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.

A developing application of laser-driven currents is the generation of magnetic fields of picosecond–nanosecond duration with magnitudes exceeding $B=10~\text{T}$. Single-loop and helical coil targets can direct laser-driven discharge currents along wires to generate spatially uniform, quasi-static magnetic fields on the millimetre scale. Here, we present proton deflectometry across two axes of a single-loop coil ranging from 1 to 2 mm in diameter. Comparison with proton tracking simulations shows that measured magnetic fields are the result of kiloampere currents in the coil and electric charges distributed around the coil target. Using this dual-axis platform for proton deflectometry, robust measurements can be made of the evolution of magnetic fields in a capacitor coil target.

A cluster of Salmonella Paratyphi B variant L(+) tartrate(+) infections with indistinguishable pulsed-field gel electrophoresis patterns was detected in October 2015. Interviews initially identified nut butters, kale, kombucha, chia seeds and nutrition bars as common exposures. Epidemiologic, environmental and traceback investigations were conducted. Thirteen ill people infected with the outbreak strain were identified in 10 states with illness onset during 18 July–22 November 2015. Eight of 10 (80%) ill people reported eating Brand A raw sprouted nut butters. Brand A conducted a voluntary recall. Raw sprouted nut butters are a novel outbreak vehicle, though contaminated raw nuts, nut butters and sprouted seeds have all caused outbreaks previously. Firms producing raw sprouted products, including nut butters, should consider a kill step to reduce the risk of contamination. People at greater risk for foodborne illness may wish to consider avoiding raw products containing raw sprouted ingredients.

The Amdeh Formation is a 3.4 km stack of sparsely fossiliferous quartzites and shales which crops out in the Al Hajar mountains near Muscat. Here we describe the uppermost member (Am5) that can be dated biostratigraphically as Darriwilian and which is the outcrop equivalent, and probably the seaward continuation, of the Saih Nihayda Formation in the Ghaba Salt Basin of northern Oman. The outcrops at Wadi Daiqa and Hayl al Quwasim consist of 690 m of quartzitic sandstones, shales and bivalve-rich shell beds. Trace fossils referable to the Cruziana and Skolithos ichnofacies abound. The member comprises storm-dominated shelf, shoreface and delta deposits. A number of new discoveries have been made in the outcrops: fragments of the arandaspid fish Sacabambaspis, ossicles and moulds of the early disparid crinoid Iocrinus, two new genera of conodont, an occurrence of the rare trinucleid trilobite Yinpanolithus, and palynological and sedimentological evidence of more continuous Floian–Darriwilian deposition than is usual in the region. Sea levels during Middle Ordovician time are estimated to have been 50–200 m above present levels and a wide, low-gradient shelf covered much of Arabia. Similar trace fossils and storm-dominated, micro-tidal, sedimentary rocks occur throughout the region. Small changes of sea level, possibly caused by the growth and melting of polar ice sheets, could lead to substantial seaward or landward shifts of facies belts. The Am5 deposits are thick compared to most equivalents in Arabia implying active subsidence and a ready supply of sediment.

The first experimental measurements of intense ( ${\sim }7\times 10^{19}~ {\rm W}~ {\rm cm}^{-2}$ ) laser-driven terahertz (THz) radiation from a solid target which is preheated by an intense pulse of laser-accelerated protons is reported. The total energy of the THz radiation is found to decrease by approximately a factor of 2 compared to a cold target reference. This is attributed to an increase in the scale length of the preformed plasma, driven by proton heating, at the front surface of the target, where the THz radiation is generated. The results show the importance of controlling the preplasma scale length for THz production.

Magnetic nanoparticle-vesicle aggregates (MNPVs), a controlled release nanostructure, have been enhanced with the inclusion of a novel galactose terminated lipid for cell targeting. Quartz crystal microgravimetry with dissipation (QCM-D) demonstrated that the galactose headgroup was available to bind Erythrina Crista-galli lectin (ECL) when the lipid was incorporated into a lipid bilayer. Similarly, UV-visible spectrophotometry indicated that ECL recognized the galactose headgroup in vesicles, leading to vesicle adhesion and aggregation. Finally, confocal fluorescence microscopy was used to assess the galactose-mediated interaction of both vesicles and MNPVs with HepG2 human hepatocellular carcinoma cells expressing the asialoglycoprotein (ASGPR) galactose receptor.

New angular diameter determinations for the bright southern F8 supergiant δ CMa enable the bolometric emergent flux and effective temperature of the star to be determined with improved accuracy. The spectral flux distribution and bolometric flux have been determined from published photometry and spectrophotometry and combined with the angular diameter to derive the bolometric emergent flux ℱ = (6.50 ± 0.24) × 107 Wm−2 and the effective temperature T eff = 5818 ± 53 K. The new value for the effective temperature is compared with previous interferometric and infrared flux method determinations. The accuracy of the effective temperature is now limited by the uncertainty in the bolometric flux rather than by the uncertainty in the angular diameter.

The current banking crisis has reminded us of how risks materialising in one part of the financial system can have a widespread impact, affecting other financial markets and institutions and the broader economy. This paper, prepared on behalf of the Actuarial Profession, examines how such events have an impact on the entire financial system and explores whether such disturbances may arise within the insurance and pensions sectors as well as within banking. The paper seeks to provide an overview of a number of banking and other financial crises which have occurred in the past, illustrated by four case studies. It discusses what constitutes a systemic event and what distinguishes it from a large aggregate system wide shock. Finally, it discusses how policy-makers can respond to the risk of such systemic financial failures.

Mathematical models are potentially useful tools to aid in the design of control programmes for parasitic diseases. In this paper, a fully age structured epidemiological model of human schistosomiasis is developed and parameterized, and used to predict trends in infection prevalence, intensity and prevalence of heavy infections over age and time during several rounds of mass and age targeted treatment. The model is validated against data from a Schistosoma mansoni control programme in Kenya.

The 1973 eruption on Heimaey, Iceland, presented the opportunity to study the interaction of the principal factors that control the formation of pyroclastic fall deposits. The grain size characteristics and the dispersal of some of the scoria fall units within the Eldfell Scoria deposit are described and related to observations made on the wind speed and direction, the height of the eruption column, the ‘muzzle’ velocity and the various styles of activity during the first month of the eruption.

We propose a new technique for evaluating the RF voltage across the electrodes of low-pressure capacitively-coupled plasma reactors when direct measurements are not possible. It is based on determining the coordinates of the turning point in the RF breakdown curve and using known values of the electron drift velocity for the gas. The results are in good agreement with those obtained by direct measurements at the driven electrode. Furthermore it allows RF breakdown curves to be determined for different frequencies, giving results that are physically reasonable (coincidence of right-hand branches) and in agreement with other published results. The technique for determining RF voltage we proposed is valid when there is no discharge plasma between electrodes (e.g., before gas breakdown), as well as for negligibly small discharge currents (before extinction of the weak-currentdischarge mode).

Active surveillance for laboratory-confirmed Salmonella serotype Enteritidis (SE) infection revealed a decline in incidence in the 1990s, followed by an increase starting in 2000. We sought to determine if the fluctuation in SE incidence could be explained by changes in foodborne sources of infection. We conducted a population-based case-control study of sporadic SE infection in five of the Foodborne Diseases Active Surveillance Network (FoodNet) sites during a 12-month period in 2002–2003. A total of 218 cases and 742 controls were enrolled. Sixty-seven (31%) of the 218 case-patients and six (1%) of the 742 controls reported travel outside the United States during the 5 days before the case's illness onset (OR 53, 95% CI 23–125). Eighty-one percent of cases with SE phage type 4 travelled internationally. Among persons who did not travel internationally, eating chicken prepared outside the home and undercooked eggs inside the home were associated with SE infections. Contact with birds and reptiles was also associated with SE infections. This study supports the findings of previous case-control studies and identifies risk factors associated with specific phage types and molecular subtypes.

The Keck Interferometer Nuller (KIN) is one of the major scientific and technical precursors to the Terrestrial Planet Finder Interferometer (TPF-I) mission. KIN's primary objective is to measure the level of exo-zodiacal mid-infrared emission around nearby main sequence stars, which requires deep broad-band nulling of astronomical sources of a few Janskys at 10 microns. A number of new capabilitites are needed in order to reach that goal with the Keck telescopes: mid-infrared coherent recombination, interferometric operation in “split pupil” mode, N-band optical path stabilization using K-band fringe tracking and internal metrology, and eventually, active atmospheric dispersion correction. We report here on the progress made implementing these new functionalities, and discuss the initial levels of extinction achieved on the sky.

The Kato-Katz method is widely used for diagnosing helminth infections in epidemiological surveys, but is known to have a low sensitivity. In the case of Schistosoma mansoni, statistical methods have been developed to compensate for the poor sensitivity, but the same is not true of any other helminth parasite, or infections with multiple-helminth species. We screened 101 schoolchildren from a rural area of Côte d'Ivoire over 5 consecutive days and made 5 Kato-Katz readings from each stool specimen. We estimated single and dual-species infections with S. mansoni and hookworm based on raw egg count data and after developing a latent-class model. The cumulative prevalence of co-infections was estimated at 9·9% after reading slides on the first day, and 57·0% after reading all 25 slides per person. The latent class model yielded a co-infection prevalence estimate of 79·6%, with marginal prevalence estimates for hookworm and S. mansoni infections of 83·9% and 91·6% respectively. The sensitivities of a single Kato-Katz thick smear for detection of S. mansoni alone, hookworms alone, or S. mansoni plus hookworms were 22·4%, 8·0% and 17·7%, respectively. In the current setting this could be attributable to low infection intensities of both parasites, combined with intra-specimen and day-to-day variation in egg output. If confirmed in other settings, these findings have implications for estimating the prevalence of multiple species helminth infections, and hence the design and implementation of efficacious and cost-effective control programmes.

The local structure of iron and uranium ions in a series of iron phosphate glasses with the general composition (40 – x)Fe2O3–xUO2–60P2O5 and (1–x–y)(40Fe2O3–60P2O5)– xUO2–y(Na2O or CaO) was investigated using Fe K-edge and U LIII-edge x-ray absorption fine structure spectroscopy. Replacing Fe2O3 by UO2 in the glass caused more distortion in the coordination environment of Fe(III) ions. Extended x-ray absorption fine structure fits revealed that the Fe–P bonds observed in the base glass also existed in all the waste-loaded glasses. X-ray absorption near-edge structure showed that the uranium ions were predominantly present as U(IV) in the glasses. Uranium ions were coordinated to approximately 8 ± 1 oxygen atoms and 2.5 ± 0.6 phosphorus atoms at an average distance of 2.47 ± 0.02 and 3.8 ± 0.02 Å, respectively. There were no Fe–U or U–Fe neighbors observed, indicating that uranium ions occupied voids in the glass away from the PO4 units. These conclusions were supported by Mössbauer, x-ray photoelectron, and Raman spectroscopic data.

The basal ganglia are a group of subcortical nuclei involved in a variety of processes including motor, cognitive and mnemonic functions. One of their major roles is to integrate sensorimotor, associative and limbic information in the production of context-dependent behaviours. These roles are exemplified by the clinical manifestations of neurological disorders of the basal ganglia. Recent advances in many fields, including pharmacology, anatomy, physiology and pathophysiology have provided converging data that have led to unifying hypotheses concerning the functional organisation of the basal ganglia in health and disease. The major input to the basal ganglia is derived from the cerebral cortex. Virtually the whole of the cortical mantle projects in a topographic manner onto the striatum, this cortical information is ‘processed’ within the striatum and passed via the so-called direct and indirect pathways to the output nuclei of the basal ganglia, the internal segment of the globus pallidus and the substantia nigra pars reticulata. The basal ganglia influence behaviour by the projections of these output nuclei to the thalamus and thence back to the cortex, or to subcortical ‘premotor’ regions. Recent studies have demonstrated that the organisation of these pathways is more complex than previously suggested. Thus the cortical input to the basal ganglia, in addition to innervating the spiny projection neurons, also innervates GABA interneurons, which in turn provide a feed-forward inhibition of the spiny output neurons. Individual neurons of the globus pallidus innervate basal ganglia output nuclei as well as the subthalamic nucleus and substantia nigra pars compacta. About one quarter of them also innervate the striatum and are in a position to control the output of the striatum powerfully as they preferentially contact GABA interneurons. Neurons of the pallidal complex also provide an anatomical substrate, within the basal ganglia, for the synaptic integration of functionally diverse information derived from the cortex. It is concluded that the essential concept of the direct and indirect pathways of information flow through the basal ganglia remains intact but that the role of the indirect pathway is more complex than previously suggested and that neurons of the globus pallidus are in a position to control the activity of virtually the whole of the basal ganglia.

Fe K-edge x-ray absorption fine-structure (XAFS) measurements were performed on glass samples of (Fe3O4)0.3(P2O5)0.7 with various amounts of Na2O or UO2. Near-edge and extended XAFS regions are studied and comparisons are made to several reference compounds. We find that iron in the base glass is ∼25% divalent and that the Fe2+ coordination is predominantly octahedral, while Fe3+ sites are roughly split between tetrahedral and octahedral coordinations. Also, we measure roughly one Fe–O–P link per iron. Substitution of Na2O or UO2 up to 15 mol% primarily affects the first Fe–O shell. The results are compared to data from the related material Fe3(P2O7)2.

The technique of Chirped Pulse Amplification (CPA) developed by Strickland and Mourou (1985) is now in common use on many laser systems [see, for instance, the review by Perry & Mourou (1994)] and has resulted in massive increases in focused intensities. This paper describes CPA implementation on the Vulcan laser system which has generated multi-Joule sub-picosecond pulses whilst maintaining beam quality to produce focused intensities of 5 × 1019 Wcm−2.