A change in perspective: downhole cosmic-ray neutron sensing for the estimation of soil moisture

Above-ground cosmic-ray neutron sensing (CRNS) allows for the non-invasive estimation of the field-scale soil moisture content in the upper decimetres of the soil. However, large parts of the deeper vadose zone remain outside of its observational window. Retrieving soil moisture information from these deeper layers requires extrapolation, modelling or other methods, all of which come with methodological challenges. Against this background, we investigate CRNS for downhole soil moisture measurements in deeper layers of the vadose zone. To render calibration with in situ soil moisture measurements unnecessary, we rescaled neutron intensities observed below the terrain surface with intensities measured above a waterbody. An experimental set-up with a CRNS sensor deployed at different depths of up to 10 m below the surface in a groundwater observation well combined with particle transport simulations revealed the response of downhole thermal neutron intensities to changes in the soil moisture content at the depth of the downhole neutron detector as well as in the layers above it. The simulation results suggest that the sensitive measurement radius of several decimetres, which depends on soil moisture and soil bulk density, exceeds that of a standard active neutron probe (which is only about 30 cm). We derived transfer functions to estimate downhole neutron signals from soil moisture information, and we describe approaches for using these transfer functions in an inverse way to derive soil moisture from the observed neutron signals. The in situ neutron and soil moisture observations confirm the applicability of these functions and prove the concept of passive downhole soil moisture estimation, even at larger depths, using cosmic-ray neutron sensing.</p

Auditory time perception in Huntington’s disease

Huntington's disease (HD) is characterized by early involvement of the striatum. It affects the pace of repetitive motor activity, as motor timing depends on basal ganglia activity. However, data are lacking on the impact of this process on auditory time perception in motor non-affected gene carriers.Objective: This work aims to test the performance in time perception of a group of mutation carriers, either without motor symptoms or at an early stage of motor involvement. This should allow designing therapies targeting compensation strategies and possibly be used as a disease progression marker.Method: Time was assessed using two different tasks. An absolute, duration-based time perception was assessed in a first task and a relative, beat-based time perception was assessed in a second one. HD-mutation carriers with low-to-middle grades of motor involvement (HD-motor, n = 10) or without motor signs (HD-premotor n = 21), were compared with age- and sex-matched healthy controls (control (n = 27)). Thresholds of time difference perception where assessed.Results: For both tasks, poorer performances were found in HD-motor patients as compared with HD-premotor and controls. Thresholds of time difference perception correlated positively with the CAP score for the whole group of HD-gene carriers in both tasks. In a post-hoc exploratory analysis performed by a multiple regression, a negative correlation was found between the thresholds in both tasks and the Stroop interference test. Furthermore, in the first task, a positive correlation was found between thresholds and a trail making B test and a negative one with a total functional score.Conclusion: Our data confirm that the impairment in time perception in persons affected by HD correlates with the advancing disease. They also suggest that time perception depends on similar cognitive mechanisms as the ones sub-serving the Stroop interference test

CRIM-negative infantile Pompe disease: 42-month treatment outcome

Pompe disease is a rare lysosomal glycogen storage disorder characterized by deficiency of acid α-glucosidase enzyme (GAA) and caused by mutations in the GAA gene. Infantile-type Pompe disease is a multiorgan disorder presenting with cardiomyopathy, hypotonia, and muscular weakness, which is usually fatal. Enzyme replacement therapy (ERT) with recombinant human GAA (rhGAA) has recently been shown to be effective and subsequently yielded promising results in cross-reactive immunologic material (CRIM)-positive patients. CRIM-negative patients showed a limited response to ERT and died or were ventilator dependant. Over a period of 44 months, we monitored cognitive and motor development, behavior, auditory function, and brain imaging of a CRIM-negative infantile Pompe disease patient on rhGAA and monoclonal anti-immunoglobulin E (anti-IgE) antibody (omalizumab) treatment due to severe allergic reaction. Cardiorespiratory and skeletal muscle response was significant, with almost normal motor development. Cognitive development-in particular, speech and language-deviated increasingly from normal age-appropriate development and was markedly delayed at 44 months, unexplained by moderate sensorineural hearing impairment. Brain magnetic resonance imaging (MRI) at 18, 30, and 44 months of age revealed symmetrical signal alteration of the deep white matter. Titer values of IgG antibodies to rhGAA always remained <1:800. The potential role of omalizumab in immune modulation remains to be elucidated; however, this is the first report presenting a ventilator-free survival of a CRIM-negative patient beyond the age of 36 months. The central nervous system (CNS) findings are hypothesized to be part of a yet not fully described CNS phenotype in treated patients with longer survival

Comparative Live-Cell Imaging Analyses of SPA-2, BUD-6 and BNI-1 in Neurospora crassa Reveal Novel Features of the Filamentous Fungal Polarisome

A key multiprotein complex involved in regulating the actin cytoskeleton and secretory machinery required for polarized growth in fungi, is the polarisome. Recognized core constituents in budding yeast are the proteins Spa2, Pea2, Aip3/Bud6, and the key effector Bni1. Multicellular fungi display a more complex polarized morphogenesis than yeasts, suggesting that the filamentous fungal polarisome might fulfill additional functions. In this study, we compared the subcellular organization and dynamics of the putative polarisome components BUD-6 and BNI-1 with those of the bona fide polarisome marker SPA-2 at various developmental stages of Neurospora crassa. All three proteins exhibited a yeast-like polarisome configuration during polarized germ tube growth, cell fusion, septal pore plugging and tip repolarization. However, the localization patterns of all three proteins showed spatiotemporally distinct characteristics during the establishment of new polar axes, septum formation and cytokinesis, and maintained hyphal tip growth. Most notably, in vegetative hyphal tips BUD-6 accumulated as a subapical cloud excluded from the Spitzenkörper (Spk), whereas BNI-1 and SPA-2 partially colocalized with the Spk and the tip apex. Novel roles during septal plugging and cytokinesis, connected to the reinitiation of tip growth upon physical injury and conidial maturation, were identified for BUD-6 and BNI-1, respectively. Phenotypic analyses of gene deletion mutants revealed additional functions for BUD-6 and BNI-1 in cell fusion regulation, and the maintenance of Spk integrity. Considered together, our findings reveal novel polarisome-independent functions of BUD-6 and BNI-1 in Neurospora, but also suggest that all three proteins cooperate at plugged septal pores, and their complex arrangement within the apical dome of mature hypha might represent a novel aspect of filamentous fungal polarisome architecture

Intercomparison of cosmic-ray neutron sensors and water balance monitoring in an urban environment

Sensor-to-sensor variability is a source of error common to all geoscientific instruments that needs to be assessed before comparative and applied research can be performed with multiple sensors. Consistency among sensor systems is especially critical when subtle features of the surrounding terrain are to be identified. Cosmic-ray neutron sensors (CRNSs) are a recent technology used to monitor hectometre-scale environmental water storages, for which a rigorous comparison study of numerous co-located sensors has not yet been performed. In this work, nine stationary CRNS probes of type CRS1000 were installed in relative proximity on a grass patch surrounded by trees, buildings, and sealed areas. While the dynamics of the neutron count rates were found to be similar, offsets of a few percent from the absolute average neutron count rates were found. Technical adjustments of the individual detection parameters brought all instruments into good agreement. Furthermore, we found a critical integration time of 6 h above which all sensors showed consistent dynamics in the data and their RMSE fell below 1 % of gravimetric water content. The residual differences between the nine signals indicated local effects of the complex urban terrain on the scale of several metres. Mobile CRNS measurements and spatial simulations with the URANOS neutron transport code in the surrounding area (25 ha) have revealed substantial sub-footprint heterogeneity to which CRNS detectors are sensitive despite their large averaging volume. The sealed and constantly dry structures in the footprint furthermore damped the dynamics of the CRNS-derived soil moisture. We developed strategies to correct for the sealed-area effect based on theoretical insights about the spatial sensitivity of the sensor. This procedure not only led to reliable soil moisture estimation during dry-out periods, it further revealed a strong signal of intercepted water that emerged over the sealed surfaces during rain events. The presented arrangement offered a unique opportunity to demonstrate the CRNS performance in complex terrain, and the results indicated great potential for further applications in urban climate research

Factors increasing the risk for a severe reaction in anaphylaxis: An analysis of data from The European Anaphylaxis Registry

BACKGROUND Preventive measures to decrease the frequency and intensity of anaphylactic events are essential to provide optimal care for allergic patients. Aggravating factors may trigger or increase the severity of anaphylaxis and therefore need to be recognized and avoided. OBJECTIVE To identify and prioritize factors associated with an increased risk of developing severe anaphylaxis. METHODS Data from the Anaphylaxis Registry (122 centers in 11 European countries) were used in logistic regression models considering existing severity grading systems, elicitors, and symptoms to identify the relative risk of factors on the severity of anaphylaxis. RESULTS We identified higher age and concomitant mastocytosis (OR: 3.1, CI: 2.6-3.7) as the most important predictors for an increased risk of severe anaphylaxis. Vigorous physical exercise (OR: 1.5, CI: 1.3-1.7), male sex (OR: 1.2, CI: 1.1-1.3), and psychological burden (OR: 1.4, CI: 1.2-1.6) were more often associated with severe reactions. Additionally, intake of beta-blockers (OR: 1.9, CI: 1.5-2.2) and ACE-I (OR: 1.28, CI: 1.05, 1.51) in temporal proximity to allergen exposition was identified as an important factor in logistic regression analysis. CONCLUSION Our data suggest it may be possible to identify patients who require intensified preventive measures due to their relatively higher risk for severe anaphylaxis by considering endogenous and exogenous factors