tbuckley/cabot-search huggingface dataset:
RTHP-35. RE-IRRADIATION USING PULSED REDUCED DOSE RATE RADIATION THERAPY IN PATIENTS WITH GRADE 2 GLIOMAS
Authors: Colin Harari, Adam Burr, H Ian Robins, Steven Howard
Journal: Neuro-Oncology (2019)

Abstract BACKGROUND The time course of progressive grade 2 glioma can span nearly a decade. However, much less is known about re-irradiation in this cohort than in high grade patients. Pulsed reduced dose rate (PRDR) radiation therapy is a method of radiation delivery, which may spare normal tissues by slowing the rate of radiation delivery to allow repair. METHODS We identified consecutive patients from an institutional database initially diagnosed with grade 2 glioma from 2001 to 2017 who underwent pulsed reduced dose rate radiation therapy. Radiation was delivered in 20 cGy pulses every three minutes, most commonly with a 3D conformal, three field technique, encompassing gross disease with a 1–2 cm margin. Kaplan Meier method was utilized to analyze patient outcomes. RESULTS 35 patients with grade 2 glioma were identified. The median survival from the date of the first surgery was 103 months (95% CI 78–129 mo). 71% underwent at least a second resection and 86% of patients receiving chemotherapy. 80% of patients underwent transformation to grade 3 (51%) or grade 4 (29%) glioma. Overall survival from PRDR was 9.8 months (95% CI 4.5–14.5mo) and progression free survival was 6.2 months (95% CI 5.1–7.4) in all patients. Overall response rate was 37%. In patients who remained grade 2 histology, overall survival was 13.3 months and two patients remain disease free at 83 and 34 months with overall response of 71%. Four seizures were documented, otherwise treatment was well tolerated. CONCLUSIONS Progressive grade 2 glioma can progress and evolve over nearly the course of a decade. This study demonstrates the efficacy of re-irradiation in prolonging survival in these patients by delaying progression. In patients who remain with grade 2 disease, prolonged salvage of their disease appears possible.
One Year Data from a Phase 2b Trial of AMT-061 (AAV5-Padua hFIX variant), an Enhanced Vector for Gene Transfer in Adults with Severe or Moderate-Severe Hemophilia B
Authors: Steven Pipe, Adam Giermasz, Giancarlo Castaman, Nigel S. Key, Susan U Lattimore, Frank Leebeek, Wolfgang Miesbach, Michael Recht, Esteban Gomez, Alison Long, Robert Gut, Annette von Drygalski
Journal: Blood (2019)
Fostering Resilience Among Youths Exposed to Adverse Childhood Experiences
Authors: Kyle Wong, Helen Puusepp-Benazzouz, Ralph Nanan
Journal: JAMA Pediatrics (2019)

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 Pediatrics 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 Pediatrics journal
HOUT-08. PATIENT AND PHYSICIAN PERSPECTIVES ON LUMBAR PUNCTURE
Authors: Yoshie Umemura, Benjamin Weill, Baber Khan, Justin Buthorn, Andrew Ridder, Kathryn Nevel, Katherine Panageas, Adrienne Boire
Journal: Neuro-Oncology (2019)

Abstract OBJECTIVE To investigate patients’ and physicians’ perceptions of lumbar puncture (LP) and tolerability. METHODS Adult patients were surveyed prior to and after the procedure regarding patient perceptions and actual experience of LP. Presence of symptoms pre- and post-LP were compared by McNemar test; symptom severity score was rated 0–10, compared by Wilcoxon test; and relative risks were compared by Fisher’s exact test. In addition to patient surveys, email requests to complete an eight-question survey regarding LP tolerability was sent to faculty physicians at two academic centers. RESULTS A total of 154 patients and 302 physicians completed the surveys during 1/2017-5/2019. Ninety-one patients (59%) reported anxiety prior to-LP compared to 52 (34%) who reported anxiety after the LP (p&lt; 0.0001). Whereas 74% of patients predicted LP to be painful prior to LP, only 48% reported that LP was painful after the procedure (p&lt; 0.0001). On an 11-point scale, patients anticipated significantly greater pain from LP than they reported after the procedure (mean 3.4 and 2.0 respectively, p&lt; 0.0001). Patients who anticipated pain were more likely to experience pain (RR=1.65, 95% CI 1.07–2.73). There was no significant difference in symptoms such as headache, nausea, generalized pain, vision or gait disturbance before and after the procedure. After the LP, 117 patients (77%) answered they would repeat LP if recommended by their doctors. Out of 302 physicians (40% women), the majority (81%) felt that the LP induces anxiety or worry in patients, and 50% answered that the LP is painful for patients in general. CONCLUSION We observed that although anticipation of pain contributed to pre-procedure anxiety, LP was generally well-tolerated. The majority of patients experienced minimal pain. The results of this study suggest that measures to reduce pre-procedure anxiety may improve the tolerability of LP, an important diagnostic tool in neuro-oncology.
CMET-13. THE COMPOSITION OF NEWLY-DIAGNOSED BRAIN METASTASES VARIES WIDELY BY PATIENT DEMOGRAPHICS
Authors: Vasileios Kavouridis, Matthew Torre, Maya Harary, Timothy Smith, Bryan Iorgulescu
Journal: Neuro-Oncology (2019)

Abstract INTRODUCTION Metastases comprise a significant subset of all intracranial neoplasms, but their national composition remains unclear and challenging to track in national cancer registries. METHODS The patient demographics and histologic distribution of newly-diagnosed brain metastasis (BM) patients aged &gt;18yo without a prior history of cancer (2010–2015) were evaluated using the National Cancer Database, which comprises &gt;70% of all newly-diagnosed cancers in the U.S. RESULTS 91,686 adults presented with a newly-diagnosed BM between 2010–2015. The most common sites of brain metastases overall were lung (82% of metastatic cases), breast (4.1%), melanoma (3.2%), kidney (2.9%), and colorectal (1.8%). The overall 1-year and 5-year OS rates for all metastatic tumors were 27.0% (95%CI=26.7–27.3]) and 5.3% (95%CI=5.1–5.5), respectively. The distribution of primary sites for newly diagnosed intracranial metastases varied by sex, age, and race. Compared to males, more females had BMs from breast (8.4% versus 0.8%) and fewer had BMs from kidney (1.9% versus 3.8%), melanoma (1.9% versus 4.5%), and esophagus (0.3% versus 2.0%). In young adults, particularly those 20-29yo, BMs were more likely from melanoma, genitourinary (in males), and soft tissue than adults in middle and advanced age. Lung carcinomas comprised fewer BMs in Hispanics (66%) compared to Whites (82%), Blacks (83%), and Asian/Pacific Islanders (85%). BMs from kidney and genitourinary primaries were higher in Hispanics (7.3% and 2.4% of BMs, respectively) than in Whites (2.8% and 0.3%, respectively), Blacks (1.8% and 0.1%, respectively), and Asian/Pacific Islanders (2.6% and 0.2%, respectively). Melanoma was more frequent in Whites (3.8% of BMs) and Hispanics (2.5%) compared to Blacks (0.3%) and Asian/Pacific Islanders (0.6%). CONCLUSION Our results illustrate the national distribution of newly-diagnosed BMs and investigates how the distribution varies by patient demographics.
INNV-23. GLIOBLASTOMA AND FACEBOOK: AN ANALYSIS OF PERCEIVED ETIOLOGIES AND TREATMENTS.
Authors: Naveen Kumar Reddy, Nicholas Blondin
Journal: Neuro-Oncology (2019)

Abstract Facebook has become one of the most widely used platforms by patients and caregivers for information on GBM. As such, physicians treating GBM are challenged with reconciling their medical advice with online media sources. In many cases, the information from these online sources can run counter to the advice given by physicians. OBJECTIVE This study sought to understand the type of information being shared on a popular GBM Facebook community titled, “GLIOBLASTOMA SURVIVORS TO THRIVERS!” with regards to 1. The perceived causes of GBM and 2. The therapies that led to GBM remission. METHODS All the posts in a 30-day period (5/01/2019-6/01/2019) were screened for information on GBM etiologies and GBM therapies. Within each group, posts were sorted into distinct sub-categories with posts of similar content. The sub-categories were ranked to determine which etiologies and therapies were most commonly seen by group members. RESULTS A total of 83 posts were on the topic of “GBM Etiologies” and 80 on the topic of “GBM Therapies.” Within the “GBM Etiologies”, the reasons for developing GBM were due to 1. Unknown (31.3%) 2. Previous Radiation Exposure (24.1%) 3. Chemical Exposure (17%) 4. Genetic (12%) 5. Infectious Disease (6%) 6. Losartan/Valsartan (4.8 %). and 7. Head Trauma (2.4%) and Emotional Trauma (2.4%). Within the GBM Therapy category, the therapies that led to remission were 1. Standard of Care (36.3%) 2. CBD/THC (16.3%) 3. Ketogenic Diet (12.5%) 4. Avastin (7.5%) and Optune (7.5%) 5. IV Vitamin C (6.25%) and COC Protocol (6.25%) 6. Meditation/Yoga/Acupuncture (5.0%) and 7. Faith Healing (2.5%). CONCLUSION In the Facebook group titled, ““GLIOBLASTOMA SURVIVORS TO THRIVERS!,” the top three most commonly posted reasons for developing GBM were “Unknown” “Previous Radiation Exposure” and “Chemical Exposure.” The top three therapies that led to remission were “Standard of Care” “CBD/THC” and “Ketogenic Diet.”
Comparative Efficacy and Safety of Once Versus Twice Weekly Carfilzomib Based Therapies in Relapsed Refractory Multiple Myeloma- a Systematic Review
Authors: Saif Ur Rehman, Saad Sharif, Syeda Sabeeka Batool, Hamza Bajwa, Arafat Ali Ali Farooqui, Zahoor Ahmed, Ahmad Anjum, Talha Bin Farooq, Samana Mukhtar, Sibgha Gull Chaudhary, Muhammad Junaid Tariq, Tariq Iqtidar Sadiq Syed, Syed Maaz Abdullah, Muhammad Salman Faisal, Faiz Anwer
Journal: Blood (2019)
IMMU-43. POLYAMINE METABOLISM REGULATES MYELOID IMMUNE SUPPRESSION IN GLIOBLASTOMA
Authors: Jason Miska, Catalina Lee Chang, Aida Rashidi, Yu Han, Aurora Lopez-Rosas, Maciej S Lesniak
Journal: Neuro-Oncology (2019)

Abstract Tumor-associated myeloid cells, which consist of tumor associated macrophages and myeloid-derived suppressor cells (MDSCs), make up a majority of cellular infiltrates in glioma. Glioma infiltrating MDSCs highly express arginase-1 (Arg-1), a catabolic enzyme thought to deplete arginine from the tumor microenvironment. Despite being a well-known marker of immunosuppressive cells, the metabolic reasons for this choice are not clear. Examination of MDSC phenotype in murine glioma models using: RNA-seq, bulk metabolomics, and Carbon-13 arginine flux revealed that two separate pathways of arginine catabolism converge on the generation of ornithine. Ornithine is the prerequisite substrate for the de-novo generation of polyamines, a group of nitrogen-rich metabolites with foundational importance to all mammalian, bacterial, and plant biology. Importantly, we found that the rate-limiting step of polyamine generation, ornithine decarboxylase 1 (ODC1), is dramatically upregulated by glioma infiltrating MDSCs, suggesting de-novo polyamine generation is important for MDSC function. Treatment with a specific inhibitor of de-novo polyamine synthesis, difluoromethylornithine (DMFO), inhibited the immunosuppressive function of in-vitro generated glioma associated MDSCs. However, DFMO only exerted effects before differentiation, as DFMO treatment post-generation did not change their suppressive functions. This suggests that the generation of the polyamine pool is critical to immune suppression by MDSCs. Interestingly the expression of the rate limiting step of polyamine degradation (SAT1) is inversely correlated with (ODC1) during MDSC differentiation, suggesting that utilization of this polyamine pool may be required for the suppressive functions of these cells. Inhibition of SAT1 after MDSC generation blunted MDSC mediated T-cell suppression. The results of this study show that the role of arginine metabolism in tumor infiltrating MDSCs is to generate pools of polyamines which maintain MDSC function in glioma. Therapeutic targeting of this pathway may be a novel and powerful tool to combat immunosuppression in glioma.
Familial Haploidentical (FHI) Allogeneic Stem Cell Transplantation (AlloSCT) Utilizing CD34 Enrichment and PB MNC Addback in Children and Adolescents with High Risk Sickle Cell Disease (SCD): Rapid Engraftment, Immune Cell Reconstitution, and Sustained Donor Chimerism (IND 14359)
Authors: Janet Ayello, Yaya Chu, Carolyn A. Keever-Taylor, Julie-An Talano, Rona Weinberg, Mildred Semidei-Pomlaes, Lee Ann Baxter-Lowe, Theodore B. Moore, Sandra Fabricatore, Shalini Shenoy, Cori M. Abikoff, Mitchell S. Cairo
Journal: Blood (2016)
Pozelimab, a Human Antibody Against Complement Factor C5, Demonstrates Robust Inhibition of Alternative Complement Activity Both in Normal Human Serum and in Phase I Normal Healthy Volunteers
Authors: Kishor Devalaraja-Narashimha, Yan G Ni, Cong Huang, Ming-Dauh Wang, Umesh Chaudhari, Srinivasa Prasad, Olivier Harari, Andrew J Rankin, Lori Morton, Jonathan Weyne
Journal: Blood (2019)
Siberian hamster: a new indoor source of allergic sensitization and respiratory disease
Authors: J. M. Bertó, A. Peláez, E. Fernández, M. Lombardero, M. Ferrer
Journal: Allergy (2002)

Background: The identification of ‘unknown’ allergic sensitizations may determine the prognosis and treatment of patients with respiratory airway disease. Currently, the presence in homes of ‘exotic’ animals as pets is increasing. In this article the Siberian hamster or dwarf hamster ( Phodopus sungorus ) was identified as a new indoor source of aeroallergens and respiratory disease. Methods: The subjects were six outpatients who were treated for asthma and rhinitis. Siberian hamster hair extract was prepared with a standard wt/vol method, and patients were skin‐prick tested with the extract. Serum‐specific immunoglobulin (Ig)E against the Siberian hamster, common hamster ( Cricetus cricetus ) and golden hamster ( Mesocricetus auratus ) was determined. IgE‐immunoblotting was also performed for all six sera. Specific bronchial challenge was carried out with the Siberian hamster extract. Results: Skin prick tests (SPT) with the Siberian hamster extract, and specific IgE‐antibodies against Siberian hamster, were strongly positive in all six patients. Determinations of specific IgE‐antibodies against C. cricetus and M. auratus were negative in all patients. IgE‐immunoblotting of the sera revealed two IgE‐binding fractions (MW 18 and 32 kDa) in five of the six sera. Specific bronchial provocation tests resulted in early asthmatic responses in the two patients who were challenged. Conclusions: The present study reveals the Siberian hamster to be able to induce both sensitization and disease, and this species of hamster should be taken into consideration as a cause of respiratory disease in exposed subjects. A noteworthy finding was the lack of sensitization in our patients to common hamster allergens ( M. auratus and C. cricetus ) that are usually tested when hamster allergy is suspected.
Deep Immunoprofiling By Flow Cytometry and NGS Reveals Distinct T Cell Profile in Pediatric Hepatitis-Associated Aplastic Anemia
Authors: Michaela Novakova, Michael Svaton, Martina Sukova, Veronika Kanderova, Nikos Darzentas, Lenka Kramna, Ondrej Cinek, Dagmar Pospisilova, Jan Trka, Ondrej Hrusak, Jan Stary, Ester Mejstrikova, Eva Fronkova
Journal: Blood (2019)
Uterine sarcomas: Analysis of clinicopathologic factors and treatment outcomes—A single institution experience in long-term disease control.
Authors: Mylene Sy Go, Le Wang, Michael Styler, Stephanie King
Journal: Journal of Clinical Oncology (2012)

e15522 Background: Uterine sarcomas behave aggressively and are associated with poor prognosis. Carcinosarcomas was re-classified as more akin to endometrial adenocarcinomas. The objective was to analyze the survival outcomes of all uterine sarcomas treated with various combinations of chemotherapy, radiation, and lymph node dissections. Methods: 60 patients with proven uterine sarcomas were treated at Hahnemann Hospital from 2002-2011. Survival analyses in each treatment group were performed by Kaplan- Meier method Results: Among the 60 women examined, 51% were carcinosarcoma, 25% leiomyosarcoma, 20% endometrial stromal sarcoma, and 4% adenosarcoma. 27% presented with stage I disease, 20% stage II, 13% stage III, and 40% with metastatic disease at diagnosis. One third of the leiomyosarcomas and endometrial stromal sarcomas have advanced disease at diagnosis. Median survival of all patients was 26 months, 20 months for carcinosarcoma, 25 months for high grade endometrial stromal sarcoma, and a trend toward superior overall survival at 73 months for leiomyosarcoma. After surgery, 51% of patients mostly carcinosarcomas received carboplatin and paclitaxel as adjuvant chemotherapy and 66% of leiomyosarcomas received gemcitabine and docetaxel. However, the survival analysis showed no statistically significant benefit in disease progression free survival. On the other hand, 50% of patients were also treated with adjunctive pelvic external beam radiation and 21% received additional vaginal brachytherapy. Our results demonstrated a small but clear survival benefit after pelvic radiation therapy. Pelvic lymphadenectomy was performed in 55% of these patients and lymph node involvement was found in 30% of uterine sarcomas mostly carcinosarcomas. Survival analysis indicated that pelvic lymphadenectomy was associated with significant survival benefit. Conclusions: Size, stage, nodal involvement, and histology are independent prognostic factors. Complete cytoreduction followed by lymph node dissection, and radiation therapy offered a survival advantage than surgery alone. However, the role of adjuvant chemotherapy remains uncertain.
Predicting Benefit From Evolocumab Therapy in Patients With Atherosclerotic Disease Using a Genetic Risk Score
Authors: Nicholas A. Marston, Frederick K. Kamanu, Francesco Nordio, Yared Gurmu, Carolina Roselli, Peter S. Sever, Terje R. Pedersen, Anthony C. Keech, Huei Wang, Armando Lira Pineda, Robert P. Giugliano, Steven A. Lubitz, Patrick T. Ellinor, Marc S. Sabatine, Christian T. Ruff
Journal: Circulation (2019)

Background: The ability of a genetic risk score to predict risk in established cardiovascular disease and identify individuals who derive greater benefit from PCSK9 (proprotein convertase subtilisin/kexin type 9) inhibition has not been established. Methods: We studied 14 298 patients with atherosclerotic cardiovascular disease from the FOURIER trial (Further Cardiovascular Outcomes Researh With PCSK9 Inhibition in Subjects With Elevated Risk). A 27–single-nucleotide polymorphism genetic risk score defined low (quintile 1), intermediate (quintiles 2–4), and high (quintile 5) genetic risk. Patients were also categorized by major atherosclerotic risk factors including diabetes mellitus, hypertension, low-density lipoprotein cholesterol ≥100 mg/dl, and smoking; multiple (≥2) risk factors was considered high clinical risk. Outcomes consisted of major coronary events (coronary heart death, myocardial infarction, or coronary revascularization) and major vascular events (major coronary events and ischemic stroke). Median follow-up was 2.3 years. Results: After we adjusted for clinical factors, the genetic risk score was associated with risk for both major vascular events ( P trend =0.007) and major coronary events ( P trend &lt;0.0001). Individuals with intermediate and high genetic risk scores had 1.32- and 1.66-fold increased hazard for major coronary events, respectively. Elevated genetic risk was additive to major atherosclerotic risk factors and identified patients more likely to benefit from evolocumab. There was no benefit for major vascular events in patients without multiple clinical risk factors or high genetic risk (hazard ratio [HR], 0.99; absolute risk reduction [ARR], 0.06%, P =0.89). In contrast, there was a 15% relative risk reduction (HR, 0.85 [0.74–0.98], P =0.024) and a 1.6% ARR in patients with multiple clinical risk factors but without high genetic risk and a 25% relative risk reduction (HR, 0.75 [0.60–0.94], P =0.013), and 3.0% ARR in patients with high genetic risk, irrespective of clinical risk ( P trend for HR=0.095, ARR P trend =0.034). Patients with high genetic risk who received evolocumab had event rates similar to patients with a low burden of both genetic and clinical risk. Conclusion: Patients without multiple clinical risk factors or high genetic risk had a low event rate and did not appear to derive benefit from evolocumab over 2.3 years. Conversely, patients with multiple clinical risk factors but without high genetic risk had intermediate risk and intermediate risk reduction. Patients with high genetic risk, regardless of clinical risk, had a high event rate and derived the greatest benefit from evolocumab, which mitigated this risk.
Incidental Detection of Iliac Artery Occlusion on 3-Phase Bone Scintigraphy
Authors: Myoung Hyoun Kim, Dae-Weung Kim
Journal: Clinical Nuclear Medicine (2019)

Our patient was a 72-year-old man with absent activity of the right femoral artery and mildly decreased Tc-DPD activity on the right leg as indicated on the blood pool and delayed images, respectively. Subsequent peripheral angiography revealed a total occlusion of the right external iliac artery with good collateral flow. Careful review of blood flow and blood pool images of 3-phase bone scintigraphy could provide additional information about peripheral vascular disease.