Abstract
BackgroundChildhood interstitial lung disease (chILD) represents a rare heterogeneous group of respiratory disorders. In the absence of randomized controlled clinical trials, global collaborations have utilized case series with an aim to standardising approaches to diagnosis and management. Australasian data are lacking. The aim of this study was to calculate prevalence and report the experience of chILD in Australasia over a decade.MethodsPaediatric pulmonologists in Australia and New Zealand involved in the care of patients aged 0–18 years with chILD completed a questionnaire on demographics, clinical features and outcomes, over a 10 year period. These data, together with data from the 2 reference genetics laboratories, were used to calculate prevalence.ResultsOne hundred fifteen cases were identified equating to a period prevalence (range) of 1.5 (0.8–2.1) cases/million for children aged 0–18years. Clinical data were provided on 106 patients: the <2 year group comprised 66 children, median age (range) 0.50 years (0.01–1.92); the ≥2 year group comprised 40 children, median age 8.2 years (2.0–18.0). Management approach was heterogeneous. Overall, 79% of patients had a good clinical outcome. Mortality rate was 7% in the study population.ConclusionchILD is rare in Australasia. This study demonstrates variation in the investigations and management of chILD cases across Australasia, however the general outcome is favorable. Further international collaboration will help finesse the understanding of these disorders.
Highlights
Childhood interstitial lung disease represents a rare heterogeneous group of respiratory disorders
Childhood interstitial lung disease is a heterogenous group of rare chronic respiratory disorders in children, most prevalent in early infancy. It is associated with variable lung pathology which often impairs gas exchange [1]. chILD is characterized by dyspnoea, tachypnoea, crackles, hypoxaemia, failure to thrive and results in significant morbidity and mortality [2, 3]
Eight tertiary hospitals across all States in Australia, and a tertiary paediatric hospital in Auckland, NZ participated in the study
Summary
Childhood interstitial lung disease (chILD) represents a rare heterogeneous group of respiratory disorders. Childhood interstitial lung disease (chILD) is a heterogenous group of rare chronic respiratory disorders in children, most prevalent in early infancy. It is associated with variable lung pathology which often impairs gas exchange [1]. The classification of chILD has evolved as new disorders have been identified. As these conditions are very rare, there are limited scientific data on which to inform appropriate management, with a compelling need to develop this evidence base through national and international collaboration
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