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- Title
Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade's experience.
- Authors
Saddi, Vishal; Beggs, Sean; Bennetts, Bruce; Harrison, Joanne; Hime, Neil; Kapur, Nitin; Lipsett, Jill; Nogee, Lawrence M.; Phu, Amy; Suresh, Sadasivam; Schultz, André; Selvadurai, Hiran; Sherrard, Stephanie; Strachan, Roxanne; Vyas, Julian; Zurynski, Yvonne; Jaffé, Adam
- Abstract
<bold>Background: </bold>Childhood 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.<bold>Methods: </bold>Paediatric 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.<bold>Results: </bold>One 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.<bold>Conclusion: </bold>chILD 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.
- Subjects
AUSTRALIA; NEW Zealand; INTERSTITIAL lung diseases in children; INTERSTITIAL lung diseases; PEDIATRIC respiratory diseases; TREATMENT of respiratory diseases; DISEASE prevalence; THERAPEUTICS; ETHICS; IMMUNE response; IMMUNOCOMPETENT cells; RESEARCH funding; ACQUISITION of data
- Publication
Orphanet Journal of Rare Diseases, 2017, Vol 12, p1
- ISSN
1750-1172
- Publication type
journal article
- DOI
10.1186/s13023-017-0637-x