Severe dilated cardiomyopathy in children may uncommonly be caused by abnormal loading conditions such as mid-aortic pathology and renal artery stenosis. Refractory hypertension and left ventricular dilatation with hypertrophy are important clues to reversible causes. We present a case of dilated cardiomyopathy in a child secondary to mid-aortic syndrome with renal artery stenosis.

To evaluate growth and body composition at NICU discharge in term-born neonates with structural congenital anomalies and identify predictors of body fat percentage. This prospective observational study included 61 term neonates (≥ 37 weeks' gestation) with cardiac or non-cardiac congenital anomalies requiring surgery. Anthropometric measures at birth and discharge and body composition at discharge were measured. Mean z-scores declined significantly between birth and discharge for weight (-0.38 to -1.53), length (-0.02 to -0.64), and head circumference (-0.04 to -0.79) (all p < 0.001). Infants with congenital heart disease demonstrated a more pronounced weight z-score decline. At discharge, mean body fat was 9% (SD 4.9), with no significant differences between cardiac and non-cardiac anomalies or sexes. Fat-free mass was relatively preserved (91%, 910 g/kg). Multivariate analysis identified time of full enteral feeds (p = 0.02) and maternal ethnicity (p = 0.03) as independent predictors of body fat. Term neonates with congenital anomalies experience significant postnatal growth restriction and reduced fat mass by NICU discharge. Maternal ethnicity and time to full enteral feeds independently influence body fat percentage. These findings support the need for individualised nutrition and routine body composition monitoring to optimise outcomes in this high-risk group.

The 2017 Paediatric Research in Emergency Departments International Collaborative (PREDICT) Bronchiolitis Knowledge Translation (KT) Study, a cluster randomised trial in 26 Australasian hospitals, found targeted interventions provided over one bronchiolitis season effectively de-implemented five low-value practices (salbutamol, glucocorticoids, chest radiography, antibiotics and epinephrine) by 14.1% (adjusted risk difference, 95% CI 6.5% to 21.7%; p<0.001). A 2-year follow-up study found de-implementation was sustained. This process evaluation aimed to identify factors that influenced sustainability of de-implementation of these five low-value practices in PREDICT Bronchiolitis KT Study intervention hospitals and examine fidelity and/or adaptation of the targeted interventions over 4 years post intervention delivery (sustainment). Semistructured qualitative interviews were conducted, over 2021 and 2022, with a purposive sample of emergency department (ED) and paediatric inpatient clinicians. Data were analysed thematically into facilitators and barriers using the Consolidated Framework for Sustainability Constructs in Healthcare (CFSCH). The Framework for Reporting Adaptations and Modifications to Evidence-based Implementation Strategies was used to explore fidelity and adaptation. 50 clinicians (nurses: n=26; doctors: n=24) from 12 intervention hospitals were interviewed. Eight themes were identified and mapped to three CFSCH domains: (1) organisational setting; (2) initiative design and delivery and (3) people involved. Facilitators were a culture of evidence-based practice, ongoing multimodal education, strong clinical leadership as unofficial champions and the previous effectiveness of the PREDICT Bronchiolitis KT Study interventions. Barriers were lack of paediatric trained ED staff, assumptions by senior clinicians that junior doctors can provide evidence-based bronchiolitis management, bronchiolitis not a current improvement priority and lack of bronchiolitis education sessions. Use of the targeted interventions reduced over time and, when used, was adapted locally. This study provides insights into factors influencing the sustainability of de-implementation of low-value care in acute care settings. Fostering an evidence-based practice culture, supported by senior leadership and ongoing multimodal education, supports sustainability of improvements in this setting. Australian and New Zealand Clinical Trials Registry No: ACTRN12621001287820.

Abstract Background Joubert Syndrome (JS) is a rare autosomal recessive neurodevelopmental disorder, characterised by hypotonia, developmental delay, and a “molar tooth sign” on MRI brain. Sleep disordered breathing (SDB) is common, although referral for polysomnography (PSG) is variable. The aim of this study was to collate overnight PSG results from a series of patients with JS, and monitor the pattern of SDB over time. Methods A retrospective analysis of children with JS in a tertiary paediatric hospital from 2010-2025. Progess to date Twenty-one children (16 males) with confirmed JS were identified during the study period. Fourteen had PSGs, at a median age of 4 years (1mo – 10 years), while 11 had repeat PSGs at a median age of 5 years. Genetic sequencing has been performed in 18. Initial and repeat PSGs have been analysed. The mean initial AHI was 9.4 events/hr, compared to 7.2 events/hr in the repeat. The mean OAHI in initial studies was 5.4 events/hr compared to 2.2 events/hr in repeat studies. Six children received treatment between studies, including adenotonsillectomy, non-invasive ventilation, or a combination of both. Objective improvement was seen in 7 children, while 3 had stable but persisting SDB. SDB worsened in 1 child. Intended outcome and impact Children with JS show a range of SDB, ranging from normal PSGs, to those showing severe OSA requiring varying levels of intervention. Patients with JS would benefit from serial PSGs during childhood, although not all are referred for sleep assessment. More awareness is required to ensure these children get adequate monitoring.

Abstract Behavioural sleep problems are highly prevalent in children with neurodisability (ND) and can severely impact daytime function and family wellbeing. Management strategies are extrapolated from neurotypical populations and may not address the complex needs of children with ND. This study describes the development and pilot testing of a behavioural sleep intervention tailored for children with ND, aiming to determine feasibility and acceptability prior to use in a randomised controlled trial. A tailored behavioural sleep program for children with ND was developed using a collaborative framework that incorporated consumer and stakeholder consultation. Stakeholders included allied health professionals, paediatric sleep nurses and sleep specialists. The final program prototype was piloted with eight caregivers of children with ND recruited from a tertiary sleep clinic. Caregivers received the intervention through two psychologist led telehealth sessions. The intervention program comprised two sessions (1) Sleep Foundations and (2) Sleep Strategies and Relapse Prevention. Within pilot testing, Child Sleep Habits Questionnaire total scores reduced by 10%, 4 weeks after program completion. Greatest improvements were observed in sleep onset delay (22%), sleep duration (14.8%) and sleep anxiety (13.9%) subscales. Acceptability and feasibility of the program was high (84%). Parents found the program valuable, relevant and appropriately tailored for their child’s needs. This study demonstrates the use of a collaborative framework with consumer and stakeholder consultation to develop a tailored behavioural sleep program for children with ND. Pilot results show promise for efficacy of this intervention which is being evaluated in a multi-centre randomised controlled trial.

Neonatal encephalopathy (NE) is a significant global health concern. It is a leading cause of long-term neurodevelopmental impairment, with hypoxic-ischaemic perinatal brain injury being the most common underlying contributor. Although therapeutic hypothermia has reduced mortality and improved outcomes for some affected infants, many survivors experience neurodevelopmental disability, including cerebral palsy and/or deficits in cognition, behaviour, and executive functioning. Early and accurate prognostication and identification of injury severity remain a challenge due to evolving clinical signs and multiple etiologies. Magnetic resonance imaging (MRI) is the gold standard for characterizing NE-related brain injury. Diffusion-weighted imaging (DWI) enables early detection of injury, and proton magnetic resonance spectroscopy (1H-MRS), specifically the Lac/NAA peak area ratio from basal ganglia and thalamus, provides robust prognostic indicators of two-year neurodevelopmental outcomes. MRI scoring systems incorporating multiple modalities correlate well with later neurodevelopmental outcomes. Advanced imaging modalities, such as diffusion tensor imaging (DTI), arterial spin labelling (ASL), and blood oxygen level-dependent (BOLD) imaging, offer further insights into microstructural integrity, perfusion, and functional connectivity. By standardizing acquisition protocols and post-processing, MRI biomarkers can serve as reliable, early surrogate endpoints in neuroprotection trials, allowing smaller sample sizes and accelerating clinical translation. MRI and 1H-MRS integration enhances prognostication, guides clinical management, and supports informed decision-making in NE care. IMPACT: This article highlights the importance of state-of-the-art MRI and MRS techniques for assessing neonatal encephalopathy (NE), emphasizing optimized protocols, accurate interpretation, and the use of MRI scoring systems to enhance clinical decision-making. It provides a comprehensive guide to advanced MRI/MRS acquisition and interpretation in neonates with NE, addressing current limitations and future directions. By optimizing neonatal MRI/MRS practices, this work aims to improve early diagnosis and prognostication, guide treatment strategies, and ultimately improve the management of neonates with NE.

Abstract Introduction Children with neurodisability (ND) are at increased risk of pervasive sleep disturbances, creating significant implications and challenges for their health, learning and development which, in turn, impacts family well-being. Despite this, prevalence data highlighting the extent and nature of sleep problems in Australian children with ND remains scarce. Methods This cross-sectional study collected parent-reported data from 300 Australian families of children with ND using the Modified Simonds and Parraga Sleep Questionnaire (MSPSQ). Participants were recruited from two sub-groups 1) from three tertiary sleep clinics and (2) from the community recruited through community support organisations. MSPSQ total (TS) and composite scores (CI) were used to quantify overall and domain-specific sleep disturbances. Statistical analyses explored differences by age, ND sub-groups, and referral source. Results Across the sample, 72% of children scored (M = 69.01: SD ±15.77) above clinical cut off (56), indicating a high prevalence of clinically significant sleep disturbance. Diagnostic categories were stratified into 4 broad ND subgroups (genetic syndromes, ASD/ADHD, neurological, Other). All groups scored above the threshold, with the ASD/ADHD group showing greater severity of sleep disturbance. Primary school aged children had the highest sleep disturbance scores. Notably, the community sample reported significantly higher composite scores (CI) than the hospital-referred group, yet nearly half had never accessed specialist sleep services. Conclusion This study highlights the prevalence of significant sleep disturbance in children with ND and aims to bring attention to the need for routine sleep screening and targeted intervention. Addressing this hidden burden could meaningfully improve child and family outcomes.

Acute appendicitis is the most common paediatric emergency presentation requiring timely surgical intervention. The Sydney Children's Hospital Network (SCHN) noted in their National Surgical Quality Improvement Program-Pediatrics (NSQIP-P) data that their appendicectomy postoperative length of stay (LOS) was longer than that of other hospitals. NSQIP-P reports are calculated from case reviews with a 30-day post-operative follow-up. From 2020 to 2022, the post-operative LOS for uncomplicated appendicectomy at SCHN was a median of 24 h (n = 714). A quality improvement project was initiated using Clinical Redesign Methodology. The project team developed an Uncomplicated Appendicectomy Guideline to standardize care. The Guideline also included a criteria-led discharge that could be applied after 6 h of post-operative recovery. Comprehensive information was provided for parents/carers on discharge. In 2024, the median postoperative LOS was reduced from 24 to 16 h (n = 185). This represents a statistically significant reduction in post-operative length of stay of 33% (p < 0.001). There were no statistically significant increase in post-operative readmissions (p = 0.95) or in NSQIP-P morbidity rates, although there was an increase in post-operative Emergency Department presentations noted, from a median of 7-10 annual presentations (p < 0.05, n = 444). There was positive feedback from parents/carers with the earlier discharge (n = 16). Parents/carers who received the discharge information sheet reported that they were better informed about their child's care. Staff in focus groups highlighted a greater autonomy in post-operative care (n = 24). Paediatric patients with uncomplicated appendicitis can be safely discharged from 6 h post-operatively with comprehensive discharge information.