor devices e.g. wearables combined with apps will be discussed. In a second part, we will shortly present the OMERACT framework to evaluate the measurement properties (i.e. how good is it?) of physical activity assessment tools. We will present the latest findings in this area for people with RMDs. Reliability and validity of commonly used outcomes e.g. steps, physical activity level or bouts of moderate to vigorous activities in RMDs and beyond will be presented. In a last part, we will formulate guiding principles in selecting the right physical activity assessment tool for your clinical or research question taking into account cost, measurement properties and feasibility (usability) for both clinicians and patients. <h3>Disclosure of Interest</h3> None declared
Poor Outcomes with Hyper CVAD Induction for T-Cell Lymphoblastic Leukemia/Lymphoma
Authors: Vamsi K Kota, Amanda Redden Hathaway, Bijal D. Shah, Deniz Peker, Ling Zhang, David L. Jaye, Martha Arellano, H Jean Khoury, Anand P Jillella, Elliott F. Winton, Leonard T Heffner, Uma Borate
Journal: Blood (2015)
Mutant BCL2 Co-Operates with CBFβ/PEBP2β-MYH11 to Promote Expansion of Leukemia Initiating Cells with a Predominantly Pro-Apoptotic Mechanism Via Recruitment of Ras-GTP In a Mouse Model of Progressive Acute Myeloid Leukemia.
Authors: Nader Omidvar, Carole Le Pogam, Stephanie Beurlet, Maria-Elena Noguera, Ann Janin, Scott C. Kogan, Irving L. Weissman, Marika Pla, Christine Chomienne, Rose Ann Padua
Journal: Blood (2010)