tbuckley/cabot-search huggingface dataset:
HGG-06. REMARKABLE RESPONSE TO BRAF INHIBITOR IN AN INFANT WITH DISSEMINATED DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR (DLGNT)
Authors: Le Le Aung
Journal: Neuro-Oncology (2020)

Abstract INTRODUCTION Diffuse Leptomeningeal Glioneuronal Tumor (DLGNT) are rare CNS tumors and in infants, they can be lethal. There are several anecdotal reports in infants with low grade gliomas (LGG) with treated with BRAF inhibitors. METHODS A six-month old baby girl presented with a 2-week history of absent visual contact and vomiting. Imaging revealed a large 4.7 X 4.2 X 2.8 cm suprasellar charismatic region mass and multiple small extra-axial plaques in spinal canal. The child developed significant ascites post VP shunt requiring shunt externalization, extensive protein infusion support and hospitalization for six weeks. Immunohisto-chemical staining revealed Olig-2 and S-100, GFAP and synaptophysin positive. EMA showed patchy cytroplasmic reactivity in stromal cells and CD99 showed diffuse reactivity in stromal and lesional cells. INI-1, IDH-1, and CD117 were negative. Ki-67 proliferation index was 8–10%. PCR for BRAF V600E/E2/D was detected and KIAA1549-BRAF fusion as negative. This was confirmed by Genome Wide Next Generation Sequencing. While waiting for GNS testing results, the baby received one dose of Vinblastine. However, within seven days of initiating Debrafenib, significant clinical and radiological responses were observed. CONCLUSION The baby continues safely on Debrafenib with continued dramatic radiological response. This suggest that there may be a role in early initiation of targeted therapy such as BRAF inhibitors rather than giving standard chemotherapy such as Vinblastine or Carboplatin-Vincristine in extremely ill infants with low grade gliomas.
Amplifying Access to Hearing Aids
Authors: Jennifer Claytor, Kenneth E. Covinsky
Journal: JAMA Internal Medicine (2020)

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NIMG-15. EVALUATING FLUCTUATING ENHANCEMENT IN OLIGODENDROGLIOMAS ON MAGNETIC RESONANCE IMAGING
Authors: Marissa Barbaro, Peter Pan, David Pisapia, Theodore Schwartz, Rohan Ramakrishna, Jonathan Knisely, Howard Fine, Gloria Chiang, Rajiv Magge
Journal: Neuro-Oncology (2020)

Abstract OBJECTIVE To identify and characterize patterns of fluctuating contrast enhancement on magnetic resonance imaging (MRI) in patients with oligodendrogliomas. INTRODUCTION Gliomas, particularly oligodendrogliomas, can exhibit fluctuating enhancement (FE) on MRI that can make it difficult to differentiate between treatment effect and active tumor. METHODS We are conducting a single-center retrospective review of clinical and radiographic data for patients with oligodendrogliomas treated at Weill Cornell Medicine (WCM) from 2/2000-5/2018. We have identified patients with FE on MRI and tracked lesions &amp;gt; 5mm in at least one dimension to the resolution of the lesion or last available MRI. We have recorded time from initial diagnosis to development of FE, time from radiation to development of FE, and time from development to resolution of FE as well as molecular characteristics of each tumor. RESULTS A total of 122 patients with oligodendrogliomas were identified. Thus far, fluctuating enhancement has been identified in 11 patients (5 men, 6 women) with 38 total fluctuating lesions. Isocitrate dehydrogenase-1 (IDH-1) mutation was present in 5 tumors, and 1p/19q co-deletion was present in 6. Mean time from initial diagnosis to development of FE was 44.6 months. In patients who developed FE after radiation, mean time from radiation to development of FE was 35.0 months. Twenty-seven lesions resolved, and mean time from onset to resolution of FE was 5.6 months, while mean time from start of radiation to resolution of FE was 41.0 months. Additionally, we will perform perfusion analysis on lesions &amp;gt; 5mm and identify patients who underwent surgical biopsy of FE with pathologic diagnosis. CONCLUSIONS FE has been identified in 11 patients thus far. We are expanding our analysis to identify a larger cohort of patients with FE. Characterizing patterns of FE may aid clinicians in differentiating FE due to treatment effect from active tumor.
Stepping Outside of the Heart: Using Nontraditional Patient Characteristics to Understand and Improve Outcomes**Editorials published in the Journal of the American College of Cardiologyreflect the views of the authors and do not necessarily represent the views of JACCor the American College of Cardiology.
Authors: Lakshmi Venkitachalam, John A. Spertus
Journal: Journal of the American College of Cardiology (2010)
International consensus on a standard set of outcome measures for child and youth anxiety, depression, obsessive-compulsive disorder, and post-traumatic stress disorder
Authors: Karolin R Krause, Sophie Chung, Abiodun O Adewuya, Anne Marie Albano, Rochelle Babins-Wagner, Laura Birkinshaw, Peter Brann, Cathy Creswell, Kathleen Delaney, Bruno Falissard, Christopher B Forrest, Jennifer L Hudson, Shin-ichi Ishikawa, Meghna Khatwani, Christian Kieling, Judi Krause, Kanika Malik, Vania Martínez, Faraz Mughal, Thomas H Ollendick, Say How Ong, George C Patton, Ulrike Ravens-Sieberer, Peter Szatmari, Evie Thomas, Lucas Walters, Bryan Young, Yue Zhao, Miranda Wolpert
Journal: The Lancet Psychiatry (2020)
Articulating the effect of food systems innovation on the Sustainable Development Goals
Authors: Mario Herrero, Philip K Thornton, Daniel Mason-D'Croz, Jeda Palmer, Benjamin L Bodirsky, Prajal Pradhan, Christopher B Barrett, Tim G Benton, Andrew Hall, Ilje Pikaar, Jessica R Bogard, Graham D Bonnett, Brett A Bryan, Bruce M Campbell, Svend Christensen, Michael Clark, Jessica Fanzo, Cecile M Godde, Andy Jarvis, Ana Maria Loboguerrero, Alexander Mathys, C Lynne McIntyre, Rosamond L Naylor, Rebecca Nelson, Michael Obersteiner, Alejandro Parodi, Alexander Popp, Katie Ricketts, Pete Smith, Hugo Valin, Sonja J Vermeulen, Joost Vervoort, Mark van Wijk, Hannah HE van Zanten, Paul C West, Stephen A Wood, Johan Rockström
Journal: The Lancet Planetary Health (2020)

Food system innovations will be instrumental to achieving multiple Sustainable Development Goals (SDGs). However, major innovation breakthroughs can trigger profound and disruptive changes, leading to simultaneous and interlinked reconfigurations of multiple parts of the global food system. The emergence of new technologies or social solutions, therefore, have very different impact profiles, with favourable consequences for some SDGs and unintended adverse side-effects for others. Stand-alone innovations seldom achieve positive outcomes over multiple sustainability dimensions. Instead, they should be embedded as part of systemic changes that facilitate the implementation of the SDGs. Emerging trade-offs need to be intentionally addressed to achieve true sustainability, particularly those involving social aspects like inequality in its many forms, social justice, and strong institutions, which remain challenging. Trade-offs with undesirable consequences are manageable through the development of well planned transition pathways, careful monitoring of key indicators, and through the implementation of transparent science targets at the local level.
Abstract 12684: Development and Implementation of Blood Pressure Screening and Referral Guidelines for Community Pharmacists
Authors: Martin Schulz, Nina Griese-Mammen, Pia M Schumacher, Dorothea Strauch, Leonard Freudewald, André Said, Ross T Tsuyuki, Ulrich Laufs, Ulrich Kintscher, Michael Boehm, Felix Mahfoud
Journal: Circulation (2020)

Introduction: Involvement of community pharmacists in the detection and control of hypertension improves patient care. However, current guidelines do not provide recommendations on collaboration between pharmacists and physicians, especially when and how to refer patients with undetected or uncontrolled hypertension to a physician. Methods: The German Society of Cardiology (DGK) and the ABDA - Federal Union of German Associations of Pharmacists developed and tested a structured referral guideline for community pharmacists. The project included a guideline-directed blood pressure measurement and recommendations when patients should be referred to their physician. A “red flag” referral within 4 weeks was recommended when SBP was &gt;140 mmHg or DBP &gt;90 mmHg (for subjects &lt;80 years), and &gt;160 mmHg or &gt;90 mmHg (≥80 years) in undetected individuals, or &gt;130 mmHg or &gt;80 mmHg (&lt;65 years) and &gt;140 mmHg or &gt;80 mmHg (≥65 years) in treated patients (Figure). Results: Blood pressure was measured in 187 individuals (86 with known hypertension) from 17 community pharmacies. In patients with hypertension, poorly controlled blood pressure was detected in 55% (n=47) and were referred. A total of 16/101 subjects without a history of hypertension were referred to their physician because of uncontrolled blood pressure. Conclusion: Structured blood pressure testing in community pharmacies identified a significant number of subjects with undetected/undiagnosed hypertension and patients with poorly controlled blood pressure. Community pharmacists could play a significant role in collaboration with physicians to improve the management of hypertension.
Multivalent rubber-like RNA nanoparticles for targeted co-delivery of paclitaxel and MiRNA to silence the drug efflux transporter and liver cancer drug resistance
Authors: Hongzhi Wang, Satheesh Ellipilli, Wen-Jui Lee, Xin Li, Mario Vieweger, Yuan-Soon Ho, Peixuan Guo
Journal: Journal of Controlled Release (2020)
The Role of Complex Fractionated Atrial Electrograms in Atrial Fibrillation Ablation: Moving to the Beat of a Different Drum**Editorials published in the Journal of the American College of Cardiologyreflect the views of the authors and do not necessarily represent the views of JACCor the American College of Cardiology.
Authors: Koonlawee Nademanee, Naoya Oketani
Journal: Journal of the American College of Cardiology (2009)
CTNI-01. EFFECT OF STEREOTACTIC RADIOSURGERY COMPARED TO WHOLE-BRAIN RADIOTHERAPY FOR LIMITED BRAIN METASTASIS ON LONG TERM COGNITION AND QUALITY OF LIFE: A POOLED ANALYSIS OF RANDOMIZED CLINICAL TRIALS
Authors: Joshua Palmer, Brett Klamer, Karla Ballman, Paul Brown, Jane Cerhan, S Keith Anderson, Anthony Whitton, Jeffrey Greenspoon, Caroline Chung, Stuart Burri, Ian Parney, Volker Stieber, Bruce Pollock, Nadia Laack, Jonathon Ashman, Jean-Paul Bahary, Fred Barker, Evanthia Galanis, Anthony Asher, David Roberge
Journal: Neuro-Oncology (2020)

Abstract PURPOSE We investigated the long term impact of SRS and WBRT in two large prospective phase III trials. METHODS Patients with 1–4 BMs +/- resection were randomized to SRS or WBRT. Cognitive deterioration was a drop of &amp;gt;1 standard deviation from baseline in &amp;gt;2/6 cognitive measures (CM). Quality of life (QOL) scores were scored 0–100 point scale. CM and QOL scores were modeled using baseline adjusted Linear Mixed Models (LMM) with uncorrelated random intercept for subject and random slopes for time. Differences over time between groups and the effect of &amp;gt;2 cognitive scores with &amp;gt;2 SD change from baseline were assessed. RESULTS 88 patients were included with median follow up of 24 months. We observed decreasing CM over time (SRS: 4/6; WBRT: 5/6). Mean CM was significantly higher in SRS for Total recall and Delayed Recall at 3, 6, 9, 12 months. More patients in WBRT arm declined 1 SD in &amp;gt;1 and &amp;gt;2 CM at the 3, 6, 9, and 12 months. A 1 SD decline in &amp;gt;3 CM at 1 year was 21% SRS vs 47% WBRT (p=0.02). SRS had fewer patients with a 2 SD decline in &amp;gt;1 CM at every time point. SRS had fewer patients with a 2 SD decline at &amp;gt;2 and &amp;gt;3 CM. WBRT had lower QOL at 3 months, but switched to SRS having lower QOL at 24 months for PWB, EWB, FWB, FactG, BR, and FactBR (p&amp;lt; 0.05). A 2 SD decline in cognition decreased mean FWB by 6.4 units (95% CI: -11, -1.75; p=0.007) and decreased QOL by 5.1 units (95% CI: -7.7, -2.5; p&amp;lt; 0.001). CONCLUSIONS We report the first pooled prospective study demonstrating the long term outcomes of patients with BMs after cranial radiation. WBRT was associated with worse cognitive outcomes. Impaired cognition is associated with worse QOL.
Total Body Irradiation or Chemotherapy Conditioning in Childhood ALL: A Multinational, Randomized, Noninferiority Phase III Study
Authors: Christina Peters, Jean-Hugues Dalle, Franco Locatelli, Ulrike Poetschger, Petr Sedlacek, Jochen Buechner, Peter J. Shaw, Raquel Staciuk, Marianne Ifversen, Herbert Pichler, Kim Vettenranta, Peter Svec, Olga Aleinikova, Jerry Stein, Tayfun Güngör, Jacek Toporski, Tony H. Truong, Cristina Diaz-de-Heredia, Marc Bierings, Hany Ariffin, Mohammed Essa, Birgit Burkhardt, Kirk Schultz, Roland Meisel, Arjan Lankester, Marc Ansari, Martin Schrappe, Arend von Stackelberg, Adriana Balduzzi, Selim Corbacioglu, Peter Bader
Journal: Journal of Clinical Oncology (2020)

Total body irradiation (TBI) before allogeneic hematopoietic stem cell transplantation (HSCT) in pediatric patients with acute lymphoblastic leukemia (ALL) is efficacious, but long-term side effects are concerning. We investigated whether preparative combination chemotherapy could replace TBI in such patients.FORUM is a randomized, controlled, open-label, international, multicenter, phase III, noninferiority study. Patients ≤ 18 years at diagnosis, 4-21 years at HSCT, in complete remission pre-HSCT, and with an HLA-compatible related or unrelated donor were randomly assigned to myeloablative conditioning with fractionated 12 Gy TBI and etoposide versus fludarabine, thiotepa, and either busulfan or treosulfan. The noninferiority margin was 8%. With 1,000 patients randomly assigned in 5 years, 2-year minimum follow-up, and one-sided alpha of 5%, 80% power was calculated. A futility stopping rule would halt random assignment if chemoconditioning was significantly inferior to TBI (EudraCT: 2012-003032-22; ClinicalTrials.gov: NCT01949129).Between April 2013 and December 2018, 543 patients were screened, 417 were randomly assigned, 212 received TBI, and 201 received chemoconditioning. The stopping rule was applied on March 31, 2019. The median follow-up was 2.1 years. In the intention-to-treat population, 2-year overall survival (OS) was significantly higher following TBI (0.91; 95% CI, 0.86 to 0.95; P < .0001) versus chemoconditioning (0.75; 95% CI, 0.67 to 0.81). Two-year cumulative incidence of relapse and treatment-related mortality were 0.12 (95% CI, 0.08 to 0.17; P < .0001) and 0.02 (95% CI, < 0.01 to 0.05; P = .0269) following TBI and 0.33 (95% CI, 0.25 to 0.40) and 0.09 (95% CI, 0.05 to 0.14) following chemoconditioning, respectively.Improved OS and lower relapse risk were observed following TBI plus etoposide compared with chemoconditioning. We therefore recommend TBI plus etoposide for patients > 4 years old with high-risk ALL undergoing allogeneic HSCT.
Trends in U.S. Emergency Department Visits Related to Suspected or Confirmed Child Abuse and Neglect Among Children and Adolescents Aged &lt;18 Years Before and During the COVID-19 Pandemic — United States, January 2019–September 2020
Authors: Elizabeth Swedo, Nimi Idaikkadar, Ruth Leemis, Taylor Dias, Lakshmi Radhakrishnan, Zachary Stein, May Chen, Nickolas Agathis, Kristin Holland
Journal: MMWR Morbidity and Mortality Weekly Report (2020)

Heightened stress, school closures, loss of income, and social isolation resulting from the coronavirus disease 2019 (COVID-19) pandemic have increased the risk for child abuse and neglect (1). Using National Syndromic Surveillance Program (NSSP) data from January 6, 2019-September 6, 2020, CDC tabulated weekly numbers of emergency department (ED) visits related to child abuse and neglect and calculated the proportions of such visits per 100,000 ED visits, as well as the percentage of suspected or confirmed ED visits related to child abuse and neglect ending in hospitalization, overall and stratified by age group (0-4, 5-11, and 12-17 years). The total number of ED visits related to child abuse and neglect began decreasing below the corresponding 2019 period during week 11 (March 15-March 22, 2020) for all age groups examined, coinciding with the declaration of a national emergency on March 13 (2); simultaneously, the proportion of these visits per 100,000 ED visits began increasing above the 2019 baseline for all age groups. Despite decreases in the weekly number of ED visits related to child abuse and neglect, the weekly number of these visits resulting in hospitalization remained stable in 2020; however, the yearly percentage of ED visits related to child abuse and neglect resulting in hospitalization increased significantly among all age groups. Although the increased proportion of ED visits related to child abuse and neglect might be associated with a decrease in the overall number of ED visits, these findings also suggest that health care-seeking patterns have shifted during the pandemic. Hospitalizations for child abuse and neglect did not decrease in 2020, suggesting that injury severity did not decrease during the pandemic, despite decreased ED visits. Child abuse is preventable; implementation of strategies including strengthening household economic supports and creating family-friendly work policies can reduce stress during difficult times and increase children's opportunities to thrive in safe, stable, and nurturing relationships and environments (3).
The COVID-19 vaccines rush: participatory community engagement matters more than ever
Authors: Rochelle Ann Burgess, Richard H Osborne, Kenneth A Yongabi, Trisha Greenhalgh, Deepti Gurdasani, Gagandeep Kang, Adegoke G Falade, Anna Odone, Reinhard Busse, Jose M Martin-Moreno, Stephen Reicher, Martin McKee
Journal: The Lancet (2020)
CRISPR-Cas9 Gene Editing for Sickle Cell Disease and β-Thalassemia
Authors: Haydar Frangoul, David Altshuler, M. Domenica Cappellini, Yi-Shan Chen, Jennifer Domm, Brenda K. Eustace, Juergen Foell, Josu de la Fuente, Stephan Grupp, Rupert Handgretinger, Tony W. Ho, Antonis Kattamis, Andrew Kernytsky, Julie Lekstrom-Himes, Amanda M. Li, Franco Locatelli, Markus Y. Mapara, Mariane de Montalembert, Damiano Rondelli, Akshay Sharma, Sujit Sheth, Sandeep Soni, Martin H. Steinberg, Donna Wall, Angela Yen, Selim Corbacioglu
Journal: New England Journal of Medicine (2020)

Transfusion-dependent β-thalassemia (TDT) and sickle cell disease (SCD) are severe monogenic diseases with severe and potentially life-threatening manifestations. BCL11A is a transcription factor that represses γ-globin expression and fetal hemoglobin in erythroid cells. We performed electroporation of CD34+ hematopoietic stem and progenitor cells obtained from healthy donors, with CRISPR-Cas9 targeting the BCL11A erythroid-specific enhancer. Approximately 80% of the alleles at this locus were modified, with no evidence of off-target editing. After undergoing myeloablation, two patients - one with TDT and the other with SCD - received autologous CD34+ cells edited with CRISPR-Cas9 targeting the same BCL11A enhancer. More than a year later, both patients had high levels of allelic editing in bone marrow and blood, increases in fetal hemoglobin that were distributed pancellularly, transfusion independence, and (in the patient with SCD) elimination of vaso-occlusive episodes. (Funded by CRISPR Therapeutics and Vertex Pharmaceuticals; ClinicalTrials.gov numbers, NCT03655678 for CLIMB THAL-111 and NCT03745287 for CLIMB SCD-121.).
Procyanidins-crosslinked small intestine submucosa: A bladder patch promotes smooth muscle regeneration and bladder function restoration in a rabbit model
Authors: Xiu-Zhen Zhang, Yan-Lin Jiang, Jun-Gen Hu, Long-Mei Zhao, Qiu-Zhu Chen, Yan Liang, Yi Zhang, Xiong-Xin Lei, Rui Wang, Yi Lei, Qing-Yi Zhang, Jesse Li-Ling, Hui-Qi Xie
Journal: Bioactive Materials (2020)

Currently the standard surgical treatment for bladder defects is augmentation cystoplasty with autologous tissues, which has many side effects. Biomaterials such as small intestine submucosa (SIS) can provide an alternative scaffold for the repair as bladder patches. Previous studies have shown that SIS could enhance the capacity and compliance of the bladder, but its application is hindered by issues like limited smooth muscle regeneration and stone formation since the fast degradation and poor mechanical properties of the SIS. Procyanidins (PC), a natural bio-crosslinking agent, has shown anti-calcification, anti-inflammatory and anti-oxidation properties. More importantly, PC and SIS can crosslink through hydrogen bonds, which may endow the material with enhanced mechanical property and stabilized functionalities. In this study, various concentrations of PC-crosslinked SIS (PC-SIS) were prepared to repair the full-thickness bladder defects, with an aim to reduce complications and enhance bladder functions. In vitro assays showed that the crosslinking has conferred the biomaterial with superior mechanical property and anti-calcification property, ability to promote smooth muscle cell adhesion and upregulate functional genes expression. Using a rabbit model with bladder defects, we demonstrated that the PC-SIS scaffold can rapidly promote in situ tissue regrowth and regeneration, in particular smooth muscle remodeling and improvement of urinary functions. The PC-SIS scaffold has therefore provided a promising material for the reconstruction of a functional bladder.