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Mitochondrial trifunctional protein deficiency is a long-chain fatty acid disorder that may include manifestations of severe cardiomyopathy and arrhythmias. The pathophysiology for the severe presentation is unclear but is an indicator for worse outcomes. Triheptanoin, a synthetic medium chain triglyceride, has been reported to reverse cardiomyopathy in some individuals, but there is limited literature in severe cases. We describe a neonatal onset of severe disease whose clinical course was not improved despite mechanical support and triheptanoin.
The Cognitive Bias (CogBIAS) hypothesis proposes that cognitive biases develop as a function of environmental influences (which determine the valence of biases) and the genetic susceptibility to those influences (which determines the potency of biases). The current study employed a longitudinal, polygenic-by-environment approach to examine the CogBIAS hypothesis. To this end, measures of life experiences and polygenic scores for depression were used to assess the development of memory and interpretation biases in a three-wave sample of adolescents (12–16 years) (N = 337). Using mixed effects modeling, three patterns were revealed. First, positive life experiences (PLEs) were found to diminish negative and enhance positive forms of memory and social interpretation biases. Second, and against expectation, negative life experiences and depression polygenic scores were not associated with any cognitive outcomes, upon adjusting for psychopathology. Finally, and most importantly, the interaction between high polygenic risk and greater PLEs was associated with a stronger positive interpretation bias for social situations. These results provide the first line of polygenic evidence in support of the CogBIAS hypothesis, but also extend this hypothesis by highlighting positive genetic and nuanced environmental influences on the development of cognitive biases across adolescence.
The study of political participation in Latin America has, until very recently, been too narrowly conceived by social scientists, focusing largely on elites and violence. The former is illustrated by studies of the military (Lieuwen, 1961, 1966; Johnson, 1964; Horowitz, 1967; Fagen and Cornelius, 1970; Schmitter, 1973), the Church (Dillon Soares, 1967; Solari, 1967; Petersen, 1970; Suchlicki, 1972), industrialists (Cardoso, 1967; Polit, 1968; Petras and Cook, 1973), and large landholders (Whetten, 1948; Carroll, 1966; Feder, 1971; Cockroft, 1972). Attention to violent forms of political participation is found in studies of revolution and the military coup d'état (Payne, 1965; Needler, 1968; Von Lazar and Kaufman, 1969; Moreno and Mitrani, 1971; Kohl and Litt, 1974). Those studies that have centered on nonviolent mass participation (Horowitz, 1970) have generally been limited to elections (for example, Martz, 1967; Petras, 1970), political parties (Fitzgibbon, 1957; Ciria, 1974), and labor unions (Payne, 1965; Angell, 1972; Erickson et al., 1974). As a result of the narrowness of these approaches, we have only a partial image of the faces of the Latin American citizen political activity; we have underestimated the scope of such activity and have failed to investigate its many forms.
Despite calls to improve and systematize research on political participation in Latin America more than a decade ago (Kling 1964, Flores Olea 1967), the burgeoning literature on the subject has yet to achieve full recognition. Thus certain contradictory and incomplete traditional images still linger in the scholarly literature (Booth and Seligson 1978a). These treatments vary dramatically and almost bewilderingly: while one suggests that Latin Americans are becoming increasingly politically mobilized, two others hold that mass participation is very low and that most political activity is restricted to socioeconomic elites. Other images portray mass political participation as irrational and dwell upon political violence. Such familiar notions have often intertwined. For example, a common picture depicts most Latin Americans, and especially peasants, as politically passive and quiescent until provoked, when they may burst violently into the political arena (for example, see Forman 1971, Singelmann 1975, Handelman 1975b, Moreno 1970). Similarly Wiarda (1974, pp. 4–5) discusses how the image of mobilization often combines with that of violence, producing the notion that the rising political awareness and participation of Latin Americans leads inexorably to ever greater levels of conflict (e.g., Schmitt and Burks 1963, Hadley 1958, Petras and Zeitlin 1968, Petras 1968).
Despite certain early efforts to interpret Mexico as a pluralist constitutional democracy, or democracy-in-the-making (Scott 1959; Tucker 1957), scholars today almost universally agree that the political system of Mexico is authoritarian. The trappings of Mexico's liberal constitution and elections notwithstanding, Mexico's Partido Revolucionario Institucional (PRI) serves to integrate the polity under the highly centralized control of a single institution that dominates access to all public office. At the apex of the PRI is the Mexican president, who not only chooses his own successor but controls access to the PRI's candidate lists for all other public offices and therefore dominates both the party and the congress. In sum, as Coleman and Davis argue, Mexico fits the ideal type of authoritarian political organization because “decisions are made almost exclusively by the ruling elite rather than by democratic, pluralist processes” and because “there are severe restrictions placed upon political mobilization” (1976, 195).
Since the mid-1970s, tens of thousands of persons in three out of the five Central American countries have revolted against their governments or fought to repress such rebellions. These conflicts have cost more than a quarter of a million lives and created more than two million internal and external refugees. In 1979 a bloody insurrection toppled Nicaragua's Somoza regime. El Salvador's crippling civil war has escalated and reescalated but remains stalemated. In Guatemala since 1980, brutal counterinsurgency warfare, pro-regime terror, and political reform have failed to eliminate a resurgent guerrilla rebellion. Yet while these countries have rent themselves with political violence, their neighbors Honduras and Costa Rica have in general remained politically peaceful.
When can a hyphen be described as ‘intrusive’? As with many other ‘rules’ of grammar and of punctuation, use of the hyphen is limited to a specific syntactical context. Hence, to use a hyphen where it should not be used makes it intrusive. Just like the apostrophe, it cannot be used arbitrarily. There is, for instance, a tattooist parlour in the English town of Oldham whose shopfront advertises Inkcredible Tatoo's (sic) and, with it, both the owner's or owners’ ability to play on words in writing and their inability to sign a plural form correctly – the rule being that apostrophes are not used to mark the plural of either common or proper nouns in written English.
Coups d’état, once a common end for democracies in the Americas, have declined sharply in recent years. This article investigates whether overall public support for coups is also in decline. Examining 21 countries in Latin America and the Caribbean from 2004 to 2014 helps to evaluate two alternative theses on democratization: Mainwaring and Pérez-Liñán’s 2013 normative regime preferences theory, which inquires (but does not test) whether public opinion can signal to elites a reluctance or willingness to support a coup; and classic modernization theory (Inglehart 1988; Inglehart and Welzel 2005). We find a substantively meaningful effect of democratic attitudes on coup support and a weak effect for national wealth, from which we infer that evolving elite values and preferences are paralleled at the mass level and that together, those two trends play a stronger role in the consolidation of democratic regimes than does modernization.
OBJECTIVES/SPECIFIC AIMS: Clinical guidelines recommend using predicted atherosclerotic cardiovascular disease (ASCVD) risk to inform treatment decisions. The objective was to compare the contribution of changes in modifiable risk factors Versus aging to the development of high 10-year predicted ASCVD risk. METHODS/STUDY POPULATION: Prospective follow-up of the Jackson Heart Study, an exclusively African-American cohort, at visit 1 (2000–2004) and visit 3 (2009–2012). Analyses included 1115 African-American participants without a high 10-year predicted ASCVD risk (<7.5%), hypertension, diabetes, or ASCVD at visit 1. We used the Pooled Cohort equations to calculate the incidence of high (≥7.5%) 10-year predicted ASCVD risk at visit 3. We recalculated the percentage with a high 10-year predicted ASCVD risk at visit 3 assuming each risk factor [age, systolic blood pressure (SBP), antihypertensive medication use, diabetes, smoking, total and high-density lipoprotein cholesterol], one at a time, did not change from visit 1. RESULTS/ANTICIPATED RESULTS: The mean age at visit 1 was 45.2±9.5 years. Overall, 30.9% (95% CI 28.3%–33.4%) of participants developed high 10-year predicted ASCVD risk. Aging accounted for 59.7% (95% CI 54.2%–65.1%) of the development of high 10-year predicted ASCVD risk compared with 32.8% (95% CI 27.0%–38.2%) for increases in SBP or antihypertensive medication initiation and 12.8% (95% CI 9.6%–16.5%) for incident diabetes. Among participants <50 years, the contribution of increases in SBP or antihypertensive medication initiation was similar to aging. DISCUSSION/SIGNIFICANCE OF IMPACT: Increases in SBP and antihypertensive medication initiation are major contributors to the development of high 10-year predicted ASCVD risk in African Americans, particularly among younger adults.
There can be no doubt that the current industrial foodsystem is broken. Examples of large-scale diseaseoutbreaks such as bovine spongiform encephalopathy(BSE), and scandals such as melamine-taintedpowdered milk and horsemeat in frozen beef lasagnestill linger in the public consciousness. Asprevious chapters have illustrated, perhaps a morefitting descriptor of the global food system wouldbe that of a system of ‘organised crime’. Organisedcrime refers to illegal activities or harmfuloffences at transnational or national levelundertaken purely for profit. The charge sheet fororganised crime by the global food system documentsa litany of harmful or illegal activities againstthe environment, consumers, food producers andagricultural workers, public health and animalwelfare. Against this background consumers areincreasingly seeking truth and transparency as tothe provenance of their food.
This chapter explores ‘counter crime’, namely,activities that prevent or defend against criminalactions. These actions, some underpinned bydemocratic principles, constitute a participatorymovement whereby citizens exert some modicum ofcontrol over their food system. Collectively knownas food democracy, it offers a quantum of hope inwhat Hinrichs (2007, pp 5–6) calls ‘re-making’ thefood system. Hinrichs argues that ‘remaking shiftsus from a paralysing focus on what is worrying,wrong, destructive and oppressive about our currentfood system to a wide-angle view that takes in thebroader landscape whose troubling contours we beginto notice, are punctuated by encouraging signs ofchange.’ Starting with an investigation of foodconsumption crimes, this chapter explores examplesof citizen ‘counter crime’ movements usingoppositional politics to galvanise action, thenfocusing on food democracy and constructivistapproaches to remake the food system.
Crimes of consumption
Crime in this context does not relate to activitiesthat are law-breaking as such, but rather to thosewho use their powers to subvert standards of decencyand fair play. Use of these powers, especially whenturned on those who have no ability to fight back,can be considered criminal in a broad sense. InChapter 1 of this volume, Gray suggests a food crimeperspective is one that may include ‘situations oflaw-making or law-breaking, suspect or ineffectiveenforcement or lack thereof, harms resulting frominsufficient or absent regulation, or philosophicaland pragmatic questions of corruption, deviance,justice and erroneousness.’ Three examples areexplored below.
We study the asset allocation decision of a life insurance company’s general account with respect to the possibility of large negative economic shocks and examine how this account is affected by policyholder investment decisions in the company’s separate account. This is accomplished using a performance metric that incorporates downside risk measured using univariate and multivariate extreme value distributions. Because of its well-known price volatility, diversification attributes, and significant weight in the combined general and separate accounts, our primary focus is the company’s equity investments. Although industry asset allocations have varied over the past two decades, we find that the actual allocations to equity in the general account are close to the allocation percentages suggested by our extreme value metrics and both are far below the maximum values indicated by the relevant regulatory bodies.
The Neotoma Paleoecology Database is a community-curated data resource that supports interdisciplinary global change research by enabling broad-scale studies of taxon and community diversity, distributions, and dynamics during the large environmental changes of the past. By consolidating many kinds of data into a common repository, Neotoma lowers costs of paleodata management, makes paleoecological data openly available, and offers a high-quality, curated resource. Neotoma’s distributed scientific governance model is flexible and scalable, with many open pathways for participation by new members, data contributors, stewards, and research communities. The Neotoma data model supports, or can be extended to support, any kind of paleoecological or paleoenvironmental data from sedimentary archives. Data additions to Neotoma are growing and now include >3.8 million observations, >17,000 datasets, and >9200 sites. Dataset types currently include fossil pollen, vertebrates, diatoms, ostracodes, macroinvertebrates, plant macrofossils, insects, testate amoebae, geochronological data, and the recently added organic biomarkers, stable isotopes, and specimen-level data. Multiple avenues exist to obtain Neotoma data, including the Explorer map-based interface, an application programming interface, the neotoma R package, and digital object identifiers. As the volume and variety of scientific data grow, community-curated data resources such as Neotoma have become foundational infrastructure for big data science.
A longstanding scholarly debate asks what lasting changes revolution makes in political attitudes and behaviors. Scholars generally regard revolution as transformative, but research on revolution's behavioral and cultural legacies after transfers of state power has been limited. This study explores revolution's residual impact on individuals' electoral participation, civil society engagement, and social capital by comparing postrevolutionary Nicaraguans to their Central American neighbors. Using survey data on comparable samples of urban Central Americans, the research found higher electoral engagement in Nicaragua than in the other nations in the region that experienced major insurgencies but not revolution. It also found among Nicaraguans greater support for civil disobedience, greater school and union activism, and more frequent leftist political identification. Although revolution did leave residual effects in Nicaragua, more significant influence appears to have decayed rapidly.
Robert Putnam extolled the virtue of social capital by arguing that social networks, civil society, and trust contribute to democracy. Subsequent research, however, identified a weakness in the social capital “model” in its underspecification of the mechanisms by which social capital affects political systems. This article proposes the concept of political capital as a likely product of social capital that links civil society participants to the political system. The article tests this two-stage model of social capital and political capital and their effects on democratization using survey data from eight Latin American nations. Results find that civil society engagement in 2004 affected political capital variables, which, in turn, had positive effects on system-level democracy measures in 2010. The article thus shows that political capital serves as an intervening variable between social capital and democracy and democratization.
Acoustic sub-bottom profiler surveys on the northeast Antarctic Peninsula shelf indicate that parts of the seabed are underlain by an acoustically transparent layer that is thin on the inner shelf and becomes thicker and more extensive towards the outer shelf. Sedimentological and geophysical data are combined to construct a bed model where streaming ice flow, by both deformation and basal sliding, took place within cross-shelf troughs. The model suggests only limited deformation contributed to fast flow on the inner shelf, i.e. in the onset zone of ice streaming, where the bed was predominantly underlain by a stiff till. Thus, fast ice flow in this area might have been by basal sliding, with deformation confined to discontinuous patches of soft till <40 cm thick. Towards the middle and outer shelf, extensive, thick sequences of soft till suggest a change in the dominant subglacial process towards widespread deformation. This downstream change from basal sliding to subglacial deformation is manifest in the transition from stiff-till dominance to soft-till dominance, while a downstream increase in ice flow velocity is evident from the complex geomorphic imprint on the inner shelf evolving to the more restricted set of bedforms on the outer shelf.
This study investigated factors that influence occurrence and persistence of plant DNA in the soil environment in three crop rotations. In each rotation, soil was sampled in May before planting, in July and August while crops were growing, and in October after harvest. Total DNA was recovered from soil samples taken at two different depths in the soil profile and quantified. Three target plant genes (corn CP4 epsps, corn 10-kD Zein, and soybean CP4 epsps) also were quantified in these DNA extracts using species-specific quantitative real-time PCR assays. In general, total plant DNA content in the soil environment was greatest when the crop was growing in the field and decreased rapidly after harvest. Nevertheless, low levels of target plant DNA were often still detectable the following spring. Age of rotation did not influence target DNA quantities found in the soil environment. Data were collected for a combination of 10 location-years, which allowed for estimation of the variance components for six factors including time of sampling, year, location, crop, sampling depth, and herbicide to total and target DNA content in the soil samples. Mean target recombinant DNA content in soil was influenced most strongly by time of sampling and year (85 and 6%, respectively), whereas total soil DNA content was less dynamic and was most strongly influenced by location and year (49 and 25%, respectively). Over the duration of this study, no accumulation of transgenic plant DNA in the soil environment was observed.
The Protoplanetary Discussions conference—held in Edinburgh, UK, from 2016 March 7th–11th—included several open sessions led by participants. This paper reports on the discussions collectively concerned with the multi-physics modelling of protoplanetary discs, including the self-consistent calculation of gas and dust dynamics, radiative transfer, and chemistry. After a short introduction to each of these disciplines in isolation, we identify a series of burning questions and grand challenges associated with their continuing development and integration. We then discuss potential pathways towards solving these challenges, grouped by strategical, technical, and collaborative developments. This paper is not intended to be a review, but rather to motivate and direct future research and collaboration across typically distinct fields based on community-driven input, to encourage further progress in our understanding of circumstellar and protoplanetary discs.