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There are a variety of causes of acute heart failure in children including myocarditis, genetic/metabolic conditions, and congenital heart defects. In cases with a structurally normal heart and a negative personal and family history, myocarditis is often presumed to be the cause, but we hypothesise that genetic disorders contribute to a significant portion of these cases. We reviewed our cases of children who presented with acute heart failure and underwent genetic testing from 2008 to 2017. Eighty-seven percent of these individuals were found to have either a genetic syndrome or pathogenic or likely pathogenic variant in a cardiac-related gene. None of these individuals had a personal or family history of cardiomyopathy that was suggestive of a genetic aetiology prior to presentation. All of these individuals either passed away or were listed for cardiac transplantation indicating genetic testing may provide important information regarding prognosis in addition to providing information critical to assessment of family members.
We describe the motivation and design details of the ‘Phase II’ upgrade of the Murchison Widefield Array radio telescope. The expansion doubles to 256 the number of antenna tiles deployed in the array. The new antenna tiles enhance the capabilities of the Murchison Widefield Array in several key science areas. Seventy-two of the new tiles are deployed in a regular configuration near the existing array core. These new tiles enhance the surface brightness sensitivity of the array and will improve the ability of the Murchison Widefield Array to estimate the slope of the Epoch of Reionisation power spectrum by a factor of ∼3.5. The remaining 56 tiles are deployed on long baselines, doubling the maximum baseline of the array and improving the array u, v coverage. The improved imaging capabilities will provide an order of magnitude improvement in the noise floor of Murchison Widefield Array continuum images. The upgrade retains all of the features that have underpinned the Murchison Widefield Array’s success (large field of view, snapshot image quality, and pointing agility) and boosts the scientific potential with enhanced imaging capabilities and by enabling new calibration strategies.
OBJECTIVES/SPECIFIC AIMS: Drug development is a common research pursuit for basic and clinical scientists that interfaces diagnostic/therapeutic challenges with funding agencies, pharmaceutical industry, regulatory systems, and education. The University at Buffalo Clinical and Translational Science Institute (CTSI) has implemented a Drug Development Core (DDC) with goals that foster team science and collaboration, optimize laboratory use, and networks investigators. Our goals are to foster collaborations within the region and with other CTSAs. METHODS/STUDY POPULATION: The DDC met with 300 potential investigators from 14 departments and several local companies. There were 35 portal requests from 15 departments and 7 companies; 8 were from training programs. For 28 requests, a reviewer provided consultation, while 7 required discussions and review of data. DDC assisted with 15 grant applications (outcomes pending), 10 industry-related new drug development requests and 1 regulatory review. Curriculum reviews noted overlap and gaps. Cross-institute opportunities for M.D.-Ph.D. research mentoring were identified. RESULTS/ANTICIPATED RESULTS: The DDC met with 300 potential investigators from 14 departments and several local companies. There were 35 portal requests from 15 departments and 7 companies; 8 were from training programs. For 28 requests, a reviewer provided consultation, while 7 required discussions and review of data. DDC assisted with 15 grant applications (outcomes pending), 10 industry-related new drug development requests and 1 regulatory review. Curriculum reviews noted overlap and gaps. Cross-institute opportunities for M.D.-Ph.D. research mentoring were identified. DISCUSSION/SIGNIFICANCE OF IMPACT: The CTSI DDC was well received by investigators. The request process fosters collaboration among researchers with similar interests and identifies core laboratory resources that add innovation to ongoing research, funding applications, education, and interinstitutional planning.
Synthesis and solubility studies of brizziite, NaSbO3, have been undertaken to determine the possible role of this rare secondary phase in the immobilization of Sb under supergene conditions and the conditions responsible for its formation in the supergene zone. Solubility studies were undertaken at T = 298.15 K. A value of ΔGfө) (NaSbO3, s, 298.15 K) = –806.66 ± 1.4 kJ mol–1 was derived. Calculations involving tripuhyite, FeSbO4, byströmite, MgSb2O6, ordoñezite, ZnSb2O6 and rosiaite, PbSb2O6, show that brizziite is a thermodynamically stable phase only at negligible activities of Pb2+(aq) at high pH and high salinity. Calculations involving mopungite Na[Sb(OH)6] combined with reported mineral associations suggest that mopungite is the thermodynamically unstable precursor to brizziite and its presence in natural settings must be due to kinetic stability. This explains why brizziite is such a rare secondary phase and therefore why it cannot exert any significant influence on the dispersion of Sb in the supergene environment.
The discovery of the first electromagnetic counterpart to a gravitational wave signal has generated follow-up observations by over 50 facilities world-wide, ushering in the new era of multi-messenger astronomy. In this paper, we present follow-up observations of the gravitational wave event GW170817 and its electromagnetic counterpart SSS17a/DLT17ck (IAU label AT2017gfo) by 14 Australian telescopes and partner observatories as part of Australian-based and Australian-led research programs. We report early- to late-time multi-wavelength observations, including optical imaging and spectroscopy, mid-infrared imaging, radio imaging, and searches for fast radio bursts. Our optical spectra reveal that the transient source emission cooled from approximately 6 400 K to 2 100 K over a 7-d period and produced no significant optical emission lines. The spectral profiles, cooling rate, and photometric light curves are consistent with the expected outburst and subsequent processes of a binary neutron star merger. Star formation in the host galaxy probably ceased at least a Gyr ago, although there is evidence for a galaxy merger. Binary pulsars with short (100 Myr) decay times are therefore unlikely progenitors, but pulsars like PSR B1534+12 with its 2.7 Gyr coalescence time could produce such a merger. The displacement (~2.2 kpc) of the binary star system from the centre of the main galaxy is not unusual for stars in the host galaxy or stars originating in the merging galaxy, and therefore any constraints on the kick velocity imparted to the progenitor are poor.
At the end of April 1739 the trial began of Lord Santry, a leading member of Dublin's Hellfire Club. Nine months earlier when drunk at Palmerstown Fair, he had stabbed an unfortunate porter named Laughlin Murphy. Murphy died of his wounds: Santry was sentenced to death but received a reprieve from George II. Santry's actions and the publicity these attracted confirmed the notorious image and ‘hastened the demise’ of the club with which he was so closely associated. During its brief existence, the elite Dublin Hellfire Club had acquired a reputation that was irredeemable.
While participation in associational life for the purposes of leisure would rarely result in such scandal again, it has remained vital. Writing of Britain during the modern period, R. J. Morris asserted that the establishment of voluntary associations – clubs and societies with defined rules and typically charging a membership fee – has been ‘one major response to the problems posed by change and complexity’. This is also true of Ireland, and in recent years it has been the subject of increased scholarly attention. As yet, however, this scholarship has scarcely scratched the surface of the range of clubs and societies or of the variety of their roles, but we know enough about the subject of this chapter – associational forms of leisure, with an emphasis on sport – to state with certainty that between 1740 and the present day this phenomenon has consistently constituted a vibrant aspect of Irish life, reflecting and affecting the dynamics of identity formation and reformation across the period.
In his influential study of clubs and societies, Peter Clark contended that by 1800 ‘British voluntary associations had come of age’ and that the English and Scottish, particularly urban-dwellers, lived in ‘An Associational World’. In Ireland, on the other hand, he suggested, there existed a ‘lower incidence of societies’, a state of affairs he ascribed to ‘lower levels of urbanization, the problematic state of the Irish economy in the later eighteenth century, the small size of the Protestant elite, and the importance of traditional forms of socializing and solidarity’, including those associated with the Catholic Church.
When we consider the situation of the human mind in nature, its limited plasticity and few channels of communication with the outer world, we need not wonder that we grope for light, or that we find incoherence and instability in the human systems of ideas. The wonder rather is that we have done so well, that in the chaos of sensations and passions that fills the mind we have found any leisure for self-concentration and reflection, and have succeeded in gathering in even a light harvest of experience from our distracted labours.
Peter Borsay begins A history of leisure: the British experience since 1500 by noting that most histories of leisure ‘wisely eschew any concerted attempt to define’ the subject and instead assume ‘a common-sense meaning shared between writer and reader’. In this volume, harvested from work presented to the annual conference of the Society for the Study of Nineteenth-Century Ireland held at the Mater Dei Institute of Education, Dublin City University, in 2012, twelve scholars contribute chapters that reflect on a variety of manifestations of leisure. If most have taken the route described as wise by Borsay, then collectively they have addressed various issues that the study of leisure opens up to us as they seek to pursue, for different groups and from different points of view, our curiosity as to what people did with time that was their own, why they did what they did, and what this means.
In doing so, they remind us that the study of leisure can contribute to our understanding of what we used to call societies and classes, and now more often call cultures, through assessing how time was structured for individuals and groups, by seeking to understand how those people spent their ‘free’ time, by pointing to the factors that facilitated or inhibited their choices, and by examining the meaning and values the leisured, and others, assigned to those choices. It throws light on the identities people created, the relationships they forged, the ideologies they encountered and promoted, the spaces they occupied, the technologies they employed, and, it is to be hoped, the fun they found through their leisure.
It has often been argued that ‘modern’ leisure was born in the period from the mid-nineteenth century to the outbreak of World War One. Then, it has been suggested, that if leisure was not ‘invented’ its forms and meanings changed.Despite the recent expansion of the literature on Irish popular cultures – perhaps most strikingly sport – the conceptions, purposes, and practical manifestations of leisure among the Irish during this critical period have yet to receive the attention they deserve. This collection represents an attempt to address this.In twelve essays that explore vibrant expressions of associational culture, the emergence of new leisure spaces, literary manifestations and representations of leisure, the pleasures and purposes of travel, and the leisure pursuits of elite women the collection offers a variety of perspectives on the volume’s theme. As becomes apparent in these studies, all manner of activity, from music to football, reading to dining, travel to photography, dancing to dining, visiting to cycling, child’s play to fighting and attitudes to these were shaped not just by the drive to pleasure but by ideas of class, respectability, improvement and social control as well as political, social, educational, medical and religious ideologies.