17,276 research outputs found
Andersen-Tawil Syndrome
Andersen-Tawil syndrome (ATS) is a rare condition consisting of ventricular arrhythmias, periodic paralysis, and dysmorphic features. In 2001, mutations in KCNJ2, which encodes the α subunit of the potassium channel Kir2.1, were identified in patients with ATS. To date, KCNJ2 is the only gene implicated in ATS, accounting for approximately 60% of cases. ATS is a unique channelopathy, and represents the first link between cardiac and skeletal muscle excitability. The arrhythmias observed in ATS are distinctive; patients may be asymptomatic, or minimally symptomatic despite a high arrhythmia burden with frequent ventricular ectopy and bidirectional ventricular tachycardia. However, patients remain at risk for life-threatening arrhythmias, including torsades de pointes and ventricular fibrillation, albeit less commonly than observed in other genetic arrhythmia syndromes. The characteristic heterogeneity at both the genotypic and phenotypic levels contribute to the continued difficulties with appropriate diagnosis, risk stratification, and effective therapy. The initial recognition of a syndromic association of clinically diverse symptoms, and the subsequent identification of the underlying molecular genetic basis of ATS has enhanced both clinical care, and our understanding of the critical function of Kir2.1 on skeletal muscle excitability and cardiac action potentia
Tolerable versus actual soil erosion rates in Europe
Erosion is a major threat to soil resources in Europe, and may impair their ability to deliver a range of ecosystem goods and services. This is reflected by the European Commission's Thematic Strategy for Soil Protection, which recommends an indicator-based approach for monitoring soil erosion. Defined baseline and threshold values are essential for the evaluation of soil monitoring data. Therefore, accurate spatial data on both soil loss and soil genesis are required, especially in the light of predicted changes in climate patterns, notably frequency, seasonal distribution and intensity of precipitation. Rates of soil loss are reported that have been measured, modelled or inferred for most types of soil erosion in a variety of landscapes, by studies across the spectrum of the Earth sciences. Natural rates of soil formation can be used as a basis for setting tolerable soil erosion rates, with soil formation consisting of mineral weathering as well as dust deposition. This paper reviews the concept of tolerable soil erosion and summarises current knowledge on rates of soil formation, which are then compared to rates of soil erosion by known erosion types, for assessment of soil erosion monitoring at the European scale
Timing of poverty in childhood and adolescent health: Evidence from the US and UK
Childhood poverty is associated with poorer adolescent health and health behaviours, but the importance of the timing of poverty remains unclear. There may be critical or sensitive periods in early life or early adolescence, or poverty may have cumulative effects throughout childhood. Understanding when poverty is most important can support efficient timing of interventions to raise family income or buffer against the effects of low income, but answers may vary across social contexts. The US and the UK are a useful comparison with similar liberal approaches to cash transfers, but very different approaches to healthcare provision. Utilising data from large population studies in the US (n = 9408; born 1979–1996) and UK (n = 1204; born 1991–1997), this study employs a structured life course approach to compare competing hypotheses about the importance of the timing or pattern of childhood exposure to poverty in predicting adolescent health limitations, symptoms of psychiatric distress, and smoking at age 16 (age 15/16 in US). Household income histories identified experience of poverty (measured as <60% of the national median equivalised income for a given year) in early life (ages 0–5), mid-childhood (ages 6–10) and early adolescence (ages 11–15). The Bayesian Information Criterion (BIC) compared fit across models with variables representing different life course patterns of exposure to poverty. Adolescent distress was not associated with poverty in either country. In both countries, however, variables representing cumulative or persistent experiences of poverty exhibited optimal fit of all poverty exposure variables in predicting adolescent smoking and health limitations. There was also evidence of an early life sensitive period for smoking in the US. Poverty was more persistent in the US, but associations between poverty and outcomes were consistent across countries. Although poverty can have cumulative effects on health and behaviour, early interventions may offer the best long-term protection
Global auroral responses to magnetospheric compressions by shocks in the solar wind: Two case studies
The global auroral responses to shocks in the solar wind at Earth were studied. The z-component of the interplanetary magnetic field, Bz, is negative ahead and behind the first shock and positive for the second case. A sudden-commencement geomagnetic storm develops in each case, with maximum D sub st 190 nT. An immediate auroral response is detected at all longitudes around the auroral oval, in which auroral luminosities increase by a factor of 2 to 3 with the first samples after each sudden commencement. The time delay in obtaining the first sample varies with local time from approx. 1 to 18 mins. No other significant variations in the aurora are associated with the immediate response. Beginning approx. 30 mins after each sudden commencement, the aurora becomes active and displays significant variations in its luminosity and spatial distribution. For Bz 0 an intense substorm develops. A sun-aligned transpolar arc forms when Bz 0, appearing first at local midnight as a polar arc and then lengthening sunward from the auroral oval across the polar cap to noon at an average speed of approx. 1 km/sec
Arrival Timing and Seasonal Reproductive Performance in a Long-Distance Migratory Landbird
The date when a landbird migrant arrives on its breeding grounds may have reproductive consequences. Generally, early arriving individuals begin breeding earlier and consequently experience greater seasonal reproductive performance. Here, we describe relationships between arrival timing and seasonal reproductive performance in the American redstart (Setophaga ruticilla), a long-distance passerine migrant, arriving at northerly breeding grounds in Michigan’s eastern Upper Peninsula. Evidence suggests that both males and females benefited from early arrival at the breeding grounds. Early males appeared to settle on higher quality territories and hatched nestlings sooner than later arrivals. Early females began their clutches early, produced heavier nestlings and possibly laid more eggs than later arrivals. Larger clutches and heavier offspring increase the likelihood of offspring recruiting into the breeding population. The findings of this study point to fitness consequences arising from when a bird arrives at its breeding grounds. These results also have implications for understanding how events occurring during spring migration influence reproductive performance as migratory delays likely influence arrival timing
Effective risk governance for environmental policy making: a knowledge management perspective
Effective risk management within environmental policy making requires knowledge on natural, economic and social systems to be integrated; knowledge characterised by complexity, uncertainty and ambiguity. We describe a case study in a (UK) central government department exploring how risk governance supports and hinders this challenging integration of knowledge. Forty-five semi-structured interviews were completed over a two year period. We found that lateral knowledge transfer between teams working on different policy areas was widely viewed as a key source of knowledge. However, the process of lateral knowledge transfer was predominantly informal and unsupported by risk governance structures. We argue this made decision quality vulnerable to a loss of knowledge through staff turnover, and time and resource pressures. Our conclusion is that the predominant form of risk governance framework, with its focus on centralised decision-making and vertical knowledge transfer is insufficient to support risk-based, environmental policy making. We discuss how risk governance can better support environmental policy makers through systematic knowledge management practices
A Performance Assessment of an Airborne Separation Assistance System Using Realistic Complex Traffic Flows
This paper presents the results from a study that investigates the performance of a tactical Airborne Separation Assistance System (ASAS) in en route airspace, under varying demand levels, with realistic traffic flows. The ASAS concept studied here allows flight crews of equipped aircraft to perform separation from other air traffic autonomously. This study addresses the tactical aspects of an ASAS using aircraft state data (i.e. position and velocity) to detect and resolve projected conflicts. In addition, use of a conflict prevention system helps ASAS-equipped aircraft avoid maneuvers that may cause new conflicts. ASAS-capable aircraft are equipped with satellite-based navigation and Automatic Dependent Surveillance Broadcast (ADS-B) for transmission and receipt of aircraft state data. In addition to tactical conflict detection and resolution (CD&R), a complete, integrated ASAS is likely to incorporate a strategic CD&R component with a longer look-ahead time, using trajectory intent information. A system-wide traffic flow management (TFM) component, located at the FAA command center helps aircraft to avoid regions of excessive traffic density and complexity. A Traffic Alert and Collision Avoidance System (TCAS), as used today is the system of last resort. This integrated approach avoids sole reliance on the use of the tactical CD&R studied here, but the tactical component remains a critical element of the complete ASAS. The focus of this paper is to determine to what extent the proposed tactical component of ASAS alone can maintain aircraft separation at demand levels up to three times that of current traffic. The study also investigates the effect of mixing ASAS-equipped aircraft with unequipped aircraft (i.e. current day) that do not have the capability to self-separate. Position and velocity data for unequipped aircraft needs to be available to ASASequipped. Most likely, for this future concept, state data would be available from instrument flight rules (IFR) aircraft, equipped with at least ADS-B transmission capability. The objective is to reduce the number of losses of separation to a minimum and investigate the limits of tactical-only CD&R. Thus, the objective is not, expressly, to achieve zero losses of separation with tactical ASAS because this is one component of an integrated ASAS
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