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Background: SMA is a neurodegenerative disease caused by biallelic deletion/mutation of SMN1. Copies of a similar gene (SMN2) modify disease severity. In a phase 1 study, SMN GRT onasemnogene abeparvovec (AVXS-101) improved outcomes of symptomatic SMA patients with two SMN2 copies (2xSMN2) dosed ≤6 months. Because motor neuron loss can be insidious and disease progression is rapid, early intervention is critical. This study evaluates AVXS-101 in presymptomatic SMA newborns. Methods: SPR1NT is a multicenter, open-label, phase 3 study enrolling ≥27 SMA patients with 2–3xSMN2. Asymptomatic infants ≤6 weeks receive a one-time intravenous AVXS-101 infusion (1.1x1014 vg/kg). Safety and efficacy are assessed through study end (18 [2xSMN2] or 24 months [3xSMN2]). Primary outcomes: independent sitting for ≥30 seconds (18 months [2xSMN2]) or assisted standing (24 months [3xSMN2]). Results: From April–September 2018, 7 infants received AVXS-101 (4 female; 6 with 2xSMN2) at ages 8–37 days. Mean baseline CHOP-INTEND score was 41.7 (n=6), which increased by 6.8, 11.0, 18.0, and 22.5 points at day 14 (n=4), month 1 (n=3), 2 (n=3), and 3 (n=2). Updated data available at the time of the congress will be presented. Conclusions: Preliminary data from SPR1NT show rapid motor function improvements in presymptomatic SMA patients.
A lasting legacy of the International Polar Year (IPY) 2007–2008 was the promotion of the Permafrost Young Researchers Network (PYRN), initially an IPY outreach and education activity by the International Permafrost Association (IPA). With the momentum of IPY, PYRN developed into a thriving network that still connects young permafrost scientists, engineers, and researchers from other disciplines. This research note summarises (1) PYRN’s development since 2005 and the IPY’s role, (2) the first 2015 PYRN census and survey results, and (3) PYRN’s future plans to improve international and interdisciplinary exchange between young researchers. The review concludes that PYRN is an established network within the polar research community that has continually developed since 2005. PYRN’s successful activities were largely fostered by IPY. With >200 of the 1200 registered members active and engaged, PYRN is capitalising on the availability of social media tools and rising to meet environmental challenges while maintaining its role as a successful network honouring the legacy of IPY.
The recently completed I-band SBF Survey of Galaxy Distances contains about 300 galaxy distances within cz ≲ 4000 km/s. These data allow for good constraints on the local mass density and velocity fields. The mass density parameter βI ≡ Ω0.6/bI, where bI is the biasing factor of the IRAS redshift survey galaxies, is found to be βI ≍ 0.45.
I report here centimeter–wavelength observations carried out at the Very Large Array (VLA) to help resolve two questions. First, what is the source of the far infrared (FIR) emission in infrared-luminous IRAS galaxies, active nuclei or more widely distributed star formation? And what physics underlies the tight correlation (Helou et al., 1985) between FIR and radio flux? To test potential answers to these questions, we believe it is important to study the most luminous IRAS galaxies. We selected 39 for study from the ultraluminous catalog of Strauss et al.(1990 and 1992). All sources had FIR luminosity ≥ 1011.4 L⊙. Radio wavelength observations of these systems provide several advantages. First, in the radio there is no obscuration, so we can “see” the active galactic nuclei, if present. Radio spectral indices can distinguish between synchrotron and thermal emission. And finally, observations at the VLA provide sub–kpc resolution. We observed these sources with the VLA in its C configuration. At 1460 MHz, the effective resolution was ≃ 15″; and ≃ 4″ at 4860 MHz. We made follow-up observations on 24 sources in the A configuration with resolution at 4860 MHz of ≃ 0″.5 (or 300–800 h–1 pc for these sources).
We trained local public health workers on disaster recovery roles and responsibilities by using a novel curriculum based on a threat and efficacy framework and a training-of-trainers approach. This study used qualitative data to assess changes in perceptions of efficacy toward Hurricane Sandy recovery and willingness to participate in future disaster recoveries.
Purposive and snowball sampling were used to select trainers and trainees from participating local public health departments in jurisdictions impacted by Hurricane Sandy in October 2012. Two focus groups totaling 29 local public health workers were held in April and May of 2015. Focus group participants discussed the content and quality of the curriculum, training logistics, and their willingness to engage in future disaster recovery efforts.
The training curriculum improved participants’ understanding of and confidence in their disaster recovery work and related roles within their agencies (self-efficacy); increased their individual- and agency-level sense of role-importance in disaster recovery (response-efficacy); and enhanced their sense of their agencies’ effective functioning in disaster recovery. Participants suggested further training customization and inclusion of other recovery agencies.
Threat- and efficacy-based disaster recovery trainings show potential to increase public health workers’ sense of efficacy and willingness to participate in recovery efforts. (Disaster Med Public Health Preparedness. 2016;10:615–622)
We positionally match sources observed by the Sloan Digital Sky Survey (SDSS), the Two Micron All Sky Survey (2MASS), and the Faint Images of the Radio Sky at Twenty-cm (FIRST) survey. Practically all 2MASS sources are matched to an SDSS source within 2 arcsec; ~11% of them are optically resolved galaxies and the rest are dominated by stars. About 1/3 of FIRST sources are matched to an SDSS source within 2 arcsec; ~80% of these are galaxies and the rest are dominated by quasars. Based on these results, we project that by the completion of these surveys the matched samples will include about 107 stars and 106 galaxies observed by both SDSS and 2MASS, and about 250,000 galaxies and 50,000 quasars observed by both SDSS and FIRST. Here we present a preliminary analysis of the optical, infrared and radio properties for the extragalactic sources from the matched samples. In particular, we find that the fraction of quasars with stellar colors missed by the SDSS spectroscopic survey is probably not larger than ~10%, and that the optical colors of radio-loud quasars are ~0.05 mag. redder (with 4σ significance) than the colors of radio-quiet quasars.
The late Eocene – early Miocene Alpine–Carpathian fold-and-thrust belt (FTB) lies in the transition between the Eastern Alps and the Western Carpathians, SE of the Bohemian crystalline massif. Our study shows the involvement of crystalline basement from the former European Jurassic continental margin in two distinct events. A first extensional event coeval with Eggerian–Karpatian (c. 28–16 Ma) thin-skinned thrusting reactivated the rift basement fault array and resulted from the large degree of lower plate bending promoted by high lateral gradients of lithospheric strength and slab pull forces. Slab break-off during the final stages of collision around Karpatian times (c. 17–16 Ma) promoted large-wavelength uplift and an excessive topographic load. This load was reduced by broadening the orogenic wedge through the reactivation of the lower-plate deep detachment beneath and ahead of the thin-skinned thrust front (with the accompanying positive inversion of the basement fault array) and ultimately, by the collapse of the hinterland summits, enhanced by transtensional faulting. Although this work specifically deals with the involvement of the basement in the Alpine–Carpathian Junction, the main conclusions are of general interest to the understanding of orogenic systems.
The local public health agency (LPHA) workforce is at the center of the public health emergency preparedness system and is integral to locally driven disaster recovery efforts. Throughout the disaster recovery period, LPHAs have a primary responsibility for community health and are responsible for a large number of health services. In the face of decreasing preparedness funding and increasing frequency and severity of disasters, LPHAs continue to provide essential disaster life cycle services to their communities. However, little is known about the confidence that LPHA workers have in performing disaster recovery-related duties. To date, there is no widely used instrument to measure LPHA workers’ sense of efficacy, nor is there an educational intervention designed specifically to bolster disaster recovery-phase efficacy perceptions. Here, we describe the important role of the LPHA workforce in disaster recovery and the operational- and efficacy-related research gaps inherent in today’s disaster recovery practices. We then propose a behavioral framework that can be used to examine LPHA workers’ disaster recovery perceptions and suggest a research agenda to enhance LPHA workforce disaster recovery efficacy through an evidence-informed educational intervention. (Disaster Med Public Health Preparedness. 2015;9:403–408)
Cognition is increasingly being recognized as an important aspect of psychotic disorders and a key contributor to functional outcome. In the past, comparative studies have been performed in schizophrenia and schizo-affective disorder with regard to cognitive performance, but the results have been mixed and the cognitive measures used have not always assessed the cognitive deficits found to be specific to psychosis. A set of optimized cognitive paradigms designed by the Cognitive Neuroscience Test Reliability and Clinical Applications for Schizophrenia (CNTRACS) Consortium to assess deficits specific to schizophrenia was used to measure cognition in a large group of individuals with schizophrenia and schizo-affective disorder.
A total of 519 participants (188 with schizophrenia, 63 with schizo-affective disorder and 268 controls) were administered three cognitive paradigms assessing the domains of goal maintenance in working memory, relational encoding and retrieval in episodic memory and visual integration.
Across the three domains, the results showed no major quantitative differences between patient groups, with both groups uniformly performing worse than healthy subjects.
The findings of this study suggests that, with regard to deficits in cognition, considered a major aspect of psychotic disorder, schizophrenia and schizo-affective disorder do not demonstrate major significant distinctions. These results have important implications for our understanding of the nosological structure of major psychopathology, providing evidence consistent with the hypothesis that there is no natural distinction between cognitive functioning in schizophrenia and schizo-affective disorder.
Beginning roughly in the last two decades of the twentieth century and culminating with the 2008 financial crisis, the dominant Wall Street business model transformed from a customer-driven focus with a minor proprietary trading component to one where principal transactions, and in particular trading in mortgage-backed securities, came to dominate the profits and attention of the brightest minds in the financial industry. This transformation represents a major shift in Wall Street’s business model. The bulk of Wall Street’s profits today are no longer derived from providing financial services to clients. Instead, Wall Street firms generate profits mostly from investing their own capital. The rise of principal transactions occurred not coincidentally just as the financial industry was also experiencing enormous growth in scale as, in the two decades preceding the financial crisis, every major Wall Street firm went from being a privately funded partnership to a publicly held company.
In Chapter two we introduced the idea that profit disjunction occurs when the profits of Wall Street are not aligned with the prosperity of the economy as a whole. In this chapter, we examine how Wall Street profits became unhinged from and dangerous to the general welfare of society. We describe not only how Wall Street became dependent on proprietary trading and the perverse compensation incentives encouraging moral hazard and excessive risk taking, but also how Wall Street became so large that its potential failure as a business sector threatened the stability of the global economy. Our purpose in doing so is not simply to address the issue of “too big to fail” and the bailout. Our objective is to demonstrate how the shift in Wall Street’s business model from serving clients to proprietary trading accompanied a crisis in Wall Street values that in no small measure contributed to the financial crisis. We are concerned, in other words, not only with the threats Wall Street poses to the general economy when it fails, but also with the dangers it creates when it is successful following the postmillennial proprietary trading business model. We begin our analysis, however, by recalling a long bygone era when ongoing customer relations, based upon trust and mutuality, were an essential foundation for the creation and success of the financial industry.
More than four years after the financial crisis that erupted in September 2008, there has been no dearth of books analyzing its origins. Numerous government hearings, documentary films, journalistic investigations, and a national independent commission report have all added to our understanding of the crisis. Certainly a reader might reasonably question the purpose of a new book on the subject. Our simple answer is that the financial crisis was fundamentally a crisis of business ethics rooted in almost three decades of moral, financial, and institutional transformation on Wall Street. Indeed, the most important finding of Wall Street Values is that business ethics and values matter, and that no amount of structural reform and government regulation will ensure the stability of the global financial system unless the ethical practices and values of Wall Street professionals are aligned with market efficiency and the public welfare. In this book we detail when and how Wall Street’s business model and values diverged from the public interest, and we offer a roadmap for realignment.
At the same time that we seek to avoid the Charybdis of redundancy, we are mindful too of the Scylla of obsolescence. As Wall Street Values goes to press, each week brings fresh news of Wall Street’s ineptitude and malfeasance – the botched Facebook offering by a once top-tier investment bank, a “London Whale” losing billions of dollars in unsupervised trading bets for a global bank renowned for its risk management prowess, and a financial firm entrusted with executing market transactions for customers big and small losing nearly half a billion dollars in one morning because of a software glitch that kept automatically trading without any human control. A reader might reasonably question the point of a book written before these developments fully unfold. Again our answer returns to the primacy of ethics, particularly in a time of change and turmoil. Two and a half millennia ago, Confucius, writing at a time of great transformation and uncertainty in ancient China, compared virtue to the North Star, remaining in its place while all the other stars moved about it. So too we believe that focusing on the ethical roots of the financial crisis elucidates how our financial institutions can operate in a manner that nurtures both profits and social prosperity.
On Monday, September 15, 2008, Lehman Brothers, a prominent investment bank that traces its roots to 1850, declared bankruptcy and thereupon triggered a global financial crisis. Literally overnight, borrowing came to a standstill, and widely held assets could not be converted into cash. The liquidity crunch immediately crippled banks owning substantial amounts of securities linked to subprime mortgages and spread very quickly to every sector of the global economy and all types of debt securities. Unable to sell even normally safe and highly liquid investments, on Tuesday, September 16, 2008, the Primary Reserve Fund, the oldest money market fund in the United States, in an action eerily reminiscent of Depression-era bank runs, shocked the financial community by freezing customer accounts and indefinitely halting withdrawals. Ordinary consumers were thus harshly reminded that there were no safe havens for their savings in this economic storm, adding another layer of uncertainty and instability to the financial markets. Within weeks of the Lehman bankruptcy, the resulting shock to the financial system inflicted severe and long-lasting damages on the economy, throwing tens of millions of people out of work and slowing economic growth. Half a decade later, the global economy still limps along in the aftermath of the financial crisis.
The financial crisis sprang from a precipitous decline in the value of mortgage-related securities. The bursting of the mortgage bubble completely wiped out Lehman’s capital base. Other venerable Wall Street institutions including Merrill Lynch and Bear Stearns narrowly averted total collapse through hastily arranged mergers with Bank of America and JPMorgan Chase, respectively. Virtually every major financial institution had massive exposure to the mortgage market relative to its capital base, and even those banks not in danger of imminent collapse suffered staggering losses severely limiting their ability to engage in ordinary consumer lending activities and basic interbank transactions. Because of the financial sector’s centrality to capital and credit markets, the U.S. Congress authorized a $700 billion government bailout to prevent further failures and safeguard the financial system from total collapse.