Top 10 research priorities for congenital diaphragmatic hernia in Australia: James Lind Alliance Priority Setting Partnership.

Objectives: The Gaps in the Congenital Diaphragmatic Hernia (CDH) Journey Priority Setting Partnership (PSP) was developed in collaboration with CDH Australia, James Lind Alliance (JLA) and the Murdoch Children's Research Institute to identify research priorities for people with CDH, their families and healthcare workers in Australasia.

Design: Research PSP in accordance with the JLA standardised methodology.

Setting: Australian community and institutions caring for patients with CDH and their families.

Intellectual Functioning of Children With Isolated PRS, PRS-Plus, and Syndromic PRS.

Objective: Describe the intelligence quotient (IQ) of children with Pierre Robin sequence (PRS).

Design: Prospective cohort study.

Setting: Neurodevelopmental follow-up clinic within a hospital.

The mediating role of ADHD symptoms between executive function and social skills in children with neurofibromatosis type 1.

Children with neurofibromatosis type 1 (NF1) often experience executive dysfunction, attention deficit/hyperactivity disorder (ADHD) symptoms and poor social skills, however, the nature of the relationships between these domains in children with NF1 is unclear. This study investigated these relationships using primary caregiver ratings of executive functions, ADHD symptoms and social skills in children with NF1. Participants were 136 children with NF1 and 93 typically developing (TD) controls aged 3-15 years recruited from 3 multidisciplinary neurofibromatosis clinics in Melbourne and Sydney, Australia, and Washington DC, USA.

Cognitive, academic, and behavioral functioning in school-aged children born with esophageal atresia.

Purpose: To characterize cognitive, academic, and behavioral functioning in children who underwent neonatal surgical repair of esophageal atresia (OA) and compare outcomes according to clinical characteristics (presence of additional congenital anomalies, longer hospitalization, and prematurity).

Methods: Intellectual, language, attention, and executive functioning were assessed in 71 5-year-olds and 72 8-year-olds born with OA. At 8 years, memory and academic skills were also assessed.

Post-traumatic stress symptoms and cognition in children exposed to motor vehicle accident trauma.

Research investigating the cognition of children exposed to non-familial trauma is scarce and the effects of post-traumatic stress symptoms in this population remain unclear. Thus, this research aimed to investigate the cognition of children exposed to motor vehicle accidents given the high incidence of this trauma globally. It was hypothesized that children with post-traumatic stress symptoms (PTSS; i.

Neonatal neuroimaging after repair of congenital diaphragmatic hernia and long-term neurodevelopmental outcome.

Objective: Previous outcome reports of congenital diaphragmatic hernia (CDH) have described neuroimaging anomalies and neurodevelopmental impairment. However, the link between imaging and outcome has not been described. We aimed to determine whether routine postoperative neonatal neuroimaging in infants with CDH detects later neurodevelopmental impairment.

Outcome of vein of Galen malformation presenting in the neonatal period.

Objective: Vein of Galenaneurysmal malformation (VGAM) is a rare but important congenital malformation presenting to neonatal intensive care units (NICUs), and with a change from surgical to endovascular management, survival for this condition has improved. However, there is little reported about the medical management decisions of infants with this condition and the associated long-term neurodevelopmental outcomes. We aim to report a single centre experience of both acute treatment and long-term outcomes of VGAM for those infants admitted to our NICU soon after birth.

Effects of methylphenidate on cognition and behaviour in children with neurofibromatosis type 1: a study protocol for a randomised placebo-controlled crossover trial.

Introduction: Dopamine dysregulation has been identified as a key modulator of behavioural impairment in neurofibromatosis type 1 (NF1) and a potential therapeutic target. Preclinical research demonstrates reduced dopamine in the brains of genetically engineered NF1 mouse strains is associated with reduced spatial-learning and attentional dysfunction. Methylphenidate, a stimulant medication that increases dopaminergic and noradrenergic neurotransmission, rescued the behavioural and dopamine abnormalities.

Social Function and Autism Spectrum Disorder in Children and Adults with Neurofibromatosis Type 1: a Systematic Review and Meta-Analysis.

In light of the proliferation of recent research into social function in neurofibromatosis type 1 (NF1), a systematic review and meta-analysis is required to synthesise data and place findings within the context of a theoretical framework. This paper reviews findings from research into social function and autism spectrum disorder (ASD) in children and adults with NF1 and integrates these findings with the Socio-Cognitive Integration Abilities Model (SOCIAL). It also critically appraises links between social outcomes, internal and external factors moderating social functioning, cognitive domains implicated in social functioning, and underlying neural pathology in NF1.

Cognitive deficits and posttraumatic stress disorder in children: A diagnostic dilemma illustrated through a case study.

Studies investigating the neuropsychological functioning of children who experience trauma have predominantly focused on maltreated populations. This article presents a case study that details the longitudinal outcome of a girl who experienced a motor vehicle accident at 5 years of age. It highlights the clinical relevance of research investigating the neuropsychological impact of single-incident trauma on children.

Characterizing the behavior of children emerging with delirium from general anesthesia.

Background: Emergence delirium (ED) frequently occurs in young children awakening from general anesthesia (GA). To date, research is limited by scales that are unable to discriminate the condition from other forms of agitation.

Aim: The primary aim of this study was to determine the core behaviors of ED that discriminate the condition from pain and tantrum in young children and to cluster these behaviors according to the DSM-IV/V core diagnostic criteria and associated behaviors of delirium.