bluuebunny/crossref_metadata_2025_split huggingface dataset:
Major Risks, Uncertain Outcomes: Making Ensemble Forecasts Work for Multiple Audiences
Authors: Carr Rachel Hogan , Montz Burrell , Semmens Kathryn , Maxfield Keri , Connolly Samantha , Ahnert Peter , Shedd Rob , Elliott Jason 
Published: September 2018

AbstractWhen extreme river levels are possible in a community, effective communication of weather and hydrologic forecasts is critical to protecting life and property. Residents, emergency personnel, and water resource managers need to make timely decisions about how and when to prepare. Uncertainty in forecasting is a critical component of this decision-making, but often poses a confounding factor for public and professional understanding of forecast products. A new suite of products from the National Weather Service’s Hydrologic Ensemble Forecast System (HEFS) provides short- and long-range forecasts, ranging from 6 h to 1 yr, and shows uncertainty in hydrologic forecasts. To understand how various audiences use and interpret ensemble forecasts showing a range of hydrologic forecast possibilities, a research project was conducted using scenario-based focus groups and surveys with community residents, emergency managers, and water resource managers in West Virginia and Maryland. The research assessed the utility of the HEFS products, identified barriers to proper understanding of the products, and suggested modifications to product design that could improve the understandability and accessibility for a range of users. There was a difference between the residential users’ reactions to the HEFS compared to the emergency managers and water resource managers, with the public reacting less favorably to all versions. The emergency managers preferred the revised HEFS products but had suggestions for additional changes, which were incorporated. Features such as interactive text boxes and forecaster’s notes further enhanced the utility and understandability of the products.
Gender Differences in Energy Expenditure During Walking With Backpack and Double-Pack Loads
Authors: Li Simon S. W. , Chan Otto H. T. , Ng T. Y. , Kam L. H. , Ng C. Y. , Chung W. C. , Chow Daniel H. K. 
Published: September 2018

Objective: To investigate gender differences in energy expenditure during walking with backpack and double-pack loads. Background: Studies have reported that energy expenditure during walking with double-pack loads is lower compared with backpack carriage. However, the effect of gender on energy expenditure while walking with these two load distribution systems has not been investigated. Method: Thirty healthy young adults (15 female and 15 male participants) walked on a treadmill with backpack and double-pack loads weighing 30% of their body weight at a speed of 0.89 m/s for 10 min. The energy expenditure in terms of oxygen consumption (VO2) and respiratory exchange ratio (RER) were continuously monitored using a portable gas analyzer throughout each walking exercise. A mixed-design analysis of variance model was adopted to test the effects of gender, pack, and time on VO2 and RER. Results: No time effect was observed on VO2. However, significant gender, pack, and interaction effects were observed. The lowest VO2 was found in female participants under double-pack carriage. No significant gender or pack differences existed in RER. However, RER significantly and incrementally increased in time from the 4th through 6th, 8th, and 10th min. Conclusion: This study revealed that heavy double-pack load carriage for healthy young female participants had significantly lower energy expenditure (normalized by the entire system weight, i.e., the participant’s weight plus the weight of the pack) than that of the male participants in a 10-min walking exercise. Application: The findings of this study indicated that healthy young female participants carried a heavy double-pack with less energy cost (normalized by the entire system weight, i.e., the participant’s weight plus the weight of the pack) compared with their male counterparts during a 10-min walking exercise.
Murder with no Apparent Motive
Authors: Stone Alan A. 
Published: September 2018

The following article was adapted from the first annual Paul Mendelsohn Memorial Lecture, delivered at Tufts Medical School in Boston. Dr. Mendelsohn, a psychiatric resident, was killed while jogging by a deranged motorist who drove onto the sidewalk and struck him down. The circumstances of this tragedy provided the theme for this essay. Can psychiatry explain such crimes? Do the experts' answers resolve the question of criminal responsibility? The insanity defense is conceptualized as three domains: society's demand for retribution, psychiatric expertise, and legal rules. Three cases are discussed that indicate why “scientific” psychiatry should not expect to dominate the other domains.
Attorneys' and Judges' Needs for Continuing Legal Education on Mental Disability Law: Findings from a Survey
Authors: Mossman Douglas , Kapp Marshall B. 
Published: September 2018

Attorneys leave law school with limited knowledge and skills concerning the issues that arise in mental disability law. Yet psychiatrists and psychologists are appearing with increasing frequency as witnesses in the nation's courts, and more attorneys and judges can therefore expect to have to deal with testimony from mental health professionals. To our knowledge, this article is the first published assessment of practicing attorneys' and judges' needs for continuing legal education (CLE) on mental disability issues. The 267 Dayton-area attorneys and 41 southwestern Ohio judges who responded to our mailed survey said that one-seventh of their cases raise issues related to mental health or mental disability. Most responders had not taken any law school courses that dealt with mental disability issues; those who had said their courses were only modestly helpful. CLE was the attorneys' and judges' principal source of information about mental disability law. For practicing attorneys, perceived need for CLE was related to the rate at which psychological issues arose in their practices; practicing lawyers and judges were interested primarily in CLE topics that related to the types of cases they handled or heard. Three-fourths of the attorneys and 95% of the judges said they would probably or definitely attend locally offered CLE on at least one subject. Our findings are consistent with the hypothesis that traditional law school course work relating to mental disability does not give future attorneys and judges the skills and knowledge necessary to their practices (e.g., the ability to challenge expert witnesses); CLE might help remedy this deficiency. Legal educators should use our findings when thinking about law school course content and postgraduate legal education.
Hand strength and dexterity in patients with Prader–Willi syndrome: a pilot intervention study
Authors: Hsu Wan-Ling , Chiu Valeria Jia-Yi , Chang Wei-Hsiu , Lin Mei-Chun , Wei Jang-Ting , Tzeng I-Shiang 
Published: September 2018

Objective The study aim was to examine the hand function (hand strength and dexterity) and intervention effects of training in adults with Prader–Willi syndrome (PWS). Methods Six adults with PWS (two females; mean age 26.14 years) underwent hand muscle strength and dexterity training for 3 months (2 hours per week). The following hand function tests were performed pre- and post-intervention: (1) hand grip, lateral pinch, and tip pinch hand strength tests, (2) the Box and Block test (BBT) for gross manual dexterity and (3) the Purdue Pegboard test for finger dexterity. Results Before treatment, all subjects showed lower hand grip, lateral pinch, tip pinch strength, and poorer manual/finger dexterity relative to healthy adults. After training, hand function scores improved on many test items, but only the left hand tip pinch and the right hand BBT performance showed significant improvements. Conclusions All subjects showed lower hand strength and poorer manual/finger dexterity compared with healthy adults; this should be considered during physical training programs. Owing to limitations in the intervention intensity and possible subject behavioral deficits, further research is needed to clarify the effects of this intervention on hand function in PWS patients.
“Real-World” Application of Thrombolysis in Cardiac Arrest
Authors: Smith Susan E. , Johnson Daniel C. 
Published: September 2018

Background: Current guidelines recommend consideration of thrombosis as a reversible cause of cardiac arrest. The use of thrombolytic therapy during cardiac arrest, however, is controversial. Objective: We sought to characterize the use of thrombolytic therapy during cardiac arrest and to evaluate the rate of return of spontaneous circulation (ROSC) in a “real-word” setting. Methods: A single-center, retrospective, cohort study of adult patients who received alteplase during cardiac arrest between 2010 and 2015 were performed at a tertiary academic medical center. Results: Twenty-six patients were identified. Patients were predominantly male (65%) and Caucasian (89%) and were a median age of 64 years. Five patients had a history of preexisting venous thromboembolism, and eight patients were receiving systemic anticoagulation. Pulmonary embolism was confirmed prior to the administration of alteplase in 5 patients. The median dose of alteplase administered was 100 mg. ROSC was achieved in 65% of patients, 2 of whom survived to hospital discharge. Both surviving patients experienced a bleeding event. Conclusion: In a single center’s experience, thrombolytic therapy is used infrequently for the management of cardiac arrest. Thrombolysis during cardiac arrest should be considered on a case-by-case basis and should be utilized only when there is a high suspicion for pulmonary embolism as the cause of arrest and when thrombolytic therapy is readily available.
Wandering as a Sociomaterial Practice: Extending the Theorization of GPS Tracking in Cognitive Impairment
Authors: Wherton Joseph , Greenhalgh Trisha , Procter Rob , Shaw Sara , Shaw James 
Published: September 2018

Electronic tracking through global positioning systems (GPSs) is used to monitor people with cognitive impairment who “wander” outside the home. This ethnographic study explored how GPS-monitored wandering was experienced by individuals, lay carers, and professional staff. Seven in-depth case studies revealed that wandering was often an enjoyable and worthwhile activity and helped deal with uncertainty and threats to identity. In what were typically very complex care contexts, GPS devices were useful to the extent that they aligned with a wider sociomaterial care network that included lay carers, call centers, and health and social care professionals. In this context, “safe” wandering was a collaborative accomplishment that depended on the technology’s materiality, affordances, and aesthetic properties; a distributed knowledge of the individual and the places they wandered through, and a collective and dynamic interpretation of risk. Implications for design and delivery of GPS devices and services for cognitive impairment are discussed.
Impact of Cardiac Risk Factors in the Postsurgical Outcomes of Patients With Cleft Palate: Analysis of the 2012-2014 NSQIP Database
Authors: Goodenough Christopher J. , Anderson Kathryn T. , Smith Kari E. , Hanfland Robert A. , Wadhwa Nitin , Teichgraeber John F. , Greives Matthew R. 
Published: September 2018

Objective: To assess the risk of complication in patients undergoing cleft palate repair with congenital cardiac comorbidities in a large, national cohort. Design: Retrospective review. Patients/Setting: Using the 2012-2014 National Surgical Quality Improvement Program (NSQIP) Pediatric database, patients undergoing cleft palate repair were selected for analysis. Patients with cleft palate repairs were stratified based on the presence or absence congenital cardiac comorbidities. Univariate and stepwise forward logistic regression were conducted. Main Outcome Measures: It is hypothesized that risk of postoperative adverse events in patients with congenital cardiac comorbidities is higher than in patients without cardiac disease. Results: Nationally, between 2012 and 2014, 3240 patients underwent cleft palate repair, 422 (13.0%) with cardiac disease, and 2818 (87.0%) without cardiac disease. Patients with cardiac disease were smaller (10.5 [6.6] kg vs 11.6 [8.6] kg, P < .01) and more likely to be premature (4.6% vs 13.0%, P < .01) compared to those without cardiac disease. Postoperatively, patients with cardiac conditions were more likely to experience an adverse event (8.8% vs 4.2%, P < .01). Specifically, they were more likely to experience reintubation (1.7% vs 0.4%, P < .01), reoperation (2.1% vs 0.6%, P < .01), and longer length of stay (2.7 [7.0] vs 1.6 [2.8] days, P < .01). Rates of surgical site infection and dehiscence were not different. Conclusions: Cleft palate repair in patients with concurrent congenital cardiac defects is a safe procedure but carries elevated risk in the postoperative period as demonstrated in this analysis of the NSQIP-Pediatric database. Technical risks are equivalent. Additional anesthesia and surgical awareness of these potential complications is essential to minimize perianesthesia risks.
Ideology, Party, and Opinion: Explaining Individual Legislator ACA
Implementation Votes in the States
Authors: Shor Boris 
Published: September 2018

Abstract
Why do state legislators vote the way they do? Which influence is
predominant: ideology, party, or public opinion? The implementation votes
surrounding the Affordable Care Act (ACA) provides a unique setting to
examine this question, as they make all three considerations highly salient.
State roll call votes on ACA implementation were sometimes polarized and
sometimes unexpectedly bipartisan. What accounts for the heterogeneity in
individual legislator behavior on bills implementing the ACA at the state
level? Using new data on legislator ideology and votes from 2011–2015, I
show evidence that legislator ideology was by far the most important
predictor of voting on implementation votes, far more so than legislator
party or public opinion. Moreover, I show the influence of ideology is
heterogeneous by issue area and bill.
Cost-effectiveness of ibrutinib as first-line therapy for chronic lymphocytic leukemia in older adults without deletion 17p
Authors: Barnes James I. , Divi Vasu , Begaye Adrian , Wong Russell , Coutre Steven , Owens Douglas K. , Goldhaber-Fiebert Jeremy D. 
Published: September 2018

Key Points
At current prices, ibrutinib is not a cost-effective initial CLL therapy in older patients without 17p deletion. The cost of ibrutinib would need to be <$6800 per month to be cost-effective.
CD40L/IL-4–stimulated CLL demonstrates variation in translational regulation of DNA damage response genes including ATM
Authors: Lezina Larissa , Spriggs Ruth V. , Beck Daniel , Jones Carolyn , Dudek Kate M. , Bzura Aleksandra , Jones George D. D. , Packham Graham , Willis Anne E. , Wagner Simon D. 
Published: September 2018

Key Points
CD40L/IL-4 responses mediate translational regulation of DNA damage repair genes, including ATM, and associate with baseline levels of ATM. Lower levels of baseline ATM, independent of 11q deletion, associate with reduced overall survival.
How ibrutinib, a B-cell malignancy drug, became an FDA-approved second-line therapy for steroid-resistant chronic GVHD
Authors: Jaglowski Samantha M. , Blazar Bruce R. 
Published: September 2018

Abstract
Allogeneic hematopoietic stem cell transplantation (allo-SCT) is potentially curative for a number of hematologic conditions, both malignant and nonmalignant. However, its success can be limited by the development of acute and chronic graft-versus-host disease (GVHD). Chronic GVHD (cGVHD) is the most common long-term complication following allo-SCT, and patients who develop this condition have significantly higher morbidity and mortality and significantly lower quality of life than patients who do not. Until recently, there were no US Food and Drug Administration (FDA)–approved therapies for cGVHD treatment. In this review article, we describe how ibrutinib was identified as potential cGVHD therapy based on preclinical cGVHD models and clinical studies in B-cell malignancies and elucidation of its mechanisms of action in cGVHD. Results from a phase 2 clinical trial that was designed based on National Institutes of Health Criteria for the grading and staging of cGVHD culminated in the FDA-approval of ibrutinib as second line therapy of steroid-refractory or steroid-resistant cGVHD. Results of ibrutinib studies in phase 3 randomized studies, for cGVHD prophylaxis and as first -line testing along with steroids will be especially important in selecting the preferred indications for ibrutinib in patients at risk for or who have developed cGVHD.
Evaluation of induction chemotherapies after hypomethylating agent failure in myelodysplastic syndromes and acute myeloid leukemia
Authors: Ball Brian , Komrokji Rami S. , Adès Lionel , Sekeres Mikkael A. , DeZern Amy E. , Pleyer Lisa , Vey Norbert , Almeida Antonio , Germing Ulrich , Cluzeau Thomas , Platzbecker Uwe , Gore Steven D. , Fenaux Pierre , Prebet Thomas 
Published: September 2018

Key Points
Induction led to response in 41% and 32%, survival of 10.8 and 6 months, and transplant in 40% and 42% of responders in MDS and AML. Treatment with high-dose cytarabine improved response rates in MDS and an anthracycline-containing regimen increased survival in AML.
Activation of MYC, a bona fide client of HSP90, contributes to intrinsic ibrutinib resistance in mantle cell lymphoma
Authors: Lee Jimmy , Zhang Liang Leo , Wu Wenjun , Guo Hui , Li Yan , Sukhanova Madina , Venkataraman Girish , Huang Shengjian , Zhang Hui , Alikhan Mir , Lu Pin , Guo Ailin , Galanina Natalie , Andrade Jorge , Wang Michael L. , Wang Y. Lynn 
Published: September 2018

AbstractThe BTK inhibitor ibrutinib has demonstrated a remarkable therapeutic effect in mantle cell lymphoma (MCL). However, approximately one-third of patients do not respond to the drug initially. To identify the mechanisms underlying primary ibrutinib resistance in MCL, we analyzed the transcriptome changes in ibrutinib-sensitive and ibrutinib-resistant cell lines on ibrutinib treatment. We found that MYC gene signature was suppressed by ibrutinib in sensitive but not resistant cell lines. We demonstrated that MYC gene was structurally abnormal and MYC protein was overexpressed in MCL cells. Further, MYC knockdown with RNA interference inhibited cell growth in ibrutinib-sensitive as well as ibrutinib-resistant cells. We explored the possibility of inhibiting MYC through HSP90 inhibition. The chaperon protein is overexpressed in both cell lines and primary MCL cells from the patients. We demonstrated that MYC is a bona fide client of HSP90 in the context of MCL by both immunoprecipitation and chemical precipitation. Furthermore, inhibition of HSP90 using PU-H71 induced apoptosis and caused cell cycle arrest. PU-H71 also demonstrates strong and relatively specific inhibition of the MYC transcriptional program compared with other oncogenic pathways. In a MCL patient-derived xenograft model, the HSP90 inhibitor retards tumor growth and prolongs survival. Last, we showed that PU-H71 induced apoptosis and downregulated MYC protein in MCL cells derived from patients who were clinically resistant to ibrutinib. In conclusion, MYC activity underlies intrinsic resistance to ibrutinib in MCL. As a client protein of HSP90, MYC can be inhibited via PU-H71 to overcome primary ibrutinib resistance.
Development of a prognostically relevant cachexia index in primary myelofibrosis using serum albumin and cholesterol levels
Authors: Tefferi Ayalew , Nicolosi Maura , Penna Domenico , Mudireddy Mythri , Szuber Natasha , Lasho Terra L. , Hanson Curtis A. , Ketterling Rhett P. , Gangat Naseema , Pardanani Animesh D. 
Published: September 2018

Key PointsSerum albumin and cholesterol levels predict survival in primary myelofibrosis, independent of each other and contemporary risk models. The cachexia index, determined by serum albumin and cholesterol levels, might further refine current prognostic models in myelofibrosis.
Early platelet count kinetics has prognostic value in lower-risk myelodysplastic syndromes
Authors: Itzykson Raphael , Crouch Simon , Travaglino Erica , Smith Alex , Symeonidis Argiris , Hellström-Lindberg Eva , Sanz Guillermo , Čermák Jaroslav , Stauder Reinhard , Elena Chiara , Germing Ulrich , Mittelman Moshe , Langemeijer Saskia , Mądry Krzysztof , Tatic Aurelia , Holm Mette Skov , Almeida Antonio Medina , Savic Aleksandar , Šimec Njetočka Gredelj , Luño Elisa , Culligan Dominic , Guerci-Bresler Agnes , Malcovati Luca , van Marrewijk Corine , Bowen David , de Witte Theo , Fenaux Pierre 
Published: September 2018

Key PointsA drop in platelet count >25% relative to baseline at 6 months from diagnosis predicts inferior outcome in lower-risk MDS. Platelet drop combined with RBC-TD at 6 months provides an inexpensive and validated classifier of outcome in lower-risk MDS.
Induced pluripotent stem cell–based mapping of β-globin expression throughout human erythropoietic development
Authors: Vanuytsel Kim , Matte Taylor , Leung Amy , Naing Zaw Htut , Morrison Tasha , Chui David H. K. , Steinberg Martin H. , Murphy George J. 
Published: September 2018

Key PointsiPSC-derived definitive erythroid cells display a globin expression profile corresponding to yolk sac erythromyeloid progenitors. iPSC-derived erythroblasts resemble their postnatal counterparts in terms of gene expression and essential biological processes.
Double-blind, randomized, multicenter phase 2 study of SC411 in children with sickle cell disease (SCOT trial)
Authors: Daak Ahmed A. , Dampier Carlton D. , Fuh Beng , Kanter Julie , Alvarez Ofelia A. , Black L. Vandy , McNaull Melissa A. , Callaghan Michael U. , George Alex , Neumayr Lynne , Hilliard Lee M. , Sancilio Fredrick , Rabinowicz Adrian L. , Heeney Matthew M. 
Published: September 2018

Key PointsSC411 increases DHA in blood cell membranes and reduces home-managed SCD pain crisis and analgesic and opioid use at home to treat SCD pain. The rate of sickle cell crisis was 53% lower for the pooled active groups vs placebo.