tbuckley/cabot-search huggingface dataset:
Second series by the Italian Association of Pediatric Hematology and Oncology of children and adolescents with intracranial ependymoma: an integrated molecular and clinical characterization with a long-term follow-up
Authors: Maura Massimino, Francesco Barretta, Piergiorgio Modena, Hendrik Witt, Simone Minasi, Stefan M Pfister, Kristian W Pajtler, Manila Antonelli, Lorenza Gandola, Maria Luisa Garrè, Daniele Bertin, Angela Mastronuzzi, Maurizio Mascarin, Lucia Quaglietta, Elisabetta Viscardi, Iacopo Sardi, Antonio Ruggiero, Bianca Pollo, Annamaria Buccoliero, Luna Boschetti, Elisabetta Schiavello, Luisa Chiapparini, Alessandra Erbetta, Isabella Morra, Marco Gessi, Vittoria Donofrio, Carlo Patriarca, Felice Giangaspero, Pascal Johann, Francesca Romana Buttarelli
Journal: Neuro-Oncology (2020)

Abstract Background A prospective 2002–2014 study stratified 160 patients by resection extent and histological grade, reporting results in 2016. We re-analyzed the series after a median of 119 months, adding retrospectively patients’ molecular features. Methods Follow-up of all patients was updated. DNA copy number analysis and gene-fusion detection could be completed for 94/160 patients, methylation classification for 68. Results Progression-free survival (PFS) and overall survival (OS) at 5/10 years were 66/58%, and 80/73%. Ten patients had late relapses (range 66–126 mo), surviving after relapse no longer than those relapsing earlier (0–5 y). On multivariable analysis a better PFS was associated with grade II tumor and complete surgery at diagnosis and/or at radiotherapy; female sex and complete resection showed a positive association with OS. Posterior fossa (PF) tumors scoring ≥0.80 on DNA methylation analysis were classified as PFA (n = 41) and PFB (n = 9). PFB patients had better PFS and OS. Eighteen/32 supratentorial tumors were classified as RELA, and 3 as other molecular entities (anaplastic PXA, LGG MYB, HGNET). RELA had no prognostic impact. Patients with 1q gain or cyclin-dependent kinase inhibitor 2A (CDKN2A) loss had worse outcomes, included significantly more patients &amp;gt;3 years old (P = 0.050) and cases of dissemination at relapse (P = 0.007). Conclusions Previously described prognostic factors were confirmed at 10-year follow-up. Late relapses occurred in 6.2% of patients. Specific molecular features may affect outcome: PFB patients had a very good prognosis; 1q gain and CDKN2A loss were associated with dissemination. To draw reliable conclusions, modern ependymoma trials need to combine diagnostics with molecular risk stratification and long-term follow-up.
Venetoclax, Lenalidomide and Rituximab for Patients with Relapsed or Refractory Mantle Cell Lymphoma - Data from the Nordic Lymphoma Group NLG-MCL7 (VALERIA) Phase I Trial: Stopping Treatment in Molecular Remission Is Feasible
Authors: Mats Jerkeman, Arne Kolstad, Carsten Utoft Niemann, Kirsten Groenbaek, Martin Hutchings, Annika Pasanen, Sara Ekberg, Karin Fahl Wader, Ingrid Glimelius
Journal: Blood (2020)
A Systematic Review of Phase III Clinical Trials of Daratumumab Addition to Standard Care Regimen for Newly Diagnosed Multiple Myeloma
Authors: Zahoor Ahmed, Karun Neupane, Rabia Ashraf, Amna Khan, Moazzam Shahzad, Arafat Ali Farooqui, Muhammad Salman Faisal, Shaha Nabeel, Abdur Rehman Jamil, Faiz Anwer
Journal: Blood (2020)
Association of Serum Neurofilament Light Levels With Long-term Brain Atrophy in Patients With a First Multiple Sclerosis Episode
Authors: Tatiana Plavina, Carol M. Singh, Dipen Sangurdekar, Carl de Moor, Bob Engle, Arie Gafson, Jaya Goyal, Elizabeth Fisher, Suzanne Szak, Revere P. Kinkel, Alfred W. Sandrock, Ray Su, Bernd C. Kieseier, Richard A. Rudick
Journal: JAMA Network Open (2020)

Data are needed on the potential long-term prognostic association of serum neurofilament light in multiple sclerosis (MS).To evaluate serum neurofilament light as a biomarker associated with long-term disease outcomes in clinically isolated syndrome.This post hoc cohort study used data from the Controlled High-Risk Avonex Multiple Sclerosis Prevention Study, a 36-month, multicenter, placebo-controlled interferon β-1a randomized clinical trial conducted from April 1996 to March 2000, and its long-term (5- and 10-year) extension study from February 2001 to March 2009. Participants included individuals with a symptomatic initial demyelinating event and brain magnetic resonance imaging (MRI) lesions suggestive of MS. Data were analyzed from April 2017 through 2019.The variable of interest was naturally occurring serum neurofilament light concentration.Gadolinium-enhancing (Gd+) lesion number, T2 lesion volume, and brain parenchymal fraction, a measure of brain atrophy were measured at baseline and 5 and 10 years. Multivariate regression models evaluated whether age, sex, and baseline covariates, including serum neurofilament light, brain parenchymal fraction, Expanded Disability Status Scale, Gd+ lesion count, and T2 lesion volume, were associated with brain parenchymal fraction changes over 5 and 10 years.Among 308 included participants (mean [SD] age, 33.2 [7.6] years; 234 [76.0%] women), baseline serum neurofilament light concentrations were associated with Gd+ lesions (Spearman r = 0.41; P < .001) and T2 lesion volume (Spearman r = 0.42; P < .001). Among covariates for brain parenchymal fraction change, serum neurofilament light concentration had the greatest correlation with change in brain parenchymal fraction at 5 years (Spearman r = -0.38; P < .001) and was the only variable associated with brain parenchymal fraction at 10 years (Spearman r = -0.45; P < .001). Participants in the highest vs lowest baseline serum neurofilament light tertiles showed brain parenchymal fraction reduction at 5 years (-1.83% [95% CI, -1.49% to -2.18%] vs -0.95% [95% CI, -0.78% to -1.12%]; P < .001) and 10 years (-3.54% [95% CI, -2.90% to -4.17%] vs -1.90% [95% CI, -1.43% to -2.37%]; P < .001). At 5 years, 6 of 45 participants (13.3%) in the highest neurofilament tertile and 2 of 52 participants (3.8%) in the lowest neurofilament tertile achieved an Expanded Disability Status Scale score of 3.5 or greater.This cohort study found that higher baseline serum neurofilament light levels were associated with increased brain atrophy over 5 and 10 years. These findings suggest that serum neurofilament light could be a biomarker associated with disease severity stratification in early MS and may help to guide intervention.
Allogeneic Transplant Conditioning Regimens for Patients With Non-Hodgkin Lymphoma
Authors: Koen van Besien, Nina Orfali, Tsiporah Shore
Journal: JAMA Oncology (2020)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Oncology HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Oncology journal
OP0104 Lupus nephritis: contrasting clinical outcome during the last decade
Authors: C Fiehn, Y Hajjar, R Waldherr, K Andrassy
Journal: Annals of the Rheumatic Diseases (2001)

<h3>Background</h3> According to Cameron (1999) 5-year renal survival of WHO class IV lupus nephritis (LN) after diagnosis and treatment is now 82%. It is claimed that when terminal renal failure (ERF) supervenes, it is within the first decade of follow-up. <h3>Objectives</h3> As our recent experience is different, we evaluated the long-term renal outcome in patients with lupus nephritis treated in our institution in the last decade. <h3>Methods</h3> During the last 10 years we followed 44 caucasians (35 females, 9 males, median age 40 years, range 18–73) with biopsy proven LN (WHO class II 5, III 2, IV 31, V 5, and VI 1). 15/44 patients had hypertension, 37/44 patients anti-DNA-ab (Farr test) (median 18, range 8–1493 U/ml), 41/44 positive ANA (median 1280, range 160–40960 titer), 44/44 proteinuria (20/44 with nephrotic proteinuria (NS) and 37/44 hematuria. Median creatinine on admission was 0.9 mg/dl, range (0.3–4.0), median haemoglobin 11.7 g/dl (range 8.2–14.3). All WHO classes were treated with glucocorticoids (1 mg/kg/day for 8–12 weeks, tapering off according to the clinical situation). WHO class IV was initially treated with 3 methylprednisolone boli (then 1 mg/kg/day) and cyclophosphamide (CP) (Austin scheme), sometimes supported by cyclosporine, azathioprine or mycophenolate mofetil. Hypertension and/or NS were treated with ACE inhibitors, hyperlipidemia with statins. <h3>Results</h3> No patient died or attained ERF. Median creatinine was 1.1 mg/dl (range 0.5–5.4) after an median observation period of 39 month (range 1- 128). Impaired but stable renal function was observed in 12/44 patients (classes III 2, IV 9, V 1). Progression to renal failure was observed in 1 patient with uncontrolled hypertension. From renal histology only classes III and IV had adverse outcomes. <h3>Conclusion</h3> We attribute improved outcome of LN in the last decade to earlier detection and therapy of LN and treatment of LN-flares with CP and steroid boli. Moreover better medical management particularly the consequent treatment of hypertension with ACE inhibitors may contribute to a favourable prognosis. <h3>References</h3> Cameron JS. Lupus nephritis. J Am Soc Nephrol. 1999;10:413–35 Austin HA, Boumpas DT, Vaughan EM, Balow JE. High-risk features of lupus nephritis: importance of race and histological factors in 166 patients. Nephrol Dial Transplant. 1995;10:1620–8 Moroni G, Quaglini S, Maccario M, Banfi G, Ponticelli C. Nephritic flares are predictors of bad long-term renal outcome in lupus nephritis. Kidney Int. 1996;50:2047–53 Ward MM, Studenski S. Clinical prognostic factors in lupus nephritis. The importance of hypertension and smoking. Arch Intern Med. 1992;152:2082–8
Immune Cell Profiles in Patients Treated with Lenalidomide and Alternate Day Prednisolone Maintenance Post Upfront ASCT for Multiple Myeloma (LEOPARD Trial)
Authors: Anna Kalff, Sam Norton, Tiffany Khong, Malarmathy Ramachandran, Mary H. Young, William Pierceall, Anjan Thakurta, Udo Oppermann, Manman Guo, John Reynolds, Andrew Spencer
Journal: Blood (2020)
Re: Beatriz Goulao, Sonya Carnell, Jing Shen, et al. Surgical Treatment for Recurrent Bulbar Urethral Stricture: A Randomised Open-label Superiority Trial of Open Urethroplasty Versus Endoscopic Urethrotomy (the OPEN Trial). Eur Urol 2020;78:572–80
Authors: Amit Bansal, Ruchir Maheshwari, Anant Kumar
Journal: European Urology (2020)
BCL-2 Antagonist ABT-199 Combined with Complex I Inhibitor IACS-010759 Blocks Mitochondrial Respiration and Facilitates Anti-Leukemia Efficacy in Pre-Clinical AML Models
Authors: Qi Zhang, Lina Han, Tianyu Cai, Vinitha Mary Kuruvilla, Antonio Cavazos, Helen Ma, Venkata Lokesh Battula, Natalia Baran, Karine Harutyunyan, Michael Andreeff, Yoko Tabe, Joseph R Marszalek, Marina Konopleva
Journal: Blood (2017)
Robust Efficacy Signals in Elderly AML Patients Treated with Iadademstat in Combination with Azacitidine (ALICE Phase IIa Trial)
Authors: Olga Salamero, Tim C.P Somervaille, Antonieta Molero, Evelyn Acuña, Ana Perez, Isabel Cano, Rebeca Rodríguez-Veiga, Sonia Gutierrez, Roger Bullock, Carlos Buesa, Francesc Bosch, Pau Montesinos
Journal: Blood (2020)
Characterization of Synergistic Selinexor Combinations of Dexamethasone, Pomalidomide, Elotuzumab and Daratumumab in Primary MM Samples Ex Vivo
Authors: Kenneth H. Shain, Rafael Renatino-Canevarolo, Mark B. Meads, Praneeth Reddy Sudalagunta, Maria D Coelho Siqueira Silva, Christopher Cubitt, Gabriel De Avila, Raghunandan Reddy Alugubelli, Amit Kulkarni, Qi Zhang, Oliver Hampton, Constantine Logothetis, Christian Argueta, Yosef Landesman, Ariosto Siqueira Silva
Journal: Blood (2020)
A Phase II Study of Isatuximab (SAR650984) (NSC-795145) for Patients with Previously Treated AL Amyloidosis (SWOG S1702; NCT#03499808)
Authors: Terri L. Parker, Adam Rosenthal, Vaishali Sanchorawala, Heather J Landau, Erica Campagnaro, Prashant Kapoor, Natalia Neparidze, Patrick Hagen, Shayna Sarosiek, Emma C. Scott, Antje Hoering, Brian G.M. Durie, Saad Z. Usmani, Robert Z. Orlowski
Journal: Blood (2020)
Infectious Complications of BCMA-Targeted and CD19-Targeted Chimeric Antigen Receptor T-Cell Immunotherapy
Authors: Swetha Kambhampati, Bita Fakhri, Ying Sheng, Chiung-Yu Huang, Sophia Byslma, Kelsey Natsuhara, Dhruba Biswas, Mimi Lo, Weiyun Z. Ai, Lawrence D Kaplan, Thomas Martin, Jeffrey L. Wolf, Nina Shah, Charalambos Andreadis, Sandy W. Wong
Journal: Blood (2020)
Abstract 412: DJ-1 Deficiency Impairs Post-Ischemic Cardiac Fatty Acid Oxidation
Authors: Kevin M Casin, Yvanna Pantner, Yuuki Shimizu, Rohini Polavarapu, Lih-Shen Chin, Lian Li, John W Calvert
Journal: Circulation Research (2020)

Background: DJ-1 is a cytoprotective protein implicated in many cellular processes. We have recently shown that DJ-1 plays a protective role in the setting of acute myocardial ischemia-reperfusion injury and heart failure. However, specific mechanisms of action remain unknown. Here, we sought to determine if DJ-1 maintains fatty acid oxidation following myocardial I/R injury via the regulation of PPARalpha transcriptional activity. Methods and Results: WT and DJ-1 KO hearts were subjected to 45 minutes of ischemia via coronary artery ligation followed by up to 72 hours of reperfusion. Initial studies revealed that PPARalpha activity (PPARalpha Transcription Factor Assay Kit; abcam) was lower in DJ-1 KO hearts under both basal (sham) and I/R conditions. Next, we performed an analysis of PPAR genes using a qPCR array (Mouse PPAR Targets RT2 Profiler PCR Array from Qiagen) that profiled the expression of 84 key genes involved in PPAR activation and response. Marked differences in genes involved in FA metabolism were evident in the hearts of DJ-1 KO mice. Further studies using qPCR validated a significant decrease in the expression of acsl1 (protein ACSL1), acsl3, acsl5, cpt1b (protein CPT1), slc25a20 (protein CACT), and cpt2. The protein expression of ACSL1, CPT1, and CACT were also decreased in the hearts of DJ-1 KO mice following I/R injury. These changes were accompanied by higher cardiac lipid content and depressed mitochondrial fatty-acid β-oxidation and ATP synthesis. Conclusion: These data demonstrate that DJ-1 plays an essential role in regulating post-I/R cardiac FAO. Future studies will garner mechanistic insight for the impaired function of PPARalpha with the loss of DJ-1.
Categorized Hematologic Response to Pegcetacoplan Versus Eculizumab in Patients with Paroxysmal Nocturnal Hemoglobinuria: Post Hoc Analysis of Data from a Phase 3 Randomized Trial (PEGASUS)
Authors: Antonio Risitano, Ilene C. Weitz, Carlos M. de Castro, Jean-Jacques Kiladjian, Morag Griffin, Hisakazu Nishimori, Mohamed Hamdani, Temitayo Ajayi, Scott B. Baver, Regis Peffault De Latour
Journal: Blood (2020)
SARS-CoV-2 in the bile of a patient with COVID-19-associated gallbladder disease
Authors: Yusheng Liao, Bangmao Wang, Jian Wang, Juan Shu, Weilai Zhou, Heng Zhang
Journal: Endoscopy (2020)

See also: Gut feeling from Wuhan: advice for gastrointestinal endoscopy performed during the COVID-19 outbreakEndoscopy 2020; 52(07): E261-E262DOI: 10.1055/a-1158-9016
Schistosoma mansoni Eggs Modulate the Timing of Granuloma Formation to Promote Transmission
Authors: Kevin K. Takaki, Gabriel Rinaldi, Matthew Berriman, Antonio J. Pagán, Lalita Ramakrishnan
Journal: Cell Host & Microbe (2020)

Schistosome eggs provoke the formation of granulomas, organized immune aggregates, around them. For the host, the granulomatous response can be both protective and pathological. Granulomas are also postulated to facilitate egg extrusion through the gut lumen, a necessary step for parasite transmission. We used zebrafish larvae to visualize the granulomatous response to Schistosomamansoni eggs and inert egg-sized beads. Mature eggs rapidly recruit macrophages, which form granulomas within days. Beads also induce granulomas rapidly, through a foreign body response. Strikingly, immature eggs do not recruit macrophages, revealing that the eggshell is immunologically inert. Our findings suggest that the eggshell inhibits foreign body granuloma formation long enough for the miracidium to mature. Then parasite antigens secreted through the eggshell trigger granulomas that facilitate egg extrusion into the environment. In support of this model, we find that only mature S. mansoni eggs are shed into the feces of mice and humans.
Outcomes of GCSF and Pre-Emptive Plerixafor Based Peripheral Blood Stem Cell Mobilization Among Patients with Multiple Myeloma-Experience from Newly Established Transplant Unit in Eastern Mediterranean Area
Authors: Imran K Tailor, Nawal AlShehry, Nurah F Alenezi, Belal Albtoosh, Ammar H. Alsughayir, Mohammed Marei, Syed Y Altaf, Syed Ziauddin A Zaidi, Imran A Pukhta, Rabab Alhazeem, Mansour Alfayez, Ibraheem Motabi
Journal: Blood (2020)
Inhibitors targeting Bruton’s tyrosine kinase in cancers: drug development advances
Authors: Tingyu Wen, Jinsong Wang, Yuankai Shi, Haili Qian, Peng Liu
Journal: Leukemia (2020)

Abstract Bruton’s tyrosine kinase (BTK) inhibitor is a promising novel agent that has potential efficiency in B-cell malignancies. It took approximately 20 years from target discovery to new drug approval. The first-in-class drug ibrutinib creates possibilities for an era of chemotherapy-free management of B-cell malignancies, and it is so popular that gross sales have rapidly grown to more than 230 billion dollars in just 6 years, with annual sales exceeding 80 billion dollars; it also became one of the five top-selling medicines in the world. Numerous clinical trials of BTK inhibitors in cancers were initiated in the last decade, and ~73 trials were intensively announced or updated with extended follow-up data in the most recent 3 years. In this review, we summarized the significant milestones in the preclinical discovery and clinical development of BTK inhibitors to better understand the clinical and commercial potential as well as the directions being taken. Furthermore, it also contributes impactful lessons regarding the discovery and development of other novel therapies.
An Optimized Crovalimab Dose and Regimen Reduced the Formation of Drug-Target-Drug Complexes in Patients with Paroxysmal Nocturnal Hemoglobinuria from the Phase I/II COMPOSER Trial
Authors: Jun-ichi Nishimura, Antoine Soubret, Simon Buatois, Jean-Eric Charoin, Sasha Sreckovic, Christoph Bucher, Jules Hernández-Sánchez, Gregor Jordan, Julia Ramos, Noriko Arase, Masaki Hotta, Yoshitaka Isaka, Yoshikazu Ito, Yuzuru Kanakura, Jin Seok Kim, Taroh Kinoshita, Eiichi Morii, Jens Panse, Regis Peffault De Latour, Alexander Röth, Hubert Schrezenmeier, Simona Sica, Hiroyuki Takamori, Yasutaka Ueda, Sung-Soo Yoon, Ido Paz-Priel, Alexandre Sostelly
Journal: Blood (2020)
Therapeutic Effect of a Traditional Chinese Medicine Compound Mixture Compound Kushen Injection in Treating Multiple Myeloma
Authors: Hongqiang Jiang, Honglei Tu, Yanxia Jin, Xianjin Wu, Ziyi Luo, Yanling Chen, Dongdong Zhang, Balu Wu, Yongchang Wei, Yi Yang, Fuling Zhou
Journal: Blood (2020)
Cultural and environmental associations with body image, diet and well-being in NCAA DI female distance runners: a qualitative analysis
Authors: Traci Lyn Carson, Troy Tournat, Kendrin Sonneville, Ronald F Zernicke, Carrie Karvonen-Gutierrez
Journal: British Journal of Sports Medicine (2020)

Objective We aimed to describe current and former National Collegiate Athletic Association (NCAA) Division One (DI) female distance runners’ experiences of perceived norms of body image and disordered eating in their sport, as well as the emergence and influence of coach–athlete power dynamics. This manuscript reports a qualitative research study (consisting of interviews and thematic analysis) of women athletes’ experiences of perceived norms of body image and disordered eating in their sport. We also report athletes’ experiences of coach–athlete power dynamics. Methods The study sample included 29 current and former female NCAA DI female distance runners, defined as competing in 800-metre distance or greater. Interviews were conducted, audio-recorded and hand transcribed. A thematic analysis was performed and presented. Results Two major themes emerged: (1) sport body ideals and body image norms and myths that exist in the sport, and (2) the power dynamic between athletes and coaches. It is not clear whether sport body ideals and culture of running influences coaching culture, or whether the coaches—who maintain positions of power in the sport—perpetuate the culture. These themes likely feed into each other and reinforce the existing and dominant mentalities of the sport. Conclusion Sport body image ideals and the power dynamic between coach and athlete may contribute to female athlete’s risk of disordered eating and body image disturbance. We call for the NCAA and athletic departments to develop and implement prevention and intervention programmes to prevent eating and body image disorders in this high-risk population.
Efficacy and Safety of Concomitant Use of Ravulizumab and IST in Patients with Paroxysmal Nocturnal Hemoglobinuria up to 52 Weeks
Authors: Hubert Schrezenmeier, Jong Wook Lee, Anita Hill, Vadim V Ptushkin, Viviani Pessoa, Rosario Notaro, Jimmy Wang, Masayo Ogawa, Shinichiro Okamoto, Lily LL Wong, Regis Peffault De Latour, Austin Kulasekararaj
Journal: Blood (2020)
Sequential Treatments in Chronic Phase Chronic Myeloid Leukemia (CML) Patients without Optimal Response after Frontline Nilotinib or Dasatinib: An Italian CML Campus Study
Authors: Gabriele Gugliotta, Mario Annunziata, Isabella Capodanno, Davide Rapezzi, Immacolata Attolico, Iolanda Donatella Vincelli, Mario Tiribelli, Alessandra Malato, Michele Pizzuti, Vincenzo Accurso, Massimiliano Bonifacio, Sara Galimberti, Giuseppina Loglisci, Elisabetta Abruzzese, Monica Bocchia, Antonella Gozzini, Micaela Bergamaschi, Maria Cristina Miggiano, Agostino Tafuri, Grazia Sanpaolo, Gianni Binotto, Fausto Castagnetti, Giorgina Specchia, Francesco Di Raimondo, Michele Cavo, Gianantonio Rosti, Robin Foà, Giuseppe Saglio, Massimo Breccia, Fabio Stagno
Journal: Blood (2020)