bluuebunny/crossref_metadata_2025_split huggingface dataset:
Suppressors and activators of JAK-STAT signaling at diagnosis and relapse of acute lymphoblastic leukemia in Down syndrome
Authors: Schwartzman Omer , Savino Angela Maria , Gombert Michael , Palmi Chiara , Cario Gunnar , Schrappe Martin , Eckert Cornelia , von Stackelberg Arend , Huang Jin-Yan , Hameiri-Grossman Michal , Avigad Smadar , te Kronnie Geertruy , Geron Ifat , Birger Yehudit , Rein Avigail , Zarfati Giulia , Fischer Ute , Mukamel Zohar , Stanulla Martin , Biondi Andrea , Cazzaniga Giovanni , Vetere Amedeo , Wagner Bridget K. , Chen Zhu , Chen Sai-Juan , Tanay Amos , Borkhardt Arndt , Izraeli Shai 
Published: May 2017

SignificanceChildren with Down syndrome are at increased risk for B-cell acute lymphoblastic leukemia (DS-ALL), often expressing cytokine receptor-like factor 2 (CRLF2). Here we studied matched diagnosis and relapse DS-ALLs to understand the pathogenesis of relapse. We confirm that enhanced JAK-STAT signaling frequently “drives” CRLF2posDS-ALL at diagnosis, but discovered that clones with JAK mutations are unstable, suggesting that they also endowed the transformed cells with vulnerabilities. We find USP9X loss in up to 25% of CRLF2posALLs, and demonstrate that its ablation decreases the toxic effect of JAK2 hypersignaling. Thus, in CRLF2posALLs JAK-STAT signaling is often buffered by loss of USP9X. These results have therapeutic implications because they suggest that ALL cells can tolerate a limited range of JAK-STAT signaling.
Effect of media presentations on willingness to commit to organ donation
Authors: Harel Inbal , Kogut Tehila , Pinchas Meir , Slovic Paul 
Published: May 2017

SignificanceWe often encounter cases of organ donation in the press or on television. How might these stories affect readers? We found that reading coverage of cases that include identifying information about the receiver (saved by organ donations) increased participants’ willingness to commit to organ donation themselves, to donate the organs of a deceased relative, or to support a transition to an “opt-out” policy. Conversely, identifying the deceased donor induced thoughts of death rather than about saving lives, resulting in fewer participants willing to donate organs or to support policies that facilitate organ donation. We show that most of the stories that appear in the press include an identified donor rather than an identified receiver, possibly reducing organ donations.
Superresolution without separation
Authors: Schiebinger Geoffrey , Robeva Elina , Recht Benjamin 
Published: May 2017

Abstract
This article provides a theoretical analysis of diffraction-limited superresolution, demonstrating that arbitrarily close point sources can be resolved in ideal situations. Precisely, we assume that the incoming signal is a linear combination of $M$ shifted copies of a known waveform with unknown shifts and amplitudes, and one only observes a finite collection of evaluations of this signal. We characterize properties of the base waveform such that the exact translations and amplitudes can be recovered from $2M+1$ observations. This recovery can be achieved by solving a weighted version of basis pursuit over a continuous dictionary. Our analysis shows that $\ell_1$-based methods enjoy the same separation-free recovery guarantees as polynomial root finding techniques, such as de Prony’s method or Vetterli’s method for signals of finite rate of innovation. Our proof techniques combine classical polynomial interpolation techniques with contemporary tools from compressed sensing.
The Revolving Door in International Investment Arbitration
Authors: Langford Malcolm , Behn Daniel , Lie Runar Hilleren 
Published: May 2017

Abstract
It is often claimed that international investment arbitration is marked by a revolving door: individuals act sequentially and even simultaneously as arbitrator, legal counsel, expert witness, or tribunal secretary. If this claim is correct, it has implications for our understanding of which individuals possess power and influence within this community; and ethical debates over conflicts of interests and transparency concerning ‘double hatting’—when individuals simultaneously perform different roles across cases. In this article, we offer the first comprehensive empirical analysis of the individuals that make up the entire investment arbitration community. Drawing on our database of 1039 investment arbitration cases (including ICSID annulments) and the relationships between the 3910 known individuals that form this community, we offer the first use of social network analysis to describe the full investment arbitration community and address key sociological and normative questions in the literature. Our results partly contradict recent empirical scholarship as we identify a different configuration of central ‘power brokers’. Moreover, the normative concerns with double hatting are partly substantiated. A select but significant group of individuals score highly and continually on our double hatting index.
Diagnostic Practices for Suspected Community-Acquired Central Nervous System Infection in the Post–Conjugate Vaccine Era
Authors: Banniettis Natalie , Joshi Saumya , Kaushik Shubhi , Kohlhoff Stephan , Hammerschlag Margaret R. 
Published: May 2017

Objective
The aim of this study was to evaluate diagnostic practices for suspected community-acquired central nervous system (CNS) infection in an urban pediatric population.

Methods
This is an observational, retrospective single-center review of cerebrospinal fluid (CSF) studies in children, 1 month to 21 years old, evaluated for suspected CNS infection from 2004 to 2014. Cases of suspected nosocomial meningitis were excluded. The frequency of N-methyl-d-aspartate receptor antibody (NMDAR ab) encephalitis was analyzed from 2010 to 2014.

Results
A total of 940 unique patient visits with CSF studies were included in the final analysis. There were 940 bacterial cultures sent; 4 (0.42%) grew suspected CSF bacterial pathogens, and 18 (1.9%) grew organisms that were suspected contaminants. Bacterial pathogens included late-onset group B Streptococcus in 3 infants younger than 3 months and Streptococcus pneumoniae in an unvaccinated 9-year-old child. Viral CNS infection was 7.5 times more frequent than bacterial infection. Enterovirus was the only virus isolated. Five cases positive for NMDAR ab were identified since 2010.

Conclusions
Bacterial studies were performed more frequently than viral and other studies. Cerebrospinal fluid bacterial culture was nearly 5 times more likely to yield a contaminant than a pathogen. The frequency of viral infection was likely underestimated as only 20% were tested, mainly by culture, which is suboptimal. These data suggest diagnostic practices for the evaluation of suspected community-acquired CNS infections in children need to be modified to reflect current epidemiology and highlight the need for greater accessibility to polymerase chain reaction for viral diagnostics. Furthermore, NMDAR ab–mediated encephalitis should be considered early in children presenting with suggestive symptoms.
Epidemiology of Fractures in Children Younger Than 12 Months
Authors: Rodà Diana , Trenchs Victoria , Curcoy Ana Isabel , Martínez Anibal Dolores , Pou Jordi , Luaces Carles 
Published: May 2017

ObjectivesThe aim of this study is to determine the prevalence and characteristics of fractures in young infants attended at the pediatric emergency department (PED).MethodsThis is a retrospective study for 2 years (2011–2012) of children younger than 12 months attended with a fracture at the PED. Age, sex, site and type of fracture, mechanism of injury, time interval before seeking medical attention, and management were analyzed.ResultsOne hundred one patients were included. They represented 0.3% (95% confidence interval, 0.2%–0.4%) of all children younger than 12 months attended at the PED. The median age was 7.7 months (interquartile range, 5.2–10.1 months); 58 (57.4%) were boys. The most common fracture was skull fracture (58, 57.4%), mostly parietal, followed by long bone fractures (27, 26.7%); transverse and torus fractures were the most common types, located at the diaphysis and distal metaphysis, respectively. The principal mechanism reported was falling (83, 82.2%) mainly from furniture. Fifty-one patients (50.1%) were attended in the first 6 hours after injury. Sixty-five patients (64.4%) were admitted at the hospital and the other 9 (8.9%) were controlled in outpatient visits. One of them was injured because of negligence and another was diagnosed with osteoporosis.ConclusionsFractures in young infants are uncommon at the PED, the skull fracture being the most common. Pediatricians should alert caretakers of the risks in normal development to prevent these injuries. Fractures caused by child abuse should always be discarded.
A Rare Cause of Shock in an Infant
Authors: Vayngortin Tatyana , Rosenfeld Howard , Mansour Karim 
Published: May 2017

Abstract
Congenital heart disease can be difficult to diagnose in infants because they often present with nonspecific symptoms. Their clinical presentation can resemble that of respiratory infections or sepsis, yet typical treatments for these conditions such as fluid resuscitation may worsen their course. We report a case of an 8-week-old boy who had several weeks of viral symptoms and eventually presented to the emergency department in shock. An echocardiogram revealed the diagnosis of cor triatriatum, a rare congenital heart disease that requires urgent surgical repair. In this article, we review the evaluation and management of such patients and emphasize the importance of considering congenital heart disease in the differential diagnosis for an infant in shock.
Risk of Colorectal Cancer and Associated Mortality in HIV: A Systematic Review and Meta-Analysis
Authors: O'Neill Tyler J. , Nguemo Joseph D. , Tynan Anne-Marie , Burchell Ann N. , Antoniou Tony 
Published: May 2017

Background:
As people with HIV live longer, the numbers of colorectal cancer cases are expected to increase. We sought to compare the colorectal cancer incidence and cause-specific mortality among people living with and without HIV.

Design:
Systematic review and meta-analysis.

Methods:
We searched 5 electronic databases up to June 28, 2016, for primary studies reporting standardized incidence ratios (SIRs), standardized mortality ratios (SMRs)/hazard ratios or data sufficient for estimating these summary measures. We performed a random effects pooled analysis to estimate SIR and SMR of colorectal cancer in HIV.

Results:
Of 8110 articles, we included 27 studies from North America (n = 18), Europe (n = 7), the Pacific region (n = 4), and South America (n = 1). Overall, 1660 cases of colorectal cancer and colon cancer (excluding rectal cancer) occurred among 1,696,070 persons with HIV. In pooled analysis, we found no summary risk of malignancy among those with HIV relative to an uninfected population (SIR 1.00; 95% confidence interval 0.82 to 1.22; I2 = 89.2%). Colorectal cancer–specific mortality was higher among people with HIV but did not reach statistical significance (SMR 2.09; 95% confidence interval: 1.00 to 4.40; I2 = 85.0%).

Conclusions:
Rates of colorectal cancer are similar between people with and without HIV. Existing screening guidelines are likely adequate for people with HIV.
Industry 1.61803: the transition to an industry with reduced material demand fit for a low carbon future
Authors: Allwood Julian M. , Gutowski Timothy G. , Serrenho André C. , Skelton Alexandra C. H. , Worrell Ernst 
Published: May 2017

Arising from a discussion meeting in September 2016, this editorial introduces a special issue on the transition to a future industrial system with greatly reduced demand for material production and attempts to synthesize the main findings. The motivation for such a transition is to reduce industrial greenhouse gas emissions, but unlike previous industrial transformations, there are no major stakeholders who will pursue the change for their own immediate benefit. The special issue, therefore, explores the means by which such a transition could be brought about. The editorial presents an overview of the opportunities identified in the papers of the volume, presents examples of actions that can be taken today to begin the process of change and concludes with an agenda for research that might support a rapid acceleration in the rate of change.This article is part of the themed issue ‘Material demand reduction’.
Exploring demand reduction through design, durability and ‘usership’ of fashion clothes
Authors: Fletcher Kate 
Published: May 2017

Global planetary boundaries confer limits to production and consumption of material goods. They also confer an obligation to experiment, as individuals and collectively as society, with less-materially-intensive, but no less exuberant, ways of living. This paper takes up this mantle and explores materials demand reduction through a focus on design, fashion garments and the universal, everyday activity of wearing clothes. It takes as its starting point the design of longer-lasting products, a widely favoured strategy for increasing materials efficiency and reducing materials demand in many sectors, including fashion. Drawing on scholarship in the field of design for sustainability and ethnographic research conducted in 16 locations in nine countries about already-existing practices of intensive use and maintenance of clothing, this paper critiques the effectiveness of durability strategies to reduce the amount of materials used. It argues for an update in the familiar preference within sustainability debates for the ‘techno-fix’ to explore instead resourceful use of materials as emerging from human actions and relationships with material goods. It suggests that, while facilitated by design, technology and engineering, opportunities to reduce materials demand begin in individual and collective practices, which, in turn, have dynamic implications for use of materials.
This article is part of the themed issue ‘Material demand reduction’.
Living both well and sustainably: a review of the literature, with some reflections on future research, interventions and policy
Authors: Kasser Tim 
Published: May 2017

The idea that human well-being (WB) can be supported and even enhanced by using, producing, buying, selling and consuming less ‘stuff’ is anathema to many living under consumer capitalism. Yet a growing research literature actually finds that frequent engagement in pro-ecological behaviours (PEBs) is positively correlated with personal WB. This paper reviews data relevant to three possible explanations for the apparent compatibility of PEBs and WB: (i) engaging in PEBs leads to psychological need satisfaction, which in turn causes WB; (ii) being in a good mood causes people to engage in more prosocial behaviours, including PEBs; and (iii) personal characteristics and lifestyles such as intrinsic values, mindfulness and voluntary simplicity cause both PEBs and WB. Because each explanation has some empirical support, I close by reflecting on some relevant interventions and policies that could strengthen each of these three pathways and thereby promote living both well and sustainably.
This article is part of the themed issue ‘Material demand reduction’.
Frugal innovation: doing more with less for more
Authors: Prabhu Jaideep 
Published: May 2017

The global economy will face significant challenges over the next few decades. On the one hand, it must meet the needs of 7 billion consumers (growing to 9 billion by 2050), including the currently unmet basic needs of large numbers in developing countries in areas such as food, energy, housing and health. On the other hand, it must achieve this growth without exceeding the resources available on the planet or causing environmental devastation. This paper argues that such change is possible through a systemic shift to a frugal economy that involves radical, frugal innovation across sectors. Such a transformation will involve the participation of large and small firms, consumers and governments alike. The paper introduces the notion of frugal innovation—the creation of faster, better and cheaper solutions for more people that employ minimal resources—and discusses strategies and examples of such change already taking place in core sectors like manufacturing, food, automotive and energy in developing and developed economies. It also outlines the role of the interaction between large and small firms as well as between firms and consumers in making change possible, as well as the role of governments in driving change where market mechanisms alone will not suffice.This article is part of the themed issue ‘Material demand reduction’.
Why on earth did I buy that? A study of regretted appliance purchases
Authors: Roberts T. , Hope A. , Skelton A. 
Published: May 2017

If targets to reduce greenhouse gas emissions and thereby tackle climate change are to be achieved, it will be necessary to reduce both embodied energy costs (e.g. in terms of producing and manufacturing the products and services that society consumes) and operational energy costs. Reducing the number of purchases that people regret could be a first step in changing the overall dynamic of consumption patterns. This research looks at some potentially adverse effects of consumption on well-being (e.g. negative emotions), applying social practice theory to give insights into why people make purchases that they feel negatively about. This paper draws from: (i) findings of a national survey of over 2000 respondents which found that 53% of adults had reported regretting purchasing an electrical device at some point, and that 23% regretted making such a purchase within the past year; and (ii) a series of walking interviews around people's homes that provide detailed insights into the nature and extent of regretted purchases of electrical goods (e.g. resentment at built-in obsolescence, frustration at the pace of technological change). By combining the qualitative and quantitative data, we develop a typology of regretted consumption and explore the underlying factors that lead to such purchases. The paper concludes with a discussion of the policy implications of this research.This article is part of the themed issue ‘Material demand reduction’.
The carbon price: a toothless tool for material efficiency?
Authors: Skelton Alexandra C. H. , Allwood Julian M. 
Published: May 2017

This article explores whether a carbon price will effectively encourage the more efficient use of greenhouse gas intensive materials such as steel. The article identifies a range of distortions that arise when some of the restrictive assumptions of neoclassical economics are relaxed. Distortions occur due to the sequential nature of decision-making along supply chains, due to imperfect competition and due to government intervention to reduce the risk of carbon leakage. If upstream sectors do not pass on carbon costs, downstream sectors do not have the opportunity to react. Of the distortions identified, compensation mechanisms that reduce the risk of carbon leakage are likely to act as the greatest hinderance to appropriate incentives for the more efficient use of steel in the UK: as things currently stand, unless upstream companies are encouraged to make windfall profits, incentives downstream are weakened. The article concludes by exploring policy options to address the distortions identified, including efforts to reinstate the carbon price downstream and efforts to remove other distortive taxes.
This article is part of the themed issue ‘Material demand reduction’.
Who is reducing their material consumption and why? A cross-cultural analysis of dematerialization behaviours
Authors: Whitmarsh Lorraine , Capstick Stuart , Nash Nicholas 
Published: May 2017

The environmental and economic imperatives to dematerialize economies, or ‘do more with less’, have been established for some years. Yet, to date, little is known about the personal drivers associated with dematerializing. This paper explores the prevalence and profile of those who are taking action to reduce consumption in different cultural contexts (UK and Brazil) and considers influences on dematerialization behaviours. We find that exemplar behaviours (avoiding buying new things and avoiding packaging) are far less common than archetypal environmental behaviours (e.g. recycling), but also that cultural context is important (Brazilians are more likely to reduce their material consumption than people in the UK). We also find that the two dematerialization behaviours are associated with different pro-environmental actions (more radical action versus green consumption, respectively); and have distinct, but overlapping, psychological (e.g. identity) and socio-demographic (e.g. education) predictors. Comparing a more traditional value-identity model of pro-environmental behaviour with a motivation-based (self-determination) model, we find that the latter explains somewhat more variance than the former. However, overall, little variance is explained, suggesting that additional factors at the personal and structural levels are important for determining these consumption behaviours. We conclude by outlining policy implications and avenues for further research.
This article is part of the themed issue ‘Material demand reduction’.
Structure of the Cpf1 endonuclease R-Loop complex after target DNA cleavage
Authors: Stella Stefano , Alcón Pablo , Montoya Guillermo 
Published: April 2017

AbstractCpf1 is a single RNA-guided endonuclease of class 2 type V CRISPR-Cas system, emerging as a powerful genome editing tool1,2. To provide insight into its DNA targeting mechanism, we have determined the crystal structure ofFrancisella novicidaCpf1 (FnCpf1) in complex with the triple strand R-loop formed after target DNA cleavage. The structure reveals a unique machinery for target DNA unwinding to form a crRNA-DNA hybrid and a displaced DNA strand inside FnCpf1. The protospacer adjacent motif (PAM) is recognised by the PAM interacting (PI) domain. In this domain, the conserved K667, K671 and K677 are arranged in a dentate manner in a loop-lysine helix-loop motif (LKL). The helix is inserted at a 45° angle to the dsDNA longitudinal axis. Unzipping of the dsDNA in a cleft arranged by acidic and hydrophobic residues facilitates the hybridization of the target DNA strand with crRNA. K667 initiates unwinding by pushing away the guanine after the PAM sequence of the dsDNA. The PAM ssDNA is funnelled towards the nuclease site, which is located 70 Å away, through a hydrophobic protein cavity with basic patches that interact with the phosphate backbone. In this catalytically active conformation the PI and the helix-loop-helix (HLH) motif in the REC1 domain adopt a “rail shape” and “flap-on” conformations, channelling the PAM strand into the cavity. A steric barrier between the RuvC-II and REC1 domains forms a “septum” that separates the displaced PAM strand and the crRNA-DNA hybrid, avoiding re-annealing of the DNA. Mutations in key residues reveal a novel mechanism to determine the DNA product length, thereby linking the PAM and DNAase sites. Our study reveals a singular working model of RNA-guided DNA cleavage by Cpf1, opening up new avenues for engineering this genome modification system2-4.