Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade's experience

Vishal Saddi, Sean Beggs, Bruce Bennetts, Joanne Harrison, Neil Hime, Nitin Kapur, Jill Lipsett, Lawrence M. Nogee, Amy Phu, Sadasivam Suresh, Andre Schultz, Hiran Selvadurai, Stephanie Sherrard, Roxanne Strachan, Julian Vyas, Yvonne Zurynski, Adam Jaffe

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Background: 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.

Methods: 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.

Results: 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 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.

Conclusion: 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.

Original languageEnglish
Article number133
Number of pages9
JournalOrphanet Journal of Rare Diseases
Volume12
DOIs
Publication statusPublished - 25 Jul 2017

Cite this

Saddi, Vishal ; Beggs, Sean ; Bennetts, Bruce ; Harrison, Joanne ; Hime, Neil ; Kapur, Nitin ; Lipsett, Jill ; Nogee, Lawrence M. ; Phu, Amy ; Suresh, Sadasivam ; Schultz, Andre ; Selvadurai, Hiran ; Sherrard, Stephanie ; Strachan, Roxanne ; Vyas, Julian ; Zurynski, Yvonne ; Jaffe, Adam. / Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand : a decade's experience. In: Orphanet Journal of Rare Diseases. 2017 ; Vol. 12.
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title = "Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade's experience",
abstract = "Background: 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.Methods: 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.Results: 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 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.Conclusion: 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.",
keywords = "Interstitial lung disease, chlLD syndrome, Australia, New Zealand, HYPERSENSITIVITY PNEUMONITIS, YOUNG-CHILDREN, CLASSIFICATION, DIAGNOSIS, DEFICIENCY, INFANTS",
author = "Vishal Saddi and Sean Beggs and Bruce Bennetts and Joanne Harrison and Neil Hime and Nitin Kapur and Jill Lipsett and Nogee, {Lawrence M.} and Amy Phu and Sadasivam Suresh and Andre Schultz and Hiran Selvadurai and Stephanie Sherrard and Roxanne Strachan and Julian Vyas and Yvonne Zurynski and Adam Jaffe",
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Saddi, V, Beggs, S, Bennetts, B, Harrison, J, Hime, N, Kapur, N, Lipsett, J, Nogee, LM, Phu, A, Suresh, S, Schultz, A, Selvadurai, H, Sherrard, S, Strachan, R, Vyas, J, Zurynski, Y & Jaffe, A 2017, 'Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade's experience' Orphanet Journal of Rare Diseases, vol. 12, 133. https://doi.org/10.1186/s13023-017-0637-x

Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand : a decade's experience. / Saddi, Vishal; Beggs, Sean; Bennetts, Bruce; Harrison, Joanne; Hime, Neil; Kapur, Nitin; Lipsett, Jill; Nogee, Lawrence M.; Phu, Amy; Suresh, Sadasivam; Schultz, Andre; Selvadurai, Hiran; Sherrard, Stephanie; Strachan, Roxanne; Vyas, Julian; Zurynski, Yvonne; Jaffe, Adam.

In: Orphanet Journal of Rare Diseases, Vol. 12, 133, 25.07.2017.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand

T2 - a decade's experience

AU - Saddi, Vishal

AU - Beggs, Sean

AU - Bennetts, Bruce

AU - Harrison, Joanne

AU - Hime, Neil

AU - Kapur, Nitin

AU - Lipsett, Jill

AU - Nogee, Lawrence M.

AU - Phu, Amy

AU - Suresh, Sadasivam

AU - Schultz, Andre

AU - Selvadurai, Hiran

AU - Sherrard, Stephanie

AU - Strachan, Roxanne

AU - Vyas, Julian

AU - Zurynski, Yvonne

AU - Jaffe, Adam

PY - 2017/7/25

Y1 - 2017/7/25

N2 - Background: 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.Methods: 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.Results: 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 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.Conclusion: 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.

AB - Background: 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.Methods: 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.Results: 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 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.Conclusion: 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.

KW - Interstitial lung disease

KW - chlLD syndrome

KW - Australia

KW - New Zealand

KW - HYPERSENSITIVITY PNEUMONITIS

KW - YOUNG-CHILDREN

KW - CLASSIFICATION

KW - DIAGNOSIS

KW - DEFICIENCY

KW - INFANTS

U2 - 10.1186/s13023-017-0637-x

DO - 10.1186/s13023-017-0637-x

M3 - Article

VL - 12

JO - Orphanet Journal of Rare Diseases

JF - Orphanet Journal of Rare Diseases

SN - 1750-1172

M1 - 133

ER -