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Research

Functional skills in MECP2 duplication syndrome: developmental dynamics and regression

MECP2 duplication syndrome (MDS) is an ultrarare, X-linked neurodevelopmental disorder that is poorly understood in terms of its natural history and phenotypic variability. There is limited information on how individuals with MDS acquire, retain or lose fundamental functional skills (gross motor, purposeful hand function and communication) - that of which this study aimed to better characterise in the largest case series to date.

Research

MetaNeb Versus Usual Care During Exacerbations of Cystic Fibrosis: An RCT

During exacerbations, when symptom and treatment burden are increased, individuals with cystic fibrosis (CF) are likely to prefer airway clearance techniques (ACTs) that require minimal effort. Therefore, in adults with CF who were hospitalised with an exacerbation, we sought to compare the effect of the MetaNeb with usual ACTs on respiratory function and expectorated sputum.

Research

The effect of moderate-to-late preterm birth on lung function over the first 5 years of life in a South African birth cohort

Preterm birth is associated with increased mortality and morbidity, particularly due to lung disease. Low- and middle-income countries (LMIC) have the highest rates of preterm birth. Infants born extremely preterm rarely survive, so the largest burden is amongst moderate-to-late preterm (MLP) infants. The long-term health impact on MLP children in LMIC is poorly understood. The aim of this study was to assess the effect of MLP birth on lung function trajectories from birth to 5 years in the Drakenstein Child Health Study.

Research

The perspectives of autistic adolescents and their parents on sleep strategies for insomnia

Autistic adolescents are vulnerable to sleep difficulties, with up to 80 % experiencing sleep problems, most commonly insomnia. Little is known about how autistic adolescents are involved in their own sleep treatment, and their depth of knowledge about their sleep difficulties. The aims of this study were to investigate autistic adolescent and parent perspectives of experiencing and managing insomnia, and what factors influence the development of these perspectives on insomnia and treatment. 

Research

Oscillometry: clinical significance and applications

Respiratory oscillometry (or the forced oscillation technique) is a highly practical lung function test that can be applied in a wide range of clinical scenarios in children and adults, including the clinic, intensive care unit, patient home monitoring and emergency departments. Oscillometry measurements complement spirometry in detecting abnormal lung function, measuring effects of treatment such as inhaled corticosteroids or bronchodilators, and changes due to disease activity.

Research

Profile of the PLAY spaces & environments for children’s physical activity, sedentary behaviour and sleep (PLAYCE) cohort study, Western Australia

Childhood is a critical period for the development of movement behaviours such as physical activity, sleep and sedentary behaviour. The PLAYCE Cohort was established to investigate how movement behaviours change over early to middle childhood, across key behaviour settings and relationships with health and development. An overview of the PLAYCE cohort, summary of key findings to date, and future research opportunities are presented.

Research

Can lung disease be averted by focusing on early-life inequities?

Shannon Simpson BMedSci (hons), PhD Head, Strong Beginnings Research, Co-head Foundations of Lung Disease 08 6319 1631 Shannon.simpson@thekids.org.au

Research

Lung structural and functional impairments in young children with cystic fibrosis diagnosed following newborn screening – A nationwide observational study

Non-invasive and sensitive clinical endpoints are needed to monitor onset and progression of early lung disease in children with cystic fibrosis (CF). We compared lung clearance index (LCI), FEV1, functional and structural lung magnetic resonance imaging (MRI) outcomes in Swiss children with CF diagnosed following newborn screening. 

Research

Prematurity-associated lung disease: is it asthma?

Shannon Elizabeth Simpson Smith BMedSci (hons), PhD PhD, MSc, BSc Head, Strong Beginnings Research, Co-head Foundations of Lung Disease Program

Research

Antibiotics for the treatment of lower respiratory tract infections in children with neurodisability: Systematic review

Determine the optimal antibiotic choice for lower respiratory tract infection in children with neurodisability.