EnergyAustralia (Australia)

CLN3 disease, or Juvenile Neuronal Ceroid Lipofuscinosis (JNCL), is a rare, genetic neurodegenerative condition, typically manifesting in the first decade of life and progressing in severity, with death typically occurring in early adulthood. Despite two decades of natural history research, a clear timeline of CLN3 disease symptom onset and progression remains poorly defined, limiting optimal patient management and therapeutic development. We conducted a literature review and analysed the natural history data to better understand the age of core symptom onset and chronological disease progression. A literature review was undertaken using a pre-defined search strategy focused on CLN3 disease natural history studies, where age at onset for one or more core symptoms was reported in cohorts of ≥ 15 subjects. For each symptom, weighted mean age at onset and weighted standard deviation were calculated, with 95% confidence intervals derived from the weighted standard error. Symptom onset ages were compared using ANOVA. We identified nine natural history studies that met our pre-defined criteria. In total, 423 discrete patients aged between 4 and 39 years were reported. Thirteen core symptoms and a weighted average age at onset and weighted standard deviation were (in years): vision loss (6.1 ± 1.6, N = 254), behavioural changes (8.5 ± 3.9, N = 194), cognitive decline (9.3 ± 3.1, N = 219), seizures (10.2 ± 3.0, N = 243), sleep disturbance (11.0 ± 6.1, N = 111), motor decline (11.0 ± 3.8, N = 108), complete blindness (11.4 ± 3.6, N = 171), speech and language impairment (12.7 ± 4.8, N = 136), Parkinsonian gait (14.1 ± 2.5, N = 111), cardiac manifestations (17.8 ± 4.4, N = 45), loss of independent walking (19.5 ± 3.2, N = 70), feeding difficulties requiring enteral feeding tube (22.0 ± 1.6, N = 35), and death (22.4 ± 4.4, N = 95). This comprehensive summary of available natural history data illustrates mean age at onset of 13 core symptoms of CLN3 disease, and characterises a chronological timeline of disease progression. These results provide much-needed practical, anticipatory guidance to those involved in caring for individuals with CLN3 disease, and serve to highlight the critical importance of collecting globally standardised, quantifiable, longitudinal data for optimising patient management and advancing therapeutic approaches for CLN3 disease.

Lightweight structures, such as residential foundations, built on reactive soils experience stability issues due to the soil’s high shrink-swell movements. This study investigated the performance of a full-scale waffle raft foundation (~ 8 m x ~ 10 m) built on a highly reactive site. Spatiotemporal variations of the soil moisture content and temperature outside and underneath the foundation were monitored for more than a year. The shallow soil temperature beneath the foundation fluctuated in response to seasonal weather changes, but no significant spatial variations were observed. Seasonal weather conditions showed a dominant effect on the soil temperature variation outside and beneath the foundation at any depth, and intermittent rainfall events outside the wet/winter season displayed no significant impact on this. The fluctuations in soil moisture content diminish towards the centre of the foundation, although a notable fluctuation is still evident beneath its centre. At varying depths beneath the middle of the foundation, seasonal fluctuations in soil moisture content were observed up to a depth of 2.3 m, but the initial moisture content differences at different depths remained consistent. Overall, this study recommends revisiting the standard design procedure for waffle raft systems in terms of edge distance and depth of design soil suction change determinations.

Abstract Effective water and wetland management for waterbirds during nesting events benefits from knowledge of nesting timing, staging, duration, and associated foraging locations. Such information can inform managers about when, where, and for how long management actions such as environmental watering or predation protection are needed to support specific nesting stages. This is particularly relevant for species nesting in aggregations that are dependent on flooding of key sites to initiate and complete their nesting cycles, such as ibis and spoonbills. These species are frequently targeted for adaptive water and wetland management to support successful breeding events and can be bioindicators of wetland change. However, obtaining on-ground nesting data for highly mobile species in remote or inaccessible sites can be difficult. To solve this problem, we used GPS satellite telemetry to track nesting events for 3 species that frequently nest together in remote wetlands managed with environmental water: Threskiornis spinicollis (Straw-necked Ibis), T. molucca (Australian White Ibis), and Platalea regia (Royal Spoonbill). This is the first time that satellite telemetry for these highly mobile species has been used to analyze: (1) nest stages and nest attendance patterns; (2) distances travelled to forage during nesting; and (3) timing and duration of nesting events. We found both intra- and inter-species variation in nest attendance patterns and foraging distances. There were changes in nest attendance and foraging distance patterns corresponding with transitions between each of the 4 main nest stages: (1) nest establishment; (2) incubation; (3) immobile chicks; (4) mobile chicks. We discuss how this information can be used to implement strategic conservation management of waterbird populations and habitats including environmental watering, nest protection, and predator control.

Underground copper (Cu)-based infrastructure, including pipelines, cables, wiring and large-scale appliances are frequently situated near electrical sources, including power sources, railway systems, cathodic protection devices and domestic circuits. Currents may ‘stray’ from electrical circuits associated with these sources into surrounding soil. Such stray currents can create voltage differences across the Cu assets, thus leading to corrosion. In this study, we aim to experimentally simulate stray current corrosion of Cu to mimic real-world conditions using a laboratory electrochemical cell with different types of soils. The stray current corrosion was achieved by applying voltages between two titanium (Ti) electrodes with Cu samples placed in between them. This cell-within-cell setup was employed to investigate the influence of soil conductivity and the distance between electrodes and the electrical source on the stray current corrosion of Cu.

A free-standing thin sheet of carbon grapes (TSCG) is used as a replacement for copper in anode-free lithium metal batteries (ALMBs). The results show that TSCG functions effectively both as a current collector and as a lithium reservoir.

Hybrid solid-state electrolyte (HSSE) is a key component for the advancement of all-solid-state lithium metal batteries. The key challenges with the existing HSSEs are to ensure high ionic conductivity, activate dead inorganic/organic interface, and stimulate interfacial stability with electrodes. In this study, a novel HSSE is fabricated by integration of appropriately engineered end-of-life photovoltaic recycled high purity silicon derived nano size inorganic lithium orthosilicate and poly (vinylidene fluoride-co-hexafluoropropylene) polymer matrix through entrapping ionic liquid electrolytes. The obtained lithium orthosilicate-polymer blended HSSE is self-standing, soft, and flexible which delivers a low activation energy, leading a high ionic conductivity of 4.4 × 10−4 Scm−1. A critical lithium plating/stripping behaviour shows improved interfacial stability (both inorganic/organic interface and interfaces of HSSE/electrodes) under different current densities (0.05 mA cm−2 to 0.13 mA cm−2) when a drop of ionic liquid electrolyte is added, permitting cycling of an all-solid-state full cell (Li/HSSE/LiFePO4) at room temperature with high capacity (132 mAh g−1), stable cycle life and high Coulombic efficiency (initial ∼98 %; subsequent >99.5 %). Shaping the interfaces between inorganic/organic phases in the HSSE as well as the interfaces between electrodes/HSSE appears to be a workable path towards constructing very effective HSSE for the advancement of all-solid-state lithium metal batteries.

We have revisited recent results on the ideal ignition of H11B fuel, in the light of the latest available reactivity, an alternative self-consistent calculation of the electron temperature, an increased extent of the suprathermal effects and the impact of plasma density. At high density, we find that the ideal ignition temperature is appreciably relaxed (e.g., Ti≃150 keV for ni∼1026 cm−3 and an optimal 11B/H concentration ε=0.15) and burn becomes substantial. We have then investigated central hot-spot ignition in both isobaric and isochoric inertial confinement configurations. Although implosion-driven ignition appears to be unfeasible, the isochoric self-heating conditions foster favourable preliminary conclusions on the utilization of proton fast ignition. In the isochoric case, we find a broad minimum in the ignition energy at ρR≃8.5 g/cm2 and 220≲Ti≲340 keV (80 ≲Te≲95 keV), for ε=0.15.

We propose for the first time, a new fuel-material for laser-driven Proton Boron (P-B) fusion nuclear reactions. We propose, Hydrogen rich, Borane (B m H n ) materials as fusion fuel as compared to Boron Nitride (BN) presently used. We estimate more than 10-fold increase in the yield of nuclear fusion reactions, and Alpha-prticle flux, when, for example Ammonia Borane (BNH6) laser-target material will be used compared to the state of the art normalized flux ∼108 Alphas/sr/J from BN targets. BNH6 contains ∼1000× higher concentration of Hydrogen compared to BN. We report the manufactureof thefirst solid-pellets Ammonia Borane laser-targets. To obtain high Flux Alpha sources from repetitive lasers we propose new BNH6 target geometries: liquid (molten) droplets/jets; or translated tape- or disc-targets coated with BNH6 powder. Targets would be irradiated in low pressure, ambient buffer gas. To enhance the fusion/Alpha yield of ultra-high intensity PetaWatt laser-target interaction we propose nano- and micro-structured Borane targets.As applications, we propose to use the Alpha-driven nuclear reactions inside the laser-driven Borane targets for new schemes to produce short-lived medical radioisotopes.Such laser-driven radioisotope beamlines would be installed directly inhospitals. Borane materials, like Diborane (6), B2H6, are also proposed as nuclear-fuels for laser-driven Proton-Boron fusion energy generation. The high dilution of Boron in Hydrgen B/H = 33% would need to be further enahnced to B/H < 15% to cut radiation losses from the hot and dense fusion pellet.