Directed Forgetting and the Production Effect.

The item-based directed-forgetting effect is explained as a difference in how strongly people encode remember-cued over forget-cued targets. In contrast, the production effect is typically explained as a difference in the distinctiveness of the memory of produced over unproduced targets. The procedural alignment of the two effects - directing participants to remember or forget, produce or not - coupled with their different theoretical explanations (i.

Vascular abnormalities not to miss on routine chest CT: A pictorial review.

There is a wide and ever-increasing range of imaging indications for thoracic computed tomography (CT). Identifying and appropriately triaging cardiovascular findings is often challenging, especially in non-gated or unenhanced studies. The authors provide a pictorial review of clinically relevant abnormalities of the main intrathoracic vessels (aorta, superior vena cava, pulmonary arteries and coronary arteries), for radiologists reporting non-gated enhanced or unenhanced CT of the thorax.

THE FIGHT INHERITED RETINAL BLINDNESS! PROJECT: A NEW TREATMENT OUTCOME AND NATURAL HISTORY REGISTRY FOR INHERITED RETINAL DISEASE.

Purpose: To design and build a new disease registry to track the natural history and outcomes of approved gene therapy in patients with inherited retinal diseases (IRDs).

Methods: A core committee of 6 members was convened to oversee the construction of the FIRB! module. A further 11 experts formed a steering committee, which discussed disease classification and variables to form minimum datasets via a consensus approach.

Repeated pulses of volcanism drove the end-Permian terrestrial crisis in northwest China.

The Permo-Triassic mass extinction was linked to catastrophic environmental changes and large igneous province (LIP) volcanism. In addition to the widespread marine losses, the Permo-Triassic event was the most severe terrestrial ecological crisis in Earth's history and the only known mass extinction among insects, but the cause of extinction on land remains unclear. In this study, high-resolution Hg concentration records and multiple-archive S-isotope analyses of sediments from the Junggar Basin (China) provide evidence of repeated pulses of volcanic-S (acid rain) and increased Hg loading culminating in a crisis of terrestrial biota in the Junggar Basin coeval with the interval of LIP emplacement.

Retinal Dystrophies Associated With Peripherin-2: Genetic Spectrum and Novel Clinical Observations in 241 Patients.

Purpose: To describe the clinical, electrophysiological and genetic spectrum of inherited retinal diseases associated with variants in the PRPH2 gene.

Methods: A total of 241 patients from 168 families across 15 sites in 9 countries with pathogenic or likely pathogenic variants in PRPH2 were included. Records were reviewed for age at symptom onset, visual acuity, full-field ERG, fundus colour photography, fundus autofluorescence (FAF), and SD-OCT.

Genomic multidisciplinary teams: A model for navigating genetic mainstreaming and precision medicine.

Aim: Recent rapid advances in genomics are revolutionising patient diagnosis and management of genetic conditions. However, this has led to many challenges in service provision, education and upskilling requirements for non-genetics health-care professionals and remuneration for genomic testing. In Australia, Medicare funding with a Paediatric genomic testing item for patients with intellectual disability or syndromic features has attempted to address this latter issue.

Electrophysiological Characterization of Subclinical and Overt Hypertrophic Cardiomyopathy by Magnetic Resonance Imaging-Guided Electrocardiography.

Background: Ventricular arrhythmia in hypertrophic cardiomyopathy (HCM) relates to adverse structural change and genetic status. Cardiovascular magnetic resonance (CMR)-guided electrocardiographic imaging (ECGI) noninvasively maps cardiac structural and electrophysiological (EP) properties.

Objectives: The purpose of this study was to establish whether in subclinical HCM (genotype [G]+ left ventricular hypertrophy [LVH]-), ECGI detects early EP abnormality, and in overt HCM, whether the EP substrate relates to genetic status (G+/G-LVH+) and structural phenotype.

What is the power of a genomic multidisciplinary team approach? A systematic review of implementation and sustainability.

Due to the increasing complexity of genomic data interpretation, and need for close collaboration with clinical, laboratory, and research expertise, genomics often requires a multidisciplinary team (MDT) approach. This systematic review aims to establish the evidence for effectiveness of the genomic multidisciplinary team, and the implementation components of this model that can inform precision care. MEDLINE, Embase and PsycINFO databases were searched in 2022 and 2023.

Proliferation and anatoxin production of benthic cyanobacteria associated with canine mortalities along a stream-lake continuum.

Proliferations of benthic cyanobacteria are increasingly in the public eye, with rising animal deaths associated with benthic rather than planktonic blooms. In early June 2021, two dogs died after consuming material on the shore of Shubenacadie Grand Lake, Nova Scotia. Preliminary investigations indicated anatoxins produced by benthic cyanobacterial mats were responsible for the deaths.

Managing the apparently blind child presenting in the first year of life: A review.

Severe vision impairment and blindness in childhood have a significant health burden on the child, family and society. This review article seeks to provide a structured framework for managing the apparently blind child presenting in the first year of life, starting from a comprehensive history and examination. Different investigation modalities and the increasingly important role of genetics will also be described, in addition to common causes of severe vision impairment.

Where does the carbon go? Long-term effects of forest management on the carbon budget of a temperate-forest water-supply watershed.

While forest management commonly seeks to increase carbon (C) capture and sequestration, in some settings, a high density of C storage may be detrimental to other land uses and ecosystem services. We study a forested, drinking-water-supply watershed to determine the effects of forest management on C storage with the implicit understanding that greater storage of C will lead to increased quantity of carbon exported hydrologically into a source-water reservoir. Using a custom implementation of CBM-CFS3, a Canadian model to simulate C transformations and movement in forested systems, and a custom forest disturbance and management model, we simulate various management scenarios and their C outcomes.

AAV capsid bioengineering in primary human retina models.

Adeno-associated viral (AAV) vector-mediated retinal gene therapy is an active field of both pre-clinical as well as clinical research. As with other gene therapy clinical targets, novel bioengineered AAV variants developed by directed evolution or rational design to possess unique desirable properties, are entering retinal gene therapy translational programs. However, it is becoming increasingly evident that predictive preclinical models are required to develop and functionally validate these novel AAVs prior to clinical studies.

A natural history study of autosomal dominant GUCY2D-associated cone-rod dystrophy.

Purpose: To describe the natural history of autosomal dominant (AD) GUCY2D-associated cone-rod dystrophies (CRDs), and evaluate associated structural and functional biomarkers.

Methods: Retrospective analysis was conducted on 16 patients with AD GUCY2D-CRDs across two sites. Assessments included central macular thickness (CMT) and length of disruption to the ellipsoid zone (EZ) via optical coherence tomography (OCT), electroretinography (ERG) parameters, best corrected visual acuity (BCVA), and fundus autofluorescence (FAF).

Simulating fate and transport of non-steroidal anti-inflammatory drugs using Root Zone Water Quality Model 2.

The fate and transport of non-steroidal anti-inflammatory drugs (NSAIDs) in soil are determined by various processes, and the complexity of the system lends itself to the use of computer simulation models to help understand it. This study demonstrated the first attempt to use empirical data from lab incubation and field studies to parameterize and test a process-based agricultural systems model, Root Zone Water Quality Model 2 (RZWQM2), to simulate the fate and transport of naproxen (NPX), ibuprofen (IBF), and ketoprofen (KTF) in field-based lysimeters amended with alkaline-treated biosolids (ATBs). The model calibrated for the soil-water balance module and contaminant transport module was used to predict water seepage through the soil profile in 2017 and 2018 within a 15% error of the field measured data, with model performance statistics such as Nash-Sutcliffe model efficiency (NSE) and R all greater than 0.

Prevalence of Glaucoma Following Paediatric Cataract Surgery in an Australian Tertiary Referral Centre.

Purpose: Secondary glaucoma following childhood cataract surgery remains the most common complication in the paediatric population. This study aimed to determine the incidence, time to progression and risk factors associated with the development of secondary glaucoma following childhood cataract surgery in a paediatric population. Outcome measures were the detection of secondary glaucoma, postoperative time frame to development of glaucoma and risk factors in its development.

A computational account of item-based directed forgetting for nonwords: Incorporating orthographic representations in MINERVA 2.

Recent research on item-method directed forgetting demonstrates that forget instructions not only decrease recognition for targets, but also decrease false recognition for foils from the same semantic categories as targets instructed to be forgotten. According to the selective rehearsal account of directed forgetting, this finding suggests that remember instructions may engage elaborative rehearsal of the category-level information of items. In contrast to this explanation, Reid and Jamieson (Canadian Journal of Experimental Psychology / Revue canadienne de psychologie expérimentale, 76(2), 75-86, 2022) proposed that the differential rates of false recognition may emerge at retrieval when foils from "remember" and "forget" categories are compared to traces in memory.

The health care and societal costs of inherited retinal diseases in Australia: a microsimulation modelling study.

Objectives: To estimate the health care and societal costs of inherited retinal diseases (IRDs) in Australia.

Design, Setting, Participants: Microsimulation modelling study based on primary data - collected in interviews of people with IRDs who had ophthalmic or genetic consultations at the Children's Hospital at Westmead or the Save Sight Institute (both Sydney) during 1 January 2019 - 31 December 2020, and of their carers and spouses - and linked Medicare Benefits Schedule (MBS) and Pharmaceutical Benefits Schedule (PBS) data.

Main Outcome Measures: Annual and lifetime costs for people with IRDs and for their carers and spouses, grouped by payer (Australian government, state governments, individuals, private health insurance) and type (health care costs; societal costs: social support, National Disability Insurance Scheme (NDIS), income and taxation, costs associated with caring for family members with IRDs); estimated annual national cost of IRDs.

A direct replication and extension of Popp and Serra (2016, experiment 1): better free recall and worse cued recall of animal names than object names, accounting for semantic similarity.

Introduction: Free recall tends to be better for names of animate concepts such as animals than for names of inanimate objects. In Popp and Serra's 2016 article, the authors replicated this "animacy effect" in free recall but when participants studied words in pairs (animate-animate pairs intermixed with inanimate-inanimate pairs) and were tested with cued recall, performance was better for inanimate-inanimate pairs than for animate-animate pairs ("reverse animacy"). We tested the replicability of this surprising effect and one possible explanation for the effect (semantic similarity).

Effect of biosolids amendment on the fate and mobility of non-steroidal anti-inflammatory drugs (NSAIDs) in a field-based lysimeter cell study.

Non-steroidal anti-inflammatory drugs (NSAIDs) are widely used globally to treat and prevent illness. Biosolids change physico-chemical characteristics of soil and can affect the mobility of NSAIDs. A field-based lysimeter study evaluated the effect of three rates (0, 7, and 28 Mg ha) of alkaline treated biosolids (ATB) on the leaching potential of naproxen (NPX), ibuprofen (IBF), and ketoprofen (KTF) over 34 days in a sandy loam textured soil.

-associated autosomal dominant retinitis pigmentosa: natural history of novel variant Lys314Gln and a comprehensive literature search.

Background: Inosine monophosphate dehydrogenase (IMPDH) is a key regulatory enzyme in the de novo synthesis of the purine base guanine. Mutations in the inosine monophosphate dehydrogenase 1 gene (IMPDH1) are causative for RP10 autosomal dominant retinitis pigmentosa (adRP). This study reports a novel variant in a family with IMPDH1-associated retinopathy.

Spectrum of ocular disease in children aged between 0 and 3 years at an Australian paediatric tertiary hospital.

Background: Childhood ocular disease can be a significant health burden to the child, family and society. Previous studies have examined the spectrum of paediatric ocular disease presenting to tertiary hospitals; however, these studies have broader age ranges, smaller sample sizes, and are largely based in developing countries. This study aims to assess the spectrum of ocular disease in the first 3 years of life presenting to the eye department of an Australian tertiary paediatric hospital.

Scalable cognitive modelling: Putting Simon's (1969) ant back on the beach.

A classic goal in cognitive modelling is the integration of process and representation to form complete theories of human cognition (Estes, 1955). This goal is best encapsulated by the seminal work of Simon (1969) who proposed the parable of the ant to describe the importance of understanding the environment that a person is embedded within when constructing theories of cognition. However, typical assumptions in accounting for the role of representation in computational cognitive models do not accurately represent the contents of memory (Johns & Jones, 2010).

Early recognition of CLN3 disease facilitated by visual electrophysiology and multimodal imaging.

Background: Neuronal ceroid lipofuscinosis is a group of neurodegenerative disorders with varying visual dysfunction. CLN3 is a subtype which commonly presents with visual decline. Visual symptomatology can be indistinct making early diagnosis difficult.