tbuckley/cabot-search huggingface dataset:
Atpase Family AAA Domain-Containing Protein 2 (ATAD2) As a Novel Target in Multiple Myeloma
Authors: Lakshmi B. Potluri, Srikanth Talluri, Leutz Buon, Mehmet K. Samur, Anil Aktas-Samur, Jialan Shi, Chandraditya Chakraborty, Subodh Kumar, Rao Prabhala, Masood A. Shammas, Nikhil C. Munshi
Journal: Blood (2020)
Influence of Inherited and Acquired Hematological and Immunological Gene Variants on the Outcomes of Allogeneic Hematopoietic Stem Cell Transplantation in Patients with Hematological Malignancies
Authors: Zhihui Li, Yongqiang Zhao, Yanzhi Song, Caiyan Zhang, Qinlong Zheng, Chunrong Tong, Tong Wu
Journal: Blood (2020)
Outcomes By Cardiac Stage in Newly Diagnosed AL Amyloidosis: Results from Andromeda
Authors: Monique C. Minnema, Angela Dispenzieri, Giampaolo Merlini, Raymond L. Comenzo, Efstathios Kastritis, Ashutosh D. Wechalekar, Martha Grogan, Ronald Witteles, Frederick L. Ruberg, Mathew S. Maurer, NamPhuong Tran, Xiang Qin, Sandra Y. Vasey, Brenda Tromp, Brendan M. Weiss, Jessica Vermeulen, Arnaud Jaccard
Journal: Blood (2020)
Abstract 256: Human <i>In Vitro</i> Model for Preclinical Evaluation of Irreversible Electroporation Devices Used for Cardiac Ablation
Authors: Maura Casciola, Tromondae K Feaster, Andrei G Pakhomov, Ksenia Blinova
Journal: Circulation Research (2020)

Irreversible Electroporation (IRE) has gained significant interest as an alternative treatment for atrial fibrillation (AF). Typically, to terminate AF, thermal energy is delivered to the arrhythmic tissue causing rare but serious damage to surrounding areas. IRE is a non-thermal approach that has the potential to be faster yet more targeted. We are developing a novel, species relevant, standard laboratory test to evaluate IRE cardiac ablation using human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) as a model. hiPSC-CMs were cultured in monolayers. Pulses were delivered by a AVOZ D7B generator through contact electrodes. Electric field (E) distribution maps were obtained with numerical simulations, Sim4Life v3.2. Electroporation (EP) was quantified by florescence with YO-PRO-1 10 minutes after exposure. hiPSC-CMs were exposed to a range of electrical parameters including 120 unipolar pulses of 5 μs duration, at 1 kHz frequency (Fig. 1). We found that the EP threshold was ~1.5 kV/cm in line with previous reports on single adult ventricular mouse cardiomyocytes (Semenov et al. 2018). The treated area increased with the voltage (V) applied. These data suggest that this assay can delineate the extent of EP. In this work we will vary different pulse parameters (durations, amplitude, shape, repetition rate and number) to evaluate their effects on different human cell lines anatomically adjacent to the heart. Fig.1: (A) Representation of the EP threshold determined by overlaying the E map (B) with a fluorescence image of pulse-treated hiPSC-CMs (C). To define the E threshold the distributions values obtained by modeling 1 V applied was scaled by the experimental V.
Transplantation for Congenital Sideroblastic Anaemia Is Feasible and Offers Outcomes Comparable to Other Transfusion Dependent Anaemias. a Joint Retrospective Study of the Paediatric Diseases and Severe Aplastic Anaemia Working Parties (PDWP/SAAWP) of EBMT
Authors: Josu de la Fuente, Dirk-Jan Eikema, Paul Bosman, Robert F Wynn, Miguel Díaz, Peter J Shaw, Amal Al-Seraihy, Muhlis Cem Ar, Mohamed Salaheldin Mohamed, Dominique Bron, Cristina Díaz de Heredia, Tobias Gedde-Dahl, Brenda Gibson, Rupert Handgretinger, Professor John G. Gribben, Tessa Kerre, Vassiliki Kitra-Roussou, Stig Lenhoff, Jose Maria Moraleda, Antonio Perez Martinez, John Moppett, Henrik Sengeloev, Abdelghani Tbakhi, Hendrik Veelken, Estelle Verburgh, Giuseppe Visani, Antonio Risitano, Carlo Dufour, Selim Corbacioglu, Regis Peffault De Latour
Journal: Blood (2020)
Mobilization of Auto-Stem Cells in the Thalassemia Major and Relationship with Iron Overload
Authors: Xiaoqin Feng, Yuelin He, Bindanda Mvuama Van Timothee, Jing Du, Chunfu Li, Qiujun Liu, Jianyun Wen, Xuan Liu, Rongfang Ding, Yongsheng Ruan, Xiaoting Liu, Peifen Zhang
Journal: Blood (2020)
Excellent Clinical and Pharmacokinetic Real-World Experience after Switching to Rurioctocog ALFA Pegol
Authors: Olga Benitez Hidalgo, Francisco Jose Lopez Jaime, María Fernanda Martínez García, Juan Carlos Juarez Gimenez, Eva Alvarez Martinez, Milagros Suito, Francesc Bosch, Mercedes Gironella Mesa
Journal: Blood (2020)
Pharmacological Induction of NOXA Sensitizes High-Risk B Cell Acute Lymphoblastic Leukemia Cells to Venetoclax
Authors: Klaudyna Fidyt, Julia Cyran, Agata Pastorczak, Nicholas T. Crump, Angelika Muchowicz, Agnieszka Goral, Krzysztof Domka, Lukasz Komorowski, Karolina Siudakowska, Agnieszka Graczyk-Jarzynka, Elzbieta Patkowska, Magdalena Winiarska, Jakub Golab, Thomas A. Milne, Malgorzata Firczuk
Journal: Blood (2020)
Investigational CD47-Blocker TTI-622 Shows Single-Agent Activity in Patients with Advanced Relapsed or Refractory Lymphoma: Update from the Ongoing First-in-Human Dose Escalation Study
Authors: Krish Patel, Radhakrishnan Ramchandren, Michael Maris, Alexander M. Lesokhin, Gottfried R. von Keudell, Bruce D. Cheson, Jeff Zonder, Erlene K. Seymour, Tina Catalano, Gloria H. Y. Lin, Bob Uger, Penka S. Petrova, Kathleen Roberge, Yaping Shou, Swami P. Iyer
Journal: Blood (2020)
OP0057 The patients’ knowledge about their disease: a french multicentric survey in ankylosing spondylitis (as)
Authors: P Claudepierre, RM Flipo, J Sibilia, JM Berthelot, P Goupille, S Cortinovis, X Chevalier, D Wendling
Journal: Annals of the Rheumatic Diseases (2001)

<h3>Background</h3> It is known that, in chronic diseases, patient education is a factor of good compliance and good disease management. An index was recently proposed to assess the level of knowledge in AS.<sup>1</sup> <h3>Objectives</h3> To assess the level of knowledge of a population of AS patients from different tertiary care centres in France, and to identify some factors linked with this level. <h3>Methods</h3> Study:multicentric cross-sectional study. No structured educational program was used in these centres at the time of the study. Patients: Inclusion criteria were New York criteria of AS and patients had to read French fluently. Data collected: demographic and sociocultural, disease characteristics, the index of knowledge in a French version (obtained from a double translation) giving a maximum score of 25. Statistical analysis: to seak a link between the knowledge score and collected factors, usual non parametric tests were used. <h3>Results</h3> The mean score of the 95 included patients was only 16.4 (S. D. 4.8). The percentages of correct answers were not equally distributed among the 4 groups or domains of questions. The duration of disease management in a tertiary care centre appeared to be positively linked to the level of knowledge (r = 0.28 with p = 0.01, for the global score on 25 points, and r = 0.26 with p = 0.02, for the number of good answers (on 14) per patient). It was also observed that the level of knowledge was better in females than males, and in patients who have previously read on the disease or who knew the association of AS patients. No interaction was found between the factor "sex" or the factor "duration of disease management in a tertiary care centre" and the other identified factors, suggesting that they are independent factors associated with the level of knowledge. Conversely, the 3 other factors, e.g., level of education in 2 classes, previous reading, knowing of the association, were statistically linked two by two. The other tested factors were not significantly linked with the level of knowledge: age of the patients, duration of the disease, characteristics of the disease (axial or peripheral, psoriasis, radiological severity). <h3>Conclusion</h3> The level of knowledge of these patients appears lower than this observed in the previous UK study, reinforcing the positive role of educational programs. These data will help to determine in which domains and in which patients the highest effort of education should be initially done. <h3>Reference</h3> Lubrano E, <i>et al</i>. Br J Rheumatol. 1998;37:437–41
Characterizing Specificities of Chronic Lymphoid Leukemia Harboring a <i>BCL2</i> rearrangement
Authors: Charles Herbaux, Imelda Raczkiewicz, Kamel Laribi, Loic Ysebaert, Agnes Daudignon, Sabine Tricot, Aurelie Caillault-Venet, Alain Jacques Delmer, Pascale Cornillet-Lefebvre, Cymbalista Florence, Virginie Eclache, Pierre Morel, Gauthier Decool, Morgane Nudel, Romain Guieze, Liam Hackett, Stephen Jun Fei Chong, Mary C Collins, Florence Nguyen-Khac, Fatiha Merabet, Jasmine Chauzeix, Stéphanie Struski, Jennifer R. Brown, Matthew S. Davids, Stéphanie Poulain
Journal: Blood (2020)
Phase 2 Trial of Pomalidomide, Ixazomib and Dexamethasone in Patients with Multiple Myeloma with Extramedullary Disease or Plasma Cell Leukemia
Authors: Francis K. Buadi, Martha Q. Lacy, Gabriela Perez, Liang Phuong-Dung, Ankit Kansagra, Thomas E. Witzig, Dania Kaehlyn Helgeson, Karen Woolley, Daniel Auclair, Hearn Jay Cho, Shaji K. Kumar
Journal: Blood (2020)
Transcriptional repression of SIRT3 potentiates mitochondrial aconitase activation to drive aggressive prostate cancer to the bone
Authors: Abhisha Sawant Dessai, Mayrel Palestino Dominguez, Uan-I Chen, John Hasper, Christian Prechtl, Cuijuan Yu, Eriko Katsuta, Tao Dai, Bokai Zhu, Sung Yun Jung, Nagireddy Putluri, Kazuaki Takabe, Xiang H-F Zhang, Bert W O'Malley, Subhamoy Dasgupta
Journal: Cancer Research (2020)

Metabolic dysregulation is a known hallmark of cancer progression, yet the oncogenic signals that promote metabolic adaptations to drive metastatic cancer remain unclear. Here, we show that transcriptional repression of mitochondrial deacetylase sirtuin 3 (
Selectin-targeting glycosaminoglycan-peptide conjugate limits neutrophil-mediated cardiac reperfusion injury
Authors: Tima Dehghani, Phung N Thai, Harkanwalpreet Sodhi, Lu Ren, Padmini Sirish, Carol E Nader, Valeriy Timofeyev, James L Overton, Xiaocen Li, Kit S Lam, Nipavan Chiamvimonvat, Alyssa Panitch
Journal: Cardiovascular Research (2020)

Abstract Aims One of the hallmarks of myocardial infarction (MI) is excessive inflammation. During an inflammatory insult, damaged endothelial cells shed their glycocalyx, a carbohydrate-rich layer on the cell surface which provides a regulatory interface to immune cell adhesion. Selectin-mediated neutrophilia occurs as a result of endothelial injury and inflammation. We recently designed a novel selectin-targeting glycocalyx mimetic (termed DS-IkL) capable of binding inflamed endothelial cells. This study examines the capacity of DS-IkL to limit neutrophil binding and platelet activation on inflamed endothelial cells, as well as the cardioprotective effects of DS-IkL after acute myocardial infarction. Methods and results In vitro, DS-IkL diminished neutrophil interactions with both recombinant selectin and inflamed endothelial cells, and limited platelet activation on inflamed endothelial cells. Our data demonstrated that DS-IkL localized to regions of vascular inflammation in vivo after 45 min of left anterior descending coronary artery ligation-induced MI. Further, findings from this study show DS-IkL treatment had short- and long-term cardioprotective effects after ischaemia/reperfusion of the left anterior descending coronary artery. Mice treated with DS-IkL immediately after ischaemia/reperfusion and 24 h later exhibited reduced neutrophil extravasation, macrophage accumulation, fibroblast and endothelial cell proliferation, and fibrosis compared to saline controls. Conclusions Our findings suggest that DS-IkL has great therapeutic potential after MI by limiting reperfusion injury induced by the immune response.
Response Kinetics and Factors Predicting Survival Among Patients with Core Binding Factor (CBF) Acute Myeloid Leukemia (AML)
Authors: Prajwal Boddu, Christopher Gurguis, Jorge E. Cortes, Mary Akosile, Farhad Ravandi, David Sanford, Mark Brandt, Rita Maduike, Tapan Kadia, Hagop M. Kantarjian, Gautam Borthakur
Journal: Blood (2017)
Genetically Defined Syngeneic Mouse Models of Ovarian Cancer as Tools for the Discovery of Combination Immunotherapy
Authors: Sonia Iyer, Shuang Zhang, Simge Yucel, Heiko Horn, Sean G. Smith, Ferenc Reinhardt, Esmee Hoefsmit, Bimarzhan Assatova, Julia Casado, Marie-Charlotte Meinsohn, M. Inmaculada Barrasa, George W. Bell, Fernando Pérez-Villatoro, Kaisa Huhtinen, Johanna Hynninen, Jaana Oikkonen, Pamoda M. Galhenage, Shailja Pathania, Paula T. Hammond, Benjamin G. Neel, Anniina Farkkila, David Pépin, Robert A. Weinberg
Journal: Cancer Discovery (2020)

Despite advances in immuno-oncology, the relationship between tumor genotypes and response to immunotherapy remains poorly understood, particularly in high-grade serous tubo-ovarian carcinomas (HGSC). We developed a series of mouse models that carry genotypes of human HGSCs and grow in syngeneic immunocompetent hosts to address this gap. We transformed murine-fallopian tube epithelial cells to phenocopy homologous recombination-deficient tumors through a combined loss of Trp53, Brca1, Pten, and Nf1 and overexpression of Myc and Trp53 R172H, which was contrasted with an identical model carrying wild-type Brca1. For homologous recombination-proficient tumors, we constructed genotypes combining loss of Trp53 and overexpression of Ccne1, Akt2, and Trp53 R172H, and driven by KRAS G12V or Brd4 or Smarca4 overexpression. These lines form tumors recapitulating human disease, including genotype-driven responses to treatment, and enabled us to identify follistatin as a driver of resistance to checkpoint inhibitors. These data provide proof of concept that our models can identify new immunotherapy targets in HGSC. SIGNIFICANCE: We engineered a panel of murine fallopian tube epithelial cells bearing mutations typical of HGSC and capable of forming tumors in syngeneic immunocompetent hosts. These models recapitulate tumor microenvironments and drug responses characteristic of human disease. In a Ccne1-overexpressing model, immune-checkpoint resistance was driven by follistatin.This article is highlighted in the In This Issue feature, p. 211.
Value of Coronary Artery Calcium Scanning in Association With the Net Benefit of Aspirin in Primary Prevention of Atherosclerotic Cardiovascular Disease
Authors: Ezimamaka Ajufo, Colby R. Ayers, Rebecca Vigen, Parag H. Joshi, Anand Rohatgi, James A. de Lemos, Amit Khera
Journal: JAMA Cardiology (2020)

Higher coronary artery calcium (CAC) identifies individuals at increased atherosclerotic cardiovascular disease (ASCVD) risk. Whether it can also identify individuals likely to derive net benefit from aspirin therapy is unclear.To examine the association between CAC, bleeding, and ASCVD and explore the net estimated effect of aspirin at different CAC thresholds.Prospective population-based cohort study of Dallas Heart Study participants, free from ASCVD and not taking aspirin at baseline. Data were analyzed between February 1, 2020, and July 15, 2020.Coronary artery calcium score in the following categories: 0, 1-99, and 100 or higher.Major bleeding and ASCVD events were identified from International Statistical Classification of Diseases and Related Health Problems, Ninth Revision codes. Meta-analysis-derived aspirin effect estimates were applied to observed ASCVD and bleeding rates to model the net effect of aspirin at different CAC thresholds.A total of 2191 participants (mean [SD], age 44 [9.1] years, 1247 women [57%], and 1039 black individuals [47%]) had 116 major bleeding and 123 ASCVD events over a median follow-up of 12.2 years. Higher CAC categories (CAC 1-99 and ≥100 vs CAC 0) were associated with both ASCVD and bleeding events (hazard ratio [HR], 1.6; 95% CI, 1.1-2.4; HR, 2.6; 95% CI, 1.5-4.3; HR, 4.8; 95% CI, 2.8-8.2; P < .001; HR, 5.3; 95% CI, 3.6-7.9; P < .001), but the association between CAC and bleeding was attenuated after multivariable adjustment. Applying meta-analysis estimates, irrespective of CAC, aspirin use was estimated to result in net harm in individuals at low (<5%) and intermediate (5%-20%) 10-year ASCVD risk and net benefit in those at high (≥20%) ASCVD risk. Among individuals at lower bleeding risk, a CAC score of at least 100 identified individuals who would experience net benefit, but only in those at borderline or higher (≥5%) 10-year ASCVD risk. In individuals at higher bleeding risk, there would be net harm from aspirin irrespective of CAC and ASCVD risk.Higher CAC is associated with both ASCVD and bleeding events, with a stronger association with ASCVD. A high CAC score identifies individuals estimated to derive net benefit from primary prevention aspirin therapy from those who would not, but only in the setting of lower bleeding risk and estimated ASCVD risk that is not low.
Inflammatory Myofibroblastic Tumor of the Liver Mimicking Metastasis on 18F-FDG PET/CT
Authors: Roy A. Raad, Laura Haddad, Teddy Jabbour, Ziad El-Rassi
Journal: Clinical Nuclear Medicine (2020)

A 62-year-old woman with history of stage III endometrial cancer was found to have a small enhancing liver lesion on follow-up abdominal CT and MRI, suspicious for metastasis. Subsequent F-FDG PET/CT was performed, demonstrating intense uptake within the liver lesion, consistent with metastasis. Subsequently, laparoscopic wedge liver lesion resection was performed. Histopathologic examination confirmed the diagnosis of inflammatory myofibroblastic tumor (IMT) with predominant inflammatory cells. Although solitary liver IMTs are rare occurrences with imaging features overlapping with malignancy, IMT should be considered in the differential diagnosis of suspicious liver lesions, especially when the pattern of spread is unusual.
Why asthma might surprisingly protect against poor outcomes in COVID-19
Authors: Hugo Farne, Aran Singanayagam
Journal: European Respiratory Journal (2020)

<b>Asthma may protect against poor outcomes in COVID-19 due to several possible mechanisms, including altered viral entry receptor expression, inhaled corticosteroid use, chronic inflammation, reduced exposure due to shielding and/or mucus hypersecretion</b>https://bit.ly/3eiXOPP
Abstract 15639: Increased Capacity to Utilize Ketone Bodies in Patients With Heart Failure: Transmyocardial Gradient Study After Oral Administration of 3-hydroxybutyrate Ester
Authors: Luca Monzo, Kamil Sedlacek, Katerina Hromanikova, Lucie Tomanova, Petr Stavek, Antonin Jabor, Janka Franekova, Ivana Kuklova, Francesco Barillà, Carlo Gaudio, Josef Kautzner, Vojtech Melenovsky
Journal: Circulation (2019)

Background: Heart failure (HF) is characterised by diminished oxidation of FFA and enhanced utilisation of alternative substrates, including ketone bodies (mainly 3-hydroxybutyrate, 3-OHB). Advance...
When taking a step back is a veritable leap forward. Reversing decades of arthroscopy for managing joint pain: five reasons that could explain declining rates of common arthroscopic surgeries
Authors: Clare L Ardern, Teemu Paatela, Ville Mattila, Simo Taimela, Teppo L N Järvinen
Journal: British Journal of Sports Medicine (2020)

Arthroscopy heralded an age of surgery-as-frontline-treatment for the painful joints of middle-aged and older people. By the end of the 20th century, knee, shoulder, hip and ankle arthroscopies were some of the most frequently performed surgeries in developed countries.1 Questions were first raised about the efficacy of knee arthroscopy for advanced osteoarthritis in 2002, when Professor Moseley and colleagues published their landmark placebo-controlled trial.2 Similarly, rigorous trials followed, each questioning the efficacy of arthroscopic partial meniscectomy and subacromial decompression—the two most common arthroscopic surgeries.

Despite compelling, high-quality evidence, why did the number of arthroscopies for degenerative conditions continue to rise in the first decade of the 21st century?1 The most obvious change to clinical practice was that arthroscopies were increasingly billed using different procedure (billing) codes.3 Our Finnish colleagues investigated the trends in various arthroscopic surgeries in Finland between 1997 and 2016 and found that the incidence of knee and shoulder arthroscopy peaked in 2006 and 2007, respectively, then steadily declined.1 The rates of wrist, elbow and hip arthroscopies also declined after their 2014 peak.1 Although the rates have declined in some countries, arthroscopies for patients with degenerative joint diseases remain some of the most commonly performed surgeries around the world.

The Finnish Centre for Evidence-Based Orthopaedics (FICEBO, www.ficebo.com) has been studying arthroscopic surgery for …
FRI0162 Clinical features of a set of patients with anti-neutrophil cytoplasmic antibodies
Authors: EG Tomero Muriel, G García-Melcón, R Velayos, AM Ortiz, I González-Alvaro
Journal: Annals of the Rheumatic Diseases (2001)

<h3>Background</h3> <h3>Objectives</h3> To analyse characteristics and clinical features of patients with positive antineutrophil cytoplasmic antibodies (ANCA). <h3>Methods</h3> Retrospectively we reviewed 71 patients tested for the presence of ANCA using indirect inmunofluorescence (IFF; ptterns pANCA and cANCA) and/or antimieloperoxidase ELISA (aMPO) from 1993 to 1999. The variables observed were age, sex, origin department, clinical manifestations, final diagnosis, disease evolution and treatment. <h3>Results</h3> Mean age was 60.2 years (16–97) being women 63.3%. cANCA was positively tested in 42% of patients and pANCA in 58%(59% aMPO). The distribution among departments was: Internal Medicine 22.5%, Rheumatology 22.5%, Nephrology 16.5%, Pneumology 14.9%, Gastroenterology 13.5% and Dermatology 12%. 28 patients suffered from lung affections, 27 had kidney affections (30% rapidly progressing glomerulonephritis), 18 skin involvement, 19 musculoesqueletal manifestations, 5 hearts affections, 3 neuropathies and 2 eye involvement. Diagnosis were: 5 Wegeners Granulomatosis, 5 Microscopic polyangiitis, 8 pANCA associated vasculities, 4 leucocytoclastic vasculitis, 7 rheumatoid arthritis, 7 inflammatory bowel diseases, 6 systemic inmunolopathologycal diseases, 21 not autoimmune disorders and 8 without final diagnosis. Progress: 42% suffered from periodical relapses, 28% without clinical manifestations, 21% died. 53% of death were directly caused by ANCA associated vasculitis, 26% were due to concomitant infections and the rest were caused by non related complications. <h3>Conclusion</h3> 1. Despitethe high percentage of detected cANCA by IIF only Wegeners granulomatosis were diagnosed, so the use of ELISA antiproteinase-3 must be used. 2. It was observed a high mortality rate in patients in patients with positive ANCA.