Identification of a T2-hyperintense Perivascular Space in Brain Arteriovenous Malformations.

Background/aim: Brain arteriovenous malformations (AVMs) are vascular malformations characterized by dysmorphic, aberrant vasculature. During previous surgeries of compact nidus brain AVMs (representing the majority of cases), we have observed a "shiny" plane between nidal and perinidal AVM vessels and the surrounding grey and white matter and hypothesized that preoperative neuroimaging of brain AVMs may show a neuroradiological correlate of these intraoperative observations.

Patients And Methods: We retrospectively reviewed and analyzed multiplanar and multisequence 3-Tesla magnetic resonance (3T MR) imaging in five consecutive brain AVMs with special attention on imaging characteristics of the brain-AVM interface, i.

Proteome profiling of cerebrospinal fluid using machine learning shows a unique protein signature associated with APOE4 genotype.

Proteome changes associated with APOE4 variant carriage that are independent of Alzheimer's disease (AD) pathology and diagnosis are unknown. This study investigated APOE4 proteome changes in people with AD, mild cognitive impairment, and no impairment. Clinical, APOE genotype, and cerebrospinal fluid (CSF) proteome and AD biomarker data was sourced from the Alzheimer's Disease Neuroimaging Initiative (ADNI) database.

A retrospective review of sildenafil in the Australia and New Zealand Fontan registry: indications and treatment responses.

Introduction: Rates of morbidity and mortality are high in the setting of Fontan physiology and effective medical therapies are not well-established. Clinical trials assessing phosophodiesterase-5-inhibitors, such as sildenafil, have not demonstrated major benefit in patients with a Fontan-type circulation but have only included stable, well-functioning people.

Methods: We sought to retrospectively characterize the people followed by the ANZ Fontan Registry prescribed sildenafil >30 days post Fontan-surgery.

A national Australian Congenital Heart Disease registry; methods and initial results.

Background: Although several National Data Registries for Congenital Heart Disease (CHD) exist, few are comprehensive and contemporary. A National Australian CHD Registry has been developed that aims to redress this by creating the first comprehensive data collection for CHD children and adults, initially across Australia.

Methods: We defined and collected a minimum dataset of demographics, diagnoses, and procedures from people with CHD presenting at participating quaternary CHD services Australia-wide.

Evaluation of the Queensland JEV vaccine program response to the 2022 Australian outbreak.

In 2022, the largest ever virgin soil outbreak of Japanese encephalitis (JE) occurred in Australia resulting in 45 reported human cases of JE, with seven fatalities. Japanese encephalitis virus (JEV) was detected in 84 piggeries across Australia. In response, states implemented targeted vaccination programs for those individuals at the highest risk of JEV exposure.

Glucocorticoid dosing and implications for vaccination: Evolution of global definitions.

Despite widespread adoption of "high-dose" glucocorticoid definitions across international immunisation guidelines (i.e., prednisone-equivalent dosing >20 mg/day, or >2 mg/kg/day in children), the rationale remains unclear.

A multitiered analysis platform for genome sequencing: Design and initial findings of the Australian Genomics Cardiovascular Disorders Flagship.

Purpose: The Australian Genomics Cardiovascular Disorders Flagship was a national multidisciplinary collaboration. It aimed to investigate the feasibility of genome sequencing (GS) and functional genomics to resolve variants of uncertain significance (VUS) in the clinical management of patients and families with cardiomyopathies, primary arrhythmias, and congenital heart disease (CHD).

Methods: Between April 2019 and December 2021, 600 probands meeting cardiovascular disorder criteria from 17 cardiology and genetics clinics across Australia were enrolled in the Flagship and underwent GS.

Defining a core set of research and development priorities for virtual care in the post-pandemic environment: a call to action.

Objectives: To identify research and development priorities for virtual care following the coronavirus disease 2019 pandemic from the perspective of key stakeholders (patients, clinicians, informaticians and academics).

Design: Qualitative study using a modified nominal group technique.

Setting: Online semi-structured interviews and workshops held in November 2022 and February 2023.

Codesign and evaluation of advanced therapeutic information resources for and with families of children with neurological conditions: a mixed methods cross-sectional study.

Objective: Parents and caregivers of children with neurological conditions express interest in new and developing treatments and trials; however, they have limited knowledge of, and access to, reliable information. This study aims to empower and equip decision-making and support communication in the application of advanced neurotherapeutics and personalised medicine, covering gene therapy, stem cell therapy, neurostimulation and neuroimmunotherapies.

Design: A suite of online psychoeducational resources has been created and evaluated to establish implementation success.

Strategies to Overcome Barriers to Physical Activity Participation in Children and Adults Living With Congenital Heart Disease: A Narrative Review.

Physical activity participation is critical for optimal physical, psychological, and cognitive health in children and adults living with congenital heart disease (CHD). Majority of the general population are not sufficiently active, and with the added psychological, physical, and socioeconomic barriers faced by individuals with CHD, it is unsurprising that many people living with CHD do not meet the recommendations for physical activity either. The aim of this review is to outline lifelong physical activity barriers faced by individuals living with CHD and provide age-appropriate strategies that can be used to ensure the development of long-term positive physical activity behaviours.

A single risk assessment for the most common diseases of ageing, developed and validated on 10 cohort studies.

Background: We aimed to develop risk tools for dementia, stroke, myocardial infarction (MI), and diabetes, for adults aged ≥ 65 years using shared risk factors.

Methods: Data were obtained from 10 population-based cohorts (N = 41,755) with median follow-up time (years) for dementia, stroke, MI, and diabetes of 6.2, 7.

Predictors of school attendance among children with cerebral palsy in Bangladesh.

Aim: To determine school attendance and its predictors among children with cerebral palsy (CP) in Bangladesh using population-based data.

Method: This study utilized data from the Bangladesh Cerebral Palsy Register (BCPR), a population-based register of children with CP aged less than 18 years in Bangladesh. Sociodemographic, clinical, and educational data were documented, and descriptive statistics and multivariate regression analyses were used to identify potential predictors of school attendance.

Intracranial Arterial Aneurysms in Childhood.

Intracranial arterial aneurysms in children are rare. They differ from adult aneurysms in their etiology, natural history, and management approach. Unruptured asymptomatic aneurysms in children can often be observed for growth over time.

Travel-associated illness in children in pre-pandemic Western Sydney, 2018-2020.

Unlabelled: Objectives and importance of study: Australian children frequently travel overseas, but little is known about their travel-related morbidity. We aimed to describe the spectrum of illness and injury in returned travellers presenting to the largest paediatric referral centre in NSW, the Children's Hospital at Westmead (CHW).

Study Type: Observational cohort study.

The CHD severity classification system: development of a tool to assist with disease stratification for CHD research.

Background: Complexity stratification for CHD is an integral part of clinical research due to its heterogenous clinical presentation and outcomes. To support our ongoing research efforts into CHD requiring disease severity stratifications, a simplified CHD severity classification system was developed and verified, with potential utility for clinical researchers without specialist CHD knowledge or access to clinical/medical records.

Method: A two-tiered analysis approach was undertaken.

Parental Perceptions and Knowledge towards Asthma Action Plans.

Introduction: Personalised Asthma Action Plans (AAPs) are a cornerstone of paediatric asthma management. We aimed to assess parental knowledge and perceptions of the use of AAPs in managing their child's asthma.

Methods: In 2023 we conducted a cross-sectional survey to assess knowledge and perceptions of AAPs in parents/caregivers of children aged 0-17 years with asthma who had an AAP and attended respiratory clinics at Sydney Children's Hospital, Randwick.

Multiplexed Assays of Variant Effect and Automated Patch Clamping Improve -LQTS Variant Classification and Cardiac Event Risk Stratification.

Background: Long QT syndrome is a lethal arrhythmia syndrome, frequently caused by rare loss-of-function variants in the potassium channel encoded by . Variant classification is difficult, often because of lack of functional data. Moreover, variant-based risk stratification is also complicated by heterogenous clinical data and incomplete penetrance.

Antimicrobial susceptibility profiles of invasive bacterial infections among children from low- and middle-income countries in the Western Pacific Region (WPRO) - a systematic review and meta-analysis.

Background: Antimicrobial resistance increasingly impacts paediatric mortality, particularly in resource-constrained settings. We aimed to evaluate the susceptibility profiles of bacteria causing infections in children from the Western Pacific region.

Methods: We conducted a systematic review and meta-analysis of bacteria responsible for common infections in children.