Acute fatal presentation of ornithine transcarbamylase deficiency in a previously healthy male

Ornithine transcarbamylase (OTC) deficiency is an X-linked urea cycle defect. While hemizygous males typically present with hyperammonemic coma in infancy, reports of rare late-onset presentations exist, with poor outcomes in males up to 58 years old. Relatives with mutations identical to affected patients often remain asymptomatic, and it is likely that environmental and genetic factors influence disease penetrance and expression. Here, we present our investigation of a patient with late-onset presentation, and we emphasize the potential role of environmental and genetic factors on disease expression. The patient was a previously healthy 62-year-old man who developed mental slowing, refractory seizures, and coma over an 8-day period. Interestingly, the patient had recently used home gardening fertilizers and pesticides. Evaluations for drug and alcohol use, infections, and liver disease were negative. Despite aggressive therapy, blood NH3 concentration peaked at 2,050 μM and the patient died from cerebral edema and cerebellar herniation. Analysis of the OTC gene showed a Pro-225-Thr (P225T) change in exon 7, a mutation that has been previously implicated in OTC deficiency. This case illustrates that OTC deficiency can cause acute, severe hyperammonemia in a previously healthy adult and that the P225T mutation can be associated with late-onset OTC deficiency. We speculate that exposure to organic chemicals might have contributed to the onset of symptoms in this patient. This case also emphasizes that persistent hyperammonemia may cause irreversible neurologic damage and that after the diagnosis of hyperammonemia is established in an acutely ill patient, certain diagnostic tests should be performed to differentiate between urea cycle disorders and other causes of hyperammonemic encephalopathy

Using AI/expert system technology to automate planning and replanning for the HST servicing missions

This paper describes a knowledge-based system that has been developed to automate planning and scheduling for the Hubble Space Telescope (HST) Servicing Missions. This new system is the Servicing Mission Planning and Replanning Tool (SM/PART). SM/PART has been delivered to the HST Flight Operations Team (FOT) at Goddard Space Flight Center (GSFC) where it is being used to build integrated time lines and command plans to control the activities of the HST, Shuttle, Crew and ground systems for the next HST Servicing Mission. SM/PART reuses and extends AI/expert system technology from Interactive Experimenter Planning System (IEPS) systems to build or rebuild time lines and command plans more rapidly than was possible for previous missions where they were built manually. This capability provides an important safety factor for the HST, Shuttle and Crew in case unexpected events occur during the mission

Up and Out: Journalism, Social Media, and Historical Sensibility

Much of the modern theorizing about journalism and communication attained its robustness due to a powerful convergence of distinct middle-range scholarly findings that emerged primarily in the 1970s and 1980s. In the present day, when we turn our analytical gaze to the relationship between journalism and social media, we thus need to strike a delicate balance between conducting new qualitative research, re-conceptualizing and re-interrogating the classic conclusions of political communication scholarship, and linking these two aspects of research together. However, we might also wish to extend our analytical gaze “out,” interrogating the movement of journalistic technology across history, as well as “up,” looking at how journalism fits within larger structural explanations regarding the shape of political life

Localization and Anomalous Transport in a 1-D Soft Boson Optical Lattice

We study the dynamics of Bose-Einstein condensed atoms in a 1-D optical lattice potential in a regime where the collective (Josephson) tunneling energy is comparable with the on-site interaction energy, and the number of particles per lattice site is mesoscopically large. By directly imaging the motion of atoms in the lattice, we observe an abrupt suppression of atom transport through the array for a critical ratio of these energies, consistent with quantum fluctuation induced localization. Directly below the onset of localization, the frequency of the observed superfluid transport can be explained by a phonon excitation but deviates substantially from that predicted by the hydrodynamic/Gross-Pitaevskii equations.Comment: 14 pages, 5 figure

‘I’d be proud to spend the sacred foreign aid budget on our poor pensioners’: Representations of macro aid resourcing in the Irish, UK and US print-media during the economic crisis, 2008–2011

The news-media has been identified as an influence on donor nations’ overseas aid allocations, acting as a site where decisions are justified to ‘domestic constituencies’ and through which resistance is mobilised. Mediated pressures on aid allocations amplified between 2008 and 2011 in three donor countries experiencing domestic economic difficulties: Ireland, the UK and the US. This study suggests that each country’s print-media positioned the macro resourcing of aid primarily as an inward concern, neglected recipient country needs, and made weak connections to international policy frameworks to benchmark, contextualise and rationalise aid allocations. The research suggests that the explanatory limitations of the countries’ news-models in communicating the processes and rationales underpinning macro aid resourcing may be a factor in sustaining a knowledge and legitimacy deficit among domestic publics for international aid agreements

Harvesting Invasive Plants to Reduce Nutrient Loads and Produce Bioenergy: An Assessment of Great Lakes Coastal Wetlands

In Laurentian Great Lakes coastal wetlands (GLCWs), dominant emergent invasive plants are expanding their ranges and compromising the unique habitat and ecosystem service values that these ecosystems provide. Herbiciding and burning to control invasive plants have not been effective in part because neither strategy addresses the most common root cause of invasion, nutrient enrichment. Mechanical harvesting is an alternative approach that removes tissue‐bound phosphorus and nitrogen and can increase wetland plant diversity and aquatic connectivity between wetland and lacustrine systems. In this study, we used data from three years of Great Lakes‐wide wetland plant surveys, published literature, and bioenergy analyses to quantify the overall areal extent of GLCWs, the extent and biomass of the three most dominant invasive plants, the pools of nitrogen and phosphorus contained within their biomass, and the potential for harvesting this biomass to remediate nutrient runoff and produce renewable energy. Of the approximately 212,000 ha of GLCWs, three invasive plants (invasive cattail, common reed, and reed canary grass) dominated 76,825 ha (36%). The coastal wetlands of Lake Ontario exhibited the highest proportion of invasive dominance (57%) of any of the Great Lakes, primarily from cattail. A single growing season\u27s biomass of these invasive plants across all GLCWs was estimated at 659,545 metric tons: 163,228 metric tons of reed canary grass, 270,474 metric tons of common reed, and 225,843 metric tons of invasive cattail, and estimated to contain 10,805 and 1144 metric tons of nitrogen and phosphorus, respectively. A one‐time harvest and utilization for energy of this biomass would provide the gross equivalent of 1.8 million barrels of oil if combusted, or 0.9 million barrels of oil if converted to biogas in an anaerobic digester. We discuss the potential for mitigating non‐point source nutrient pollution with invasive wetland plant removal, and other potential uses for the harvested biomass, including compost and direct application to agricultural soils. Finally, we describe the research and adaptive management program we have built around this concept, and point to current limitations to the implementation of large‐scale invasive plant harvesting

The architectures of media power: editing, the newsroom, and urban public space

This paper considers the relation of the newsroom and the city as a lens into the more general relation of production spaces and mediated publics. Leading theoretically from Lee and LiPuma’s (2002) notion of ‘cultures of circulation’, and drawing on an ethnography of the Toronto Star, the paper focuses on how media forms circulate and are enacted through particular practices and material settings. With its attention to the urban milieus and orientations of media organizations, this paper exhibits both affinities with but also differences to current interests in the urban architectures of media, which describe and theorize how media get ‘built into’ the urban experience more generally. In looking at editing practices situated in the newsroom, an emphasis is placed on the phenomenological appearance of media forms both as objects for material assembly as well as more abstracted subjects of reflexivity, anticipation and purposiveness. Although this is explored with detailed attention to the settings of the newsroom and the city, the paper seeks to also provide insight into the more general question of how publicness is material shaped and sited

Scheduling science on television: A comparative analysis of the representations of science in 11 European countries

While science-in-the-media is a useful vehicle for understanding the media, few scholars have used it that way: instead, they look at science-in-the-media as a way of understanding science-in-the-media and often end up attributing characteristics to science-in-the-media that are simply characteristics of the media, rather than of the science they see there. This point of view was argued by Jane Gregory and Steve Miller in 1998 in Science in Public. Science, they concluded, is not a special case in the mass media, understanding science-in-the-media is mostly about understanding the media (Gregory and Miller, 1998: 105). More than a decade later, research that looks for patterns or even determinants of science-in-the-media, be it in press or electronic media, is still very rare. There is interest in explaining the media’s selection of science content from a media perspective. Instead, the search for, and analysis of, several kinds of distortions in media representations of science have been leading topics of science-in-the-media research since its beginning in the USA at the end of the 1960s and remain influential today (see Lewenstein, 1994; Weigold, 2001; Kohring, 2005 for summaries). Only a relatively small amount of research has been conducted seeking to identify factors relevant to understanding how science is treated by the mass media in general and by television in particular. The current study addresses the lack of research in this area. Our research seeks to explore which constraints national media systems place on the volume and structure of science programming in television. In simpler terms, the main question this study is trying to address is why science-in-TV in Europe appears as it does. We seek to link research focussing on the detailed analysis of science representations on television (Silverstone, 1984; Collins, 1987; Hornig, 1990; Leon, 2008), and media research focussing on the historical genesis and current political regulation of national media systems (see for instance Hallin and Mancini, 2004; Napoli, 2004; Open Society Institute, 2005, 2008). The former studies provide deeper insights into the selection and reconstruction of scientific subject matters, which reflect and – at the same time – reinforce popular images of science. But their studies do not give much attention to production constraints or other relevant factors which could provide an insight into why media treat science as they do. The latter scholars inter alia shed light on distinct media policies in Europe which significantly influence national channel patterns. However, they do not refer to clearly defined content categories but to fairly rough distinctions such as information versus entertainment or fictional versus factual. Accordingly, we know more about historical roots and current practices of media regulation across Europe than we do about the effects of these different regimes on the provision of specific content in European societies

Vertical Structure of the Outer Accretion Disk in Persistent Low-Mass X-Ray Binaries

We have investigated the influence of X-ray irradiation on the vertical structure of the outer accretion disk in low-mass X-ray binaries by performing a self-consistent calculation of the vertical structure and X-ray radiation transfer in the disk. Penetrating deep into the disk, the field of scattered X-ray photons with energy $E\gtrsim10$\,keV exerts a significant influence on the vertical structure of the accretion disk at a distance $R\gtrsim10^{10}$\,cm from the neutron star. At a distance $R\sim10^{11}$\,cm, where the total surface density in the disk reaches $\Sigma_0\sim20$\,g\,cm$^{-2}$, X-ray heating affects all layers of an optically thick disk. The X-ray heating effect is enhanced significantly in the presence of an extended atmospheric layer with a temperature $T_{atm}\sim(2\div3)\times10^6$\,K above the accretion disk. We have derived simple analytic formulas for the disk heating by scattered X-ray photons using an approximate solution of the transfer equation by the Sobolev method. This approximation has a $\gtrsim10$\,% accuracy in the range of X-ray photon energies $E<20$\,keV.Comment: 19 pages, 8 figures, published in Astronomy Letter