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Since 1968, the Australian Dung Beetle Project has carried out field releases of 43 deliberately introduced dung beetle species for the biological control of livestock dung and dung‐breeding pests. Of these, 23 species are known to have become established. For most of these species, sufficient time has elapsed for population expansion to fill the extent of their potential geographic range through both natural and human‐assisted dispersal. Consequently, over the last 20 years, extensive efforts have been made to quantify the current distribution of these introduced dung beetles, as well as the seasonal and spatial variation in their activity levels. Much of these data and their associated metadata have remained unpublished, and they have not previously been synthesized into a cohesive dataset. Here, we collate and report data from the three largest dung beetle monitoring projects from 2001 to 2022. Together, these projects encompass data collected from across Australia, and include records for all 23 species of established dung beetles introduced for biocontrol purposes. In total, these data include 22,718 presence records and 213,538 absence records collected during 10,272 sampling events at 546 locations. Most presence records (97%) include abundance data. In total, 1,752,807 dung beetles were identified as part of these data. The distributional occurrence and abundance data can be used to explore questions such as factors influencing dung beetle species distributions, dung beetle biocontrol, and insect‐mediated ecosystem services. These data are provided under a CC‐BY‐NC 4.0 license and users are encouraged to cite this data paper when using the data. [ABSTRACT FROM AUTHOR]

A new species of carnivorous sponge, Lycopodina coralseaensis sp. nov., family Cladorhizidae, is described from the Louisiade Plateau, Coral Sea, off the east coast of Australia at bathyal depths of ~2000 m, collected during the RV Investigator 2019 voyage. The new species differs from other Lycopodina with stipitate morphology by lacking forceps microscleres, similar to L. nikitawimandi Ekins et al., 2020a but differing in having only a very small single size class of palmate anisochelae, and most importantly by having a third category of mycalostyle echinating the stem and basal holdfast. This new species is the sixth Lycopodina recently described as new from the Australian Exclusive Economic Zone (L. nikitawimandi, L. helios, L. cassida, L. brochidodroma and L. hystrix), bringing the total number of carnivorous sponges known so far from Australia to 26. [ABSTRACT FROM AUTHOR]

Objectives interRAI launched this study to introduce a set of standardized self-report measures through which residents of long-term care facilities (LTCFs) could describe their quality of life and services. This article reports on the international development effort, describing measures relative to privacy, food, security, comfort, autonomy, respect, staff responsiveness, relationships with staff, friendships, and activities. First, we evaluated these items individually and then combined them in summary scales. Second, we examined how the summary scales related to whether the residents did or did not say that the LTCFs in which they lived felt like home. Design Cross-sectional self-report surveys by residents of LTCFs regarding their quality of life and services. Setting/Participants Resident self-report data came from 16,017 individuals who resided in 355 LTCFs. Of this total, 7113 were from the Flanders region of Belgium, 5143 residents were from Canada, and 3358 residents were from the eastern and mid-western United States. Smaller data sets were collected from facilities in Australia (20), the Czech Republic (72), Estonia (103), Poland (118), and South Africa (87). Measurements The interRAI Self-Report Quality of Life Survey for LTCFs was used to assess residents' quality of life and services. It includes 49 items. Each area of inquiry (eg, autonomy) is represented by multiple items; the item sets have been designed to elicit resident responses that could range from highly positive to highly negative. Each item has a 5-item response set that ranges from “never” to “always.” Results Typically, we scored individual items scored based on the 2 most positive categories: “sometimes” and “always.” When these 2 categories were aggregated, among the more positive items were: being alone when wished (83%); decide what clothes to wear (85%); get needed services (87%); and treated with dignity by staff (88%). Areas with a less positive response included: staff knows resident's life story (30%); resident has enjoyable things to do on weekends (32%); resident has people to do things with (33%); and resident has friendly conversation with staff (45%). We identified 5 reliable scales; these scales were positively associated with the resident statement that the LTCF felt like home. Finally, international score standards were established for the items and scales. Conclusions This study establishes a set of standardized, self-report items and scales with which to assess the quality of life and services for residents in LTCFs. The study also demonstrates that these scales are significantly related to resident perception of the home-like quality of the facilities. [ABSTRACT FROM AUTHOR]

There is increasing concern that widespread forest decline could occur in regions of the world where droughts are predicted to increase in frequency and severity as a result of climate change. The average annual leaf area index ( LAI) is an indicator of canopy cover and the difference between the annual maximum and minimum LAI is an indicator of annual leaf turnover. In this study, we analyzed satellite-derived estimates of monthly LAI across forested coastal catchments of southwest Western Australia over a 12 year period (2000-2011) that included the driest year on record for the last 60 years. We observed that over the 12 year study period, the spatial pattern of average annual satellite-derived LAI values was linearly related to mean annual rainfall. However, interannual changes to LAI in response to changes in annual rainfall were far less than expected from the long-term LAI-rainfall trend. This buffered response was investigated using a physiological growth model and attributed to availability of deep soil moisture and/or groundwater storage. The maintenance of high LAIs may be linked to a long-term decline in areal average underground water storage and diminished summer flows, with an emerging trend toward more ephemeral flow regimes. [ABSTRACT FROM AUTHOR]

Background Inherited retinal disease represents a significant cause of blindness and visual morbidity worldwide. With the development of emerging molecular technologies, accessible and well-governed repositories of data characterising inherited retinal disease patients is becoming increasingly important. This manuscript introduces such a repository. Design Participants were recruited from the Retina Australia membership, through the Royal Australian and New Zealand College of Ophthalmologists, and by recruitment of suitable patients attending the Sir Charles Gairdner Hospital visual electrophysiology clinic. Participants Four thousand one hundred ninety-three participants were recruited. All participants were members of families in which the proband was diagnosed with an inherited retinal disease (excluding age-related macular degeneration). Methods Clinical and family information was collected by interview with the participant and by examination of medical records. In 2001, we began collecting DNA from Western Australian participants. In 2009 this activity was extended Australia-wide. Genetic analysis results were stored in the register as they were obtained. Main Outcome Measures The main outcome measurement was the number of DNA samples (with associated phenotypic information) collected from Australian inherited retinal disease-affected families. Results DNA was obtained from 2873 participants. Retinitis pigmentosa, Stargardt disease and Usher syndrome participants comprised 61.0%, 9.9% and 6.4% of the register, respectively. Conclusions This resource is a valuable tool for investigating the aetiology of inherited retinal diseases. As new molecular technologies are translated into clinical applications, this well-governed repository of clinical and genetic information will become increasingly relevant for tasks such as identifying candidates for gene-specific clinical trials. [ABSTRACT FROM AUTHOR]

This paper considers the potential role literary tourism has in the economic future of rural regions. In many rural areas, literary tourism is critically important to the economic prosperity and social identities of those places. We argue that as many rural communities look to diversify their economic activities, previously unrecognised literary assets could be useful in establishing new economic activity. However, through a review of literary tourism in rural Western Australia, we find that to develop a successful literary tourism industry more than just an established literary canon is required. The nature of literary attractions and their geographical location are critical to the successful development of literary tourism. [ABSTRACT FROM AUTHOR]

Failing grain crops are sometimes sacrificed for grazing by mixed farmers, a decision involving a complex range of factors. This simulation study used two APSIM (Agricultural Production Systems Simulator)-based approaches to investigate the circumstances under which more revenue might be obtained by sacrificing a wheat crop for grazing rather than harvesting it for grain in Western Australia's grainbelt. First, we developed a simple partial budget calculation to estimate and compare revenue from grain or grazing alternatives using data for grain yield and standing biomass at flowering. This was simulated for a factorial of soil types and locations varying in mean annual rainfall. We then simulated wheat quality and livestock production on spring wheat grazed at different stages of crop development and at a range of stocking rates. Dynamic simulations of grazing showed that livestock production increased as grazing was delayed; stocking rate had little impact at this time. Grazing earlier necessitated lighter stocking rates but surprisingly had little benefit for animal performance. Partial budgets showed that under average commodity prices, grazing a wheat crop could be more profitable 40-75% of the time on poorer soil types in lower rainfall environments. In these situations, by tactically grazing when grain yield is below a critical level economic returns could be increased by more than A$50/ha in 30-40% of years and over the long term average revenues could be increased by A$30/ha.year. This critical grain yield ranged from 1.3 to 1.7 t/ha on shallow gravel soil and 1.9 to 2.2 t/ha on a deep sand. In higher rainfall environments and on better soil types grazing was rarely a better option unless livestock prices were high relative to grain. This approach, combining crop simulation with partial budgets, was useful for developing simple management rules for a complex system. Overall, the findings of this study suggest that making tactical use of a wheat crop for forage in situations with low grain yield prospects is a major opportunity to increase profitability and help respond to climate variability in mixed farms in many areas of the Western Australian wheatbelt. [ABSTRACT FROM AUTHOR]

Abstract: Spontaneous combustion and low-temperature oxidation of waste coal in open-cut coal mines represents a potentially large source of greenhouse gas emissions. In this paper, emission fluxes of CO2 and CH4 from spoil piles and waste coal dumps measured at 11 mines in the Hunter Valley and Bowen Basin in Australia are presented. The data displayed considerable scatter, which is consistent with the inhomogeneous nature of spoil pile material and permeability of surfaces. Despite the scatter, emissions were able to be classified into three broad categories according to the intensity of the spontaneous combustion present in the material. Average emissions ranged from about 12 kg CO2-e yr−1 m−2 to 8200 kg CO2-e yr−1 m−2, depending on the intensity of the spontaneous combustion. There was also, within the scatter of the data, an approximately linear trend of increasing emission flux with increasing surface temperature. A key finding of the research is that the emission rates of greenhouse gases from spoil piles where there is no spontaneous combustion, but only low-temperature oxidation of coal and coal waste, are similar to the emission rates due to biological activity from vegetated surfaces. However, further research is required to quantify the degree to which spoil piles that have no spontaneous combustion contribute to the anthropogenic atmospheric CO2 burden. [Copyright &y& Elsevier]

Parasitism levels and seasonal phenology of the introduced species leafblister sawfly, Phylacteophaga froggatti, in south-western Australian blue gum ( Eucalyptus globulus globulus) plantations were investigated. Leafblister sawfly was found at all times of the year, with overlapping generations. However, it became locally rare or extinct on a frequent but temporary basis at all sampled plantations for unknown reasons. Cirrospilus margiscutellum and Bracon confusus were the only parasitic wasps reared from leafblister sawfly larvae and pupae, with both species occurring at such low levels that they did not have a significant impact on leafblister sawfly populations. The parasitic wasp B. phylacteophagus, successfully used in the biological control of leafblister sawfly elsewhere, was not found. Its absence indicates that the elevated pest status of leafblister sawfly in south-western Australia may be a result of inadequate biological control, and a ‘classical’ biological control program using B. phylacteophagus is recommended for the pest. [ABSTRACT FROM AUTHOR]

Data concerning farm-related injuries were collected from the Emergency Department at Tamworth Base Hospital over a 12-month period from 1 September 1997. The aim of the study was to collect information at a local level to establish baselines with a view to developing prevention strategies. All people who presented with a farm injury or illness to the emergency department participated in the study. During this period there were 384 injuries, of which nearly three-quarters were males (72.2%). Four injuries were fatal. The average rate of injury per 100 farms per annum in the service area of the Hospital was 30 per 100 farms (range 9–80 per 100 farms, per annum). Half (54.1%) of the people injured were employed at the time of the injury. Horses (21.1%) and motorcycles (15.8%) were the two most common injury agents. The information gained can be used to direct injury prevention at a local level and may be also used at the national level as a guide when grouped with other similar studies of different commodity groups. [ABSTRACT FROM AUTHOR]

African black beetle is a major agricultural and forestry pest in south-western Australia. In this study, we examine the effectiveness of various surface-applied chlorpyrifos treatments for the control of beetle damage in commercial forestry crops. Of those treatments tested, none had a strong, consistent effect on the abundance of the subterranean adult beetles through to 9 weeks after application, nor did they consistently reduce beetle damage or improve crop productivity. When incorporated into soil at rates presently employed successfully in agriculture, chlorpyrifos caused no mortality of adult African black beetles that were added to the soil post-treatment. Chlorpyrifos decays at an exponential rate in soil with 75% of the active ingredient remaining at 1 h post-treatment, 40% at 50 days and 10% remaining after 110 up to 170 days. It appears that the capacity of chlorpyrifos to cause mortality to African black beetle is through vigorous soil incorporation bringing the chemical into direct contact with the insect. Chlorpyrifos applications are not recommended for the control of African black beetle in tree crops because the restrictions on timing and use of soil incorporation techniques will generally mean that the insecticide will not be brought into direct contact with a sufficient proportion of the beetle population. [Copyright &y& Elsevier]

Discusses the implications for base-metal mineralization process of sequence stratigraphy of the Bowthorn block in the northern Mount Isa basin, Australia. Occurrence of dolomitization and paleothermal anomalies in the area; Drill-hole, seismic and outcrop data; Reflectivity profiles.

Many Composite weeds, vegetables and ornamental plants have been introduced to Australia since European settlement began in 1788. The present distribution of 4 weeds known to cause contact dermatitis is recorded on maps, and a short history of each is given. The relatively slow spread of these weeds is contrasted with the rapid spread of Parthenium hysterophorus which caused epidemic contact dermatitis in India and which has recently caused dermatitis in Northern Australia. Australian native Composite plants may play some part in the perpetuation of contact dermatitis initiated by weeds introduced into Australia. [ABSTRACT FROM AUTHOR]

Presents the 1993 Sir Robert Garran oration delivered by the former Australian Minister for Industry, Technology and Commerce. Emphasis in housekeeping and operations in public administration changes of the 1980s; Challenges faced by governments and public managers; Capacity of the Australian Public Service to deal with macro issues of governance.

The patterns of photo-contact dermatitis from Jadit (4-chloro-2-hydroxybenzoic acid-N-n butylamide) in Europe and South Australia are described and discussed. The clinical details of 11 cases reported by European authors are compared with those of 13 Australian cases, 11 of which are reported for the first time. It is noted that the average length of application before sensitization in Europe was greater than in Australia and is suggested that this difference is the result of climatic conditions. It is suggested that Jadit photo-dermatitis is a "conventional contact dermatitis" from photo degradation products; that the drug can be retained in the skin and thereby cause persistent light reactions; and that it can be absorbed following local application and be distributed systemically. Cross-reactions between Jadit and several sulphanilamide derivatives which had been previously described in Europe were also found in 6 Australian cases. [ABSTRACT FROM AUTHOR]

The article presents the results of research on the impact of mandatory fortification of wheat flour with folic acid on the blood folate levels of an Australian population. Serum and red blood cells (RBC) folate levels of 20592 blood samples from the patients of a large public hospital was utilized in the research. It is noted that between April 2009 ad April 2010m, there was a reduction of prevalence of low serum folate level in the samples as well as a reduction of the prevalence of RBC folate levels among females of childbearing age.

Since 1964 patch testing has been carried out in a private practice in South Australia. From 1964 to 1972 aimed patch tests only were applied, hut were replaced during 1974 by the International Contact Dermatitis Research Group (ICDRG) standard tests, plus chlorocresol and chloroxylenol and aimed tests when necessary. The clinically relevant patch tests recorded in three papers, each but some sensitizers not previously recorded were found, especially photosensitizers and plants. Thus, the most important agent acting in individual patients were recorded.

The role of the standard is critical to the derivation of reliable U–Pb zircon ages by micro-beam analysis. For maximum reliability, it is critically important that the utilised standard be homogeneous at all scales of analysis. It is equally important that the standard has been precisely and accurately dated by an independent technique. This study reports the emergence of a new zircon standard that meets those criteria, as demonstrated by Sensitive High Resolution Ion MicroProbe (SHRIMP), isotope dilution thermal ionisation mass-spectrometry (IDTIMS) and excimer laser ablation–inductively coupled plasma–mass-spectrometry (ELA–ICP–MS) documentation. The TEMORA 1 zircon standard derives from the Middledale Gabbroic Diorite, a high-level mafic stock within the Palaeozoic Lachlan Orogen of eastern Australia. Its 206Pb/238U IDTIMS age has been determined to be 416.75±0.24 Ma (95% confidence limits), based on measurement errors alone. Spike-calibration uncertainty limits the accuracy to 416.8±1.1 Ma for U–Pb intercomparisons between different laboratories that do not use a common spike. [Copyright &y& Elsevier]

It is unknown whether the currently known risk factors of multiple sclerosis reflect the etiology of progressive-onset multiple sclerosis (POMS) as observational studies rarely included analysis by type of onset. We designed a case-control study to examine associations between environmental factors and POMS and compared effect sizes to relapse-onset MS (ROMS), which will offer insights into the etiology of POMS and potentially contribute to prevention and intervention practice. This study utilizes data from the Primary Progressive Multiple Sclerosis (PPMS) Study and the Australian Multi-center Study of Environment and Immune Function (the AusImmune Study). This report outlines the conduct of the PPMS Study, whether the POMS sample is representative, and the planned analysis methods. The study includes 155 POMS, 204 ROMS, and 558 controls. The distributions of the POMS were largely similar to Australian POMS patients in the MSBase Study, with 54.8% female, 85.8% POMS born before 1970, mean age of onset of 41.44 ± 8.38 years old, and 67.1% living between 28.9 and 39.4° S. The POMS were representative of the Australian POMS population. There are some differences between POMS and ROMS/controls (mean age at interview: POMS 55 years vs. controls 40 years; sex: POMS 53% female vs. controls 78% female; location of residence: 14.3% of POMS at a latitude ≤ 28.9°S vs. 32.8% in controls), which will be taken into account in the analysis. We discuss the methodological issues considered in the study design, including prevalence-incidence bias, cohort effects, interview bias and recall bias, and present strategies to account for it. Associations between exposures of interest and POMS/ROMS will be presented in subsequent publications., (© 2023. The Author(s).)

Background: To establish the proportion of patients with retinitis pigmentosa (RP) meeting the Australian fitness to drive (FTD) visual standards., Methodology: A prospective consecutive case series of patients with a clinical or genetic diagnosis of RP. Data on age at symptom onset, current driving status, inheritance pattern, better eye visual acuity (BEVA), binocular Esterman visual field (BEVF) parameters, genotype and ability to meet the driving standards based on BEVA and BEVF were collected. Outcome measures included the proportion of RP patients overall meeting the standards and clinical predictors for passing. A sub-analysis was performed on those RP patients who reported to drive. Change in BEVA and BEVF parameters across age in specific genotype groups was assessed., Results: Overall, 228 patients with RP had a BEVF assessment. Only 39% (89/228) met the driving standards. Younger age at the time of testing was the only significant predictor ( p  < 0.01) for passing. Of the 55% of RP patients who reported to drive, 52% (65/125) met the standards, decreasing to 14% in the 56- to 65-year-old age group. RP patients harbouring mutations in HK1 or RHO genes may have slower rates of decline in their VF parameters., Conclusion: Nearly 40% of RP patients met the driving standards. However, almost 50% of RP drivers were unaware of their failure to meet the current standards. BEVF testing is essential in the assessment of RP patients who are still driving. Phenotype and genotype predictors for passing the standards warrant further investigation. Abbreviation: FTD, fitness to drive; IRD, inherited retinal disease; RP, retinitis pigmentosa; RHO, rhodopsin; HK1, hexokinase 1; PRPF31 pre-mRNA processing factor 31; RPGR, retinitis pigmentosa GTPase regulator; VF, visual field; BEVA, better eye visual acuity; BEVF, binocular Esterman visual field.

Four new species of encrusting Hamacantha (Vomerula) are described from bathyal depths of seamounts off Queensland and Tasmania in southeast Australia (H. (V.) novacula sp. nov., H. (V.) melliflura sp. nov., H. (V.) ridleyi sp. nov., H. (V.) levii sp. nov.). A previously described species known only from New Caledonia (H. (V.) acerata Lévi, 1993) has also been redescribed. A tabular comparison of all known species of H. (Vomerula) is provided, bringing the number of known species in the subgenus to 26. This is also the first record of the genus Hamacantha from the Australian Exclusive Economic Zone.

This research presents three new species of carnivorous sponges from the family Cladorhizidae from the Great Barrier Reef, in Queensland, Australia: Abyssocladia falkor sp. nov., Abyssocladia jeanvaceleti sp. nov. and Axoniderma wanda sp. nov. They were collected by ROV during the expedition FK200802-Seamounts, Canyons & Reefs of the Coral Sea Cruise on the RV Falkor from the Schmidt Ocean Institute. In addition, the ROV collection of two complete specimens enabled the redescription of two other Australian species of carnivorous sponge (Chondrocladia (Chondrocladia) zygainadentonis Ekins et al., 2020a and Asbestopluma (Asbestopluma) maxisigma Ekins et al., 2020a), previously known from the East coast of Australia based on incomplete specimens.

Voltage-gated sodium (Na V ) channels are critical regulators of neuronal excitability and are targeted by many toxins that directly interact with the pore-forming α subunit, typically via extracellular loops of the voltage-sensing domains, or residues forming part of the pore domain. Excelsatoxin A (ExTxA), a pain-causing knottin peptide from the Australian stinging tree Dendrocnide excelsa, is the first reported plant-derived Na V channel modulating peptide toxin. Here we show that TMEM233, a member of the dispanin family of transmembrane proteins expressed in sensory neurons, is essential for pharmacological activity of ExTxA at Na V channels, and that co-expression of TMEM233 modulates the gating properties of Na V 1.7. These findings identify TMEM233 as a previously unknown Na V 1.7-interacting protein, position TMEM233 and the dispanins as accessory proteins that are indispensable for toxin-mediated effects on Na V channel gating, and provide important insights into the function of Na V channels in sensory neurons., (© 2023. The Author(s).)

The ATP-binding cassette subfamily A member 4 gene (ABCA4)-associated retinopathy, Stargardt disease, is the most common monogenic inherited retinal disease. Given the pathogenicity of numerous ABCA4 variants is yet to be examined and a significant proportion (more than 15%) of ABCA4 variants are categorized as splice variants in silico, we therefore established a fibroblast-based splice assay to analyze ABCA4 variants in an Australian Stargardt disease cohort and characterize the pathogenic mechanisms of ABCA4 variants. A cohort of 67 patients clinically diagnosed with Stargardt disease was recruited. Genomic DNA was analysed using a commercial panel for ABCA4 variant detection and the consequences of ABCA4 variants were predicted in silico. Dermal fibroblasts were propagated from skin biopsies, total RNA was extracted and the ABCA4 transcript was amplified by RT-PCR. Our analysis identified a total of 67 unique alleles carrying 74 unique variants. The most prevalent splice-affecting complex allele c.[5461-10T>C; 5603A>T] was carried by 10% of patients in a compound heterozygous state. ABCA4 transcripts from exon 13 to exon 50 were readily detected in fibroblasts. In this region, aberrant splicing was evident in 10 out of 57 variant transcripts (18%), carried by 19 patients (28%). Patient-derived fibroblasts provide a feasible platform for identification of ABCA4 splice variants located within exons 13-50. Experimental evidence of aberrant splicing contributes to the pathogenic classification for ABCA4 variants. Moreover, identification of variants that affect splicing processes provides opportunities for intervention, in particular antisense oligonucleotide-mediated splice correction., (Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Seven new polyaromatic bis-spiroketal-containing butenolides, the prunolides D-I ( 4 - 9 ) and cis -prunolide C ( 10 ), a new dibrominated β-carboline sulfamate named pityriacitrin C ( 11 ), alongside the known prunolides A-C ( 1 - 3 ) were isolated from the Australian colonial ascidian Synoicum prunum . The prunolides D-G ( 4 - 7 ) represent the first asymmetrically brominated prunolides, while cis -prunolide C ( 10 ) is the first reported with a cis -configuration about the prunolide's bis-spiroketal core. The prunolides displayed binding activities with the Parkinson's disease-implicated amyloid protein α-synuclein in a mass spectrometry binding assay, while the prunolides ( 1 - 5 and 10 ) were found to significantly inhibit the aggregation (>89.0%) of α-synuclein in a ThT amyloid dye assay. The prunolides A-C ( 1 - 3 ) were also tested for inhibition of pSyn aggregate formation in a primary embryonic mouse midbrain dopamine neuron model with prunolide B ( 2 ) displaying statistically significant inhibitory activity at 0.5 μM. The antiplasmodial and antibacterial activities of the isolates were also examined with prunolide C ( 3 ) displaying only weak activity against the 3D7 parasite strain of Plasmodium falciparum . Our findings reported herein suggest that the prunolides could provide a novel scaffold for the exploration of future therapeutics aimed at inhibiting amyloid protein aggregation and the treatment of numerous neurodegenerative diseases.

Background: Heterozygous c.440 G > T mutation in the S-antigen visual arrestin (SAG) gene has been described as a cause of autosomal dominant retinitis pigmentosa (adRP) in a series of patients of Hispanic origin. This study presents the early and late clinical features and disease progression rates in an Australian family with SAG adRP., Materials and Methods: An observational case series of four family members with adRP. They were examined clinically, with multi-modal retinal imaging and electroretinography (ERG) to ascertain phenotype. Disease progression rate was measured using optical coherence tomography (OCT) and fundus autofluorescence (FAF). A retinal dystrophy panel was used for the proband and cascade testing with targeted Sanger sequencing was conducted in other available family members., Results: The proband presented at 36 years of age with profoundly reduced full-field ERG responses despite a sector RP phenotype. This progressed to a classic RP pattern over several decades leaving a small residual island of central visual field. The horizontal span of the residual outer nuclear layer and the area of hyperautofluorescent ring contracted at a rate of 8-11% and 9-14% per year, respectively. DNA sequencing confirmed the segregation of SAG c.440 G > T mutation with disease., Conclusion: SAG adRP presents with a reduced full-field ERG response consistent with a rod-cone dystrophy in mid-life despite a sector RP phenotype. Centripetal progression of the disease into the macula can be tracked by OCT and FAF imaging.

This research presents three new species of carnivorous sponges from the family Cladorhizidae from the Great Australian Bight, South Australia. This research also shows a clear separation within the species currently known as Cladorhiza into those with an arbuscular or tree-like morphology from the Atlantic, to those of differing morphologies and propose three new genera, i.e. Bathytentacular gen. nov., Abyssosdiskos gen. nov. and Nullarbora gen. nov. and the resurrection of an old genus Axoniderma. nov. The three new species described in this paper are Nullarbora heptaxia sp. nov., Abyssocladia oxyasters sp. nov. and Lycopodina hystrix sp. nov. A new species in the family Guitarridae, Guitarra davidconryi sp. nov., a family closely related to the carnivorous sponges is also described in this paper. These new species are the first recorded carnivorous species from South Australia and increase the number of species recorded from around Australia to 25.

This research presents 17 new species of carnivorous sponges from the family Cladorhizidae sampled at bathyal and abyssal depths off the east coast of Australia during the RV Investigator 2017 Abyss Expedition. The species are comprised of six genera: Abyssocladia escheri sp. nov., A. annae sp. nov., A. gliscofila sp. nov., Asbestopluma (Asbestopluma) maxisigma sp. nov., Cladorhiza australis nov. sp., C. poritea sp. nov., C. investigator sp. nov., C. moniqueae sp. nov., C. pentaeiros sp. nov., Chondrocladia (Chondrocladia) freycinetensis sp. nov., Ch. (Ch.) callistemonex sp. nov., Ch. (Ch.) zygainadentonis sp. nov., Lycopodina nikitawimandi sp. nov., L. helios sp. nov., L. cassida sp. nov., L. brochidodroma sp. nov. and Euchelipluma claudochela sp. nov. Only three carnivorous species, A. desmophora, As. (As.) desmophora and C. (Meliiderma) tasmaniensis, have been recorded from Australia previously. This expedition also recovered two described species commonly recorded from the Southern Hemisphere Ch. (Ch.) clavata and L. calyx. This paper also redescribes two species from the South Pacific i.e. C. mirabilis and C. similis.

Background and Purpose- Reversal of dabigatran before intravenous thrombolysis in patients with acute ischemic stroke has been well described using alteplase but experience with intravenous tenecteplase is limited. Tenecteplase seems at least noninferior to alteplase in patients with intracranial large vessel occlusion. We report on the experience of dabigatran reversal before tenecteplase thrombolysis for acute ischemic stroke. Methods- We included consecutive patients with ischemic stroke receiving dabigatran prestroke treated with intravenous tenecteplase after receiving idarucizumab. Patients were from 2 centers in New Zealand and Australia. We reported the clinical, laboratory, and radiological characteristics and their functional outcome. Results- We identified 13 patients receiving intravenous tenecteplase after dabigatran reversal. Nine (69%) were male, median age was 79 (interquartile range, 69-85) and median baseline National Institutes of Health Stroke Scale score was 6 (interquartile range, 4-21). Atrial fibrillation was the indication for dabigatran therapy in all patients. All patients had a prolonged thrombin clotting time (median, 80 seconds [interquartile range, 57-113]). Seven patients with large vessel occlusion were referred for endovascular thrombectomy, 2 of these patients (29%) had early recanalization with tenecteplase abrogating thrombectomy. No patients had parenchymal hemorrhage or symptomatic hemorrhagic transformation. Favorable functional outcome (modified Rankin Scale score, 0-2) occurred in 8 (62%) patients. Two deaths occurred from large territory infarction. Conclusions- Our experience suggests intravenous thrombolysis with tenecteplase following dabigatran reversal using idarucizumab may be safe in selected patients with acute ischemic stroke. Further studies are required to more precisely estimate the efficacy and risk of clinically significant hemorrhage.

There is an urgent need to discover and develop new anthelmintics for the treatment of parasitic nematodes of veterinary importance to circumvent challenges linked to drug resistant parasites. Being one of the most diverse natural ecosystems, the marine environment represents a rich resource of novel chemical entities. This study investigated 2000 extracts from marine invertebrates, collected from Australian waters, for anthelmintic activity. Using a well-established in vitro bioassay, these extracts were screened for nematocidal activity against Haemonchus contortus -a socioeconomically important parasitic nematode of livestock animals. Extracts (designated Mu -1, Ha -1 and Ha -2) from two marine sponges ( Monanchora unguiculata and Haliclona sp.) each significantly affected larvae of H. contortus . Individual extracts displayed a dose-dependent inhibition of both the motility of exsheathed third-stage larvae (xL3s) and the development of xL3s to fourth-stage larvae (L4s). Active fractions in each of the three extracts were identified using bioassay-guided fractionation. From the active fractions from Monanchora unguiculata , a known pentacyclic guanidine alkaloid, fromiamycalin ( 1 ), was purified. This alkaloid was shown to be a moderately potent inhibitor of L4 development (half-maximum inhibitory concentration (IC 50 ) = 26.6 ± 0.74 µM) and L4 motility (IC 50 = 39.4 ± 4.83 µM), although it had a relatively low potency at inhibiting of xL3 motility (IC 50 ≥ 100 µM). Investigation of the active fractions from the two Haliclona collections led to identification of a mixture of amino alcohol lipids, and, subsequently, a known natural product halaminol A ( 5 ). Anthelmintic profiling showed that 5 had limited potency at inhibiting larval development and motility. These data indicate that fromiamycalin, other related pentacyclic guanidine alkaloids and/or halaminols could have potential as anthelmintics following future medicinal chemistry efforts., Competing Interests: The authors declare no conflicts of interest. Funding bodies played no role in the design of the study, collection, analysis or interpretation of data, or in the writing of the manuscript or in the decision to publish the results.

Routine monitoring of toxic cyanobacteria depends on up-to-date epidemiological information about their distribution. In Australia, anatoxin producing cyanobacteria are not regularly tested for and thought to be rare if not absent from the continent. Our study investigated the presence of anatoxin-a (ATX-a) producing cyanobacteria in surface water samples (n = 226 from 67 sampling locations) collected from 2010 to 2017 across the state of Victoria, Australia. We (1) detected the presence and distribution of anaC (anatoxin-a synthetase C) gene sequences previously associated with various cyanobacteria, including Cuspidothrix issatschenkoi, Aphanizomenon sp., D. circinale, Anabaena sp., and Oscillatoria sp., from 31 sampling locations, and (2) determined the concentration of ATX-a in samples tested using ELISA, in two instances detected at >4 µg · L -1 . These data present the first confirmation of ATX-a producers in Australia. Our study indicates that ATX-a should be included in regular testing of cyanobacterial blooms in Australia and highlights the importance of regular investigation of the distributions of toxic cyanobacteria worldwide, particularly amid the known expanding distribution of many cyanobacterial taxa in a period of increased eutrophication and rising surface water temperatures.

Inherited retinal diseases (IRDs) are genetically and phenotypically diverse, and they cause significant morbidity worldwide. Importantly, IRDs may be amenable to precision medicine strategies, and thus the molecular characterisation of causative variants is becoming increasingly important with the promise of personalised therapies on the horizon. ABCA4, involved in the translocation of visual cycle derivatives, is a well-established, frequent cause of IRDs worldwide, with pathogenic variants implicated in phenotypically diverse diseases. Identification of causative ABCA4 variants in some individuals, however, has been enigmatic, and resolution of this issue is currently a hotbed of research. Recent evidence has indicated that hypomorphic alleles, which cause disease under certain conditions, may account for some of the missing causal variants. It has been postulated that the ABCA4 c.5603A>T (p.Asn1868Ile) variant, previously considered benign, be reclassified as hypomorphic when in cis configuration with c.2588G>C (p.Gly863Ala/Gly863del), a variant previously considered to be pathogenic in its own right. We are exploring this relationship within an Australian cohort to test this theory.

Background: Leber congenital amaurosis (LCA) is a severe visual impairment responsible for infantile blindness, representing ~5% of all inherited retinal dystrophies. LCA encompasses a group of heterogeneous disorders, with 24 genes currently implicated in pathogenesis. Such clinical and genetic heterogeneity poses great challenges for treatment, with personalized therapies anticipated to be the best treatment candidates. Unraveling the individual genetic etiology of disease is a prerequisite for personalized therapies, and could identify potential treatment candidates, inform patient management, and discriminate syndromic forms of disease., Methods: We have genetically analyzed 45 affected and 82 unaffected individuals from 34 unrelated LCA pedigrees using predominantly next-generation sequencing and Array CGH technology., Results: We present the molecular findings for an Australian LCA cohort, sourced from the Australian Inherited Retinal Disease Registry & DNA Bank. CEP290 and GUCY2D mutations, each represent 19% of unrelated LCA cases, followed by NMNAT1 (12%). Genetic subtypes were consistent with other reports, and were resolved in 90% of this cohort., Conclusion: The high resolution rate achieved, equivalent to recent findings using whole exome/genome sequencing, reflects the progression from hypothesis (LCA Panel) to non-hypothesis (RD Panel) testing and, coupled with Array CGH analysis, is a highly effective first-tier test for LCA., (© 2017 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.)

Chemical investigations of an Australian sponge, Clathria hirsuta, from the Great Barrier Reef, have resulted in the isolation of two known anthraquinones, rhodocomatulin 5,7-dimethyl ether (1) and rhodocomatulin 7-methyl ether (2). Additionally, four new anthraquinone metabolites, 6-methoxyrhodocomatulin 7-methyl ether, 3-bromo-6-methoxy-12-desethylrhodocomatulin 7-methyl ether, 3-bromo-6-methoxyrhodocomatulin 7-methyl ether, and 3-bromorhodocomatulin 7-methyl ether (3-6), were also isolated and characterized. This is the first report of the rhodocomatulin-type anthraquinones from a marine sponge, as 1 and 2 were previously isolated from the marine crinoid genus Comatula. An additional chemical investigation of the marine crinoid Comatula rotalaria enabled the isolation of further quantities of 1 and 2, as well as two additional new crinoid metabolites, 12-desethylrhodocomatulin 5,7-dimethyl ether and 12-desethylrhodocomatulin 7-methyl ether (7 and 8). An NMR spectroscopic analysis of compounds 7 and 8 provided further insight into the rhodocomatulin planar structure and, together with the successful implementation of DFT-NMR calculations, confirmed that the rhodocomatulin metabolites existed as para rather than ortho quinones.

Background: Choroideremia is an X-linked inherited chorioretinal disease known to be caused by mutations in the CHM gene. In this study, Australian families clinically diagnosed with choroideremia were genetically analysed for mutations in the CHM gene., Design: The Australian Inherited Retinal Disease Register and DNA Bank (AIRDR) was investigated to identify a cohort of choroideremia-affected families for genetic analysis., Participants: Participants were sourced from the AIRDR. Thirty-two participants (15 affected, 10 carriers, 7 unaffected) sourced from 11 unrelated families having at least one member clinically diagnosed with choroideremia were included in the study., Methods: We performed sequence analysis of the CHM gene on the DNA of nine probands. We received the direct sequencing results of two probands by other means. Targeted analysis was subsequently performed for all 32 participants to confirm the direct sequencing results in the 11 probands and to establish the presence or absence of the implicated mutation in the remaining 21 affected, carrier or unaffected family members., Main Outcome Measures: Genetic characterisation of 11 choroideremia families in the Australian population., Results: A CHM mutation was detected in all 11 families. Each family had a different mutation. Mutations segregated within each family according to disease status. Five mutations were novel and six have been previously reported., Conclusions: Six previously reported and five novel CHM mutations were detected in 11 Australian families clinically diagnosed with choroideremia. We anticipate that this work will facilitate access for AIRDR participants and their progeny to CHM gene therapy trials., (© 2015 Royal Australian and New Zealand College of Ophthalmologists.)

Aim: To compare the burden and outcomes of cancer in New Zealand with those in Australia., Methods: For the years 1996-1997 and 2006-2007, the incidence and mortality of cancer in New Zealand and Australia was compared to determine if differences between the two countries had changed over the decade under study. Summarised cancer data from New Zealand and Australia, age standardised to the 2002 World Health Organisation's standard population, were used to make the comparisons., Results: For the 11 year timeframe of this study, total rates of cancer incidence reduced in New Zealand and increased in Australia. The incidence of cancer in New Zealand, relative to Australia, changed from an excess of +10.3 to a deficit of -27.5 per 100,000 people. When considering the excess in terms of gender, the annual excess of cancer registrations for New Zealand females fell from +19.9 to +0.9 per 100,000, and male cancer registration fell from an excess of +3.7 to a deficit of -58.0 per 100,000, due almost entirely to a surge in prostate cancer registration in Australia. Over the same 11-year timeframe, cancer-specific mortality rates decreased in both countries, but there was no change in the difference between New Zealand and Australian rates, which remained 10% higher in New Zealand. Similar to findings on 1996/7 data, the main cancer sites responsible for the overall excess mortality in 2006/7 were colorectal cancer in both sexes, and lung and breast cancer in females., Conclusion: The persisting different cancer mortality rates between the two countries is likely to have been partly due to lifestyle and ethnic differences in the populations, and partly due to New Zealanders presenting with more advanced cancers and having less easy access to some treatments. Until we know the relative contributions of these factors, it will be difficult for New Zealand to plan interventions in the future which have a good chance of lifting our cancer survival rates to those of our closest neighbour. The collection of clinical stage on all new cancer registrations would provide the base information required.

High throughput screening of a pre-fractionated natural product library identified 11 active fractions showing ApoE modulation activity. Mass-directed fractionation of one active crude extract from the Australian marine sponge Callyspongia sp. resulted in the isolation of 13 metabolites, including three new bromotyrosine derivatives, callyspongic acid (1), 3,5-dibromo-4-methoxyphenylpyruvic acid (2), N-acetyl-3-bromo-4-hydroxylphenylethamine (3), and ten known compounds (4-13). The structure elucidation of compounds 1-3 was based on their 1D and 2D NMR and MS spectroscopic data. 3,5-Dibromo-4-methoxyphenylpyruvic acid (2) showed weak activity in increasing the apolipoprotein E secretion from human CCF-STTG1 cells at the concentration of 40 μM., (Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Mass-guided fractionation of the MeOH extract from a specimen of the Australian marine sponge Hyrtios sp. resulted in the isolation of two new tryptophan alkaloids, 6-oxofascaplysin (2), and secofascaplysic acid (3), in addition to the known metabolites fascaplysin (1) and reticulatate (4). The structures of all molecules were determined following NMR and MS data analysis. Structural ambiguities in 2 were addressed through comparison of experimental and DFT-generated theoretical NMR spectral values. Compounds 1-4 were evaluated for their cytotoxicity against a prostate cancer cell line (LNCaP) and were shown to display IC50 values ranging from 0.54 to 44.9 μM., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Extensive surveys of the biodiversity on the seafloor of the inter-reef regions of the Great Barrier Reef, Australia, have resulted in the collection of large numbers of sponges, many of which are likely new to science. Identification of these sponges, however, was made difficult by the absence in some specimens of key diagnostic characters, such as megascleres. We used an integrated approach to the taxonomy of these sponges, incorporating morphological examination by SEM, analysis of DNA sequence data (using the COI barcoding fragment of mtDNA) and preliminary studies of the chemistry of the sponges, to describe the new species, which were found to contain no native spicules other than acanthose microrhabds. Here, we propose two new species of Theonella Gray, 1868: Theonella deliqua n. sp. (found in association with a single unidentified species of siliquariid mollusc) and Theonella maricae n. sp. from the Great Barrier Reef. Further, we propose the new combination of Theonella xantha (Sutcliffe, Hooper and Pitcher 2010) n. comb. for another microrhabd-only-bearing species. On the basis of our gene trees, we recognise Theonella (and Theonellidae Lendenfeld, 1903) within Astrophorida Sollas, 1887. We discuss the potential for chemotaxonomic and DNA-based insights into the origins and radiation of species of Theonella and explore the evolutionary significance of the reduced morphology of the three additional species recognised here.

Two consecutive prefractionated fractions of the Australian marine sponge extract, Pipestela candelabra, were identified to be selectively active on the human prostate cancer cells (PC3) compared to the human neonatal foreskin fibroblast non-cancer cells (NFF). Twelve secondary metabolites were isolated in which four compounds are new small peptides. Their structures were characterized by spectroscopic and chemical analysis. These compounds inhibited selectively the growth of prostate cancer cells with IC50 values in the picomolar to sub-micromolar range. Structure-activity relationship of these compounds is discussed.

Objective: Attention-deficit/hyperactivity disorder (ADHD) and autism spectrum disorder frequently co-occur. Several studies show increased risk of substance use disorders in ADHD, yet there is limited information related to how ADHD symptoms, autistic traits, and their combined effects are associated with nicotine, alcohol, and cannabis use and use disorders in the general population., Method: Cross-sectional interview and self-report questionnaire data from 3,080 young adult Australian twins (mean age 31.9 years) were used to assess ADHD symptoms, autistic traits, substance use, and substance use disorders. Substance use disorders-based on Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, criteria-were assessed in the full sample as well as in those who reported substance use. Logistic regression analyses were used for comparing the associations between ADHD symptoms, autistic traits, substance use, and substance misuse after conduct disorder, sex, age, and zygosity were controlled for., Results: Greater ADHD symptoms and autistic traits scores were associated with elevated levels of regular smoking; cannabis use; and nicotine, alcohol, and cannabis use disorders, even after conduct disorder was adjusted for. In contrast, for alcohol use, those with high autistic traits scores were less likely to report drinking to intoxication. However, upon initiation, and similar to the findings for nicotine and cannabis, they were at elevated risk for developing alcohol dependence., Conclusions: Increased liability to ADHD and elevated autistic traits scores were associated with substance use and misuse, with the exception of alcohol use. Given the social underpinnings of drinking, persons with autistic traits may be less likely to engage in it; however, upon engagement in drinking, their vulnerability to alcohol dependence is elevated.

Chemical investigations of two specimens of Trikentrion flabelliforme collected from Australian waters have resulted in the identification of four new indole alkaloids, trikentramides A-D (9-12). The planar chemical structures for 9-12 were established following analysis of 1D/2D NMR and MS data. The relative configurations for 9-12 were determined following the comparison of (1)H NMR data with data previously reported for related natural products. The application of a quantum mechanical modeling method, density functional theory, confirmed the relative configurations and also validated the downfield carbon chemical shift observed for one of the quaternary carbons (C-5a) in the cyclopenta[g]indole series. The indole-2,3-dione motif present in trikentramides A-C is rare in nature, and this is the first report of these oxidized indole derivatives from a marine sponge.