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...
| Main Authors: | , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2017-07-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13023-017-0637-x |
| _version_ | 1819013147754758144 |
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| author | Vishal Saddi Sean Beggs Bruce Bennetts Joanne Harrison Neil Hime Nitin Kapur Jill Lipsett Lawrence M. Nogee Amy Phu Sadasivam Suresh André Schultz Hiran Selvadurai Stephanie Sherrard Roxanne Strachan Julian Vyas Yvonne Zurynski Adam Jaffé |
| author_facet | Vishal Saddi Sean Beggs Bruce Bennetts Joanne Harrison Neil Hime Nitin Kapur Jill Lipsett Lawrence M. Nogee Amy Phu Sadasivam Suresh André Schultz Hiran Selvadurai Stephanie Sherrard Roxanne Strachan Julian Vyas Yvonne Zurynski Adam Jaffé |
| author_sort | Vishal Saddi |
| collection | DOAJ |
| description | 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 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. 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. |
| first_indexed | 2024-12-21T01:55:20Z |
| format | Article |
| id | doaj.art-e34dc34d927a4edab280cecf6991cecc |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-21T01:55:20Z |
| publishDate | 2017-07-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-e34dc34d927a4edab280cecf6991cecc2022-12-21T19:19:47ZengBMCOrphanet Journal of Rare Diseases1750-11722017-07-011211910.1186/s13023-017-0637-xChildhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade’s experienceVishal Saddi0Sean Beggs1Bruce Bennetts2Joanne Harrison3Neil Hime4Nitin Kapur5Jill Lipsett6Lawrence M. Nogee7Amy Phu8Sadasivam Suresh9André Schultz10Hiran Selvadurai11Stephanie Sherrard12Roxanne Strachan13Julian Vyas14Yvonne Zurynski15Adam Jaffé16Department of Respiratory Medicine, Discipline of Paediatrics, Sydney Children’s HospitalDepartment of Paediatrics, Royal Hobart HospitalDepartment of Molecular Genetics, The Children’s Hospital at WestmeadDepartment of Respiratory Medicine, The Children’s HospitalDiscipline of Paediatrics and Child Health, Sydney Medical School, The University of SydneyDepartment of Respiratory Medicine, Lady Cilento Children’s HospitalAnatomical Pathology, S.A. Pathology, Women’s and Children’s HospitalEudowood Neonatal Pulmonary Division, Department of Pediatrics, Johns Hopkins University School of MedicineDiscipline of Paediatrics and Child Health, Sydney Medical School, The University of SydneyDepartment of Respiratory Medicine, Lady Cilento Children’s HospitalDepartment of Respiratory Medicine, Princess Margaret Hospital for ChildrenDiscipline of Paediatrics and Child Health, Sydney Medical School, The University of SydneyDepartment of Respiratory Medicine, Princess Margaret Hospital for ChildrenDepartment of Respiratory Medicine, Discipline of Paediatrics, Sydney Children’s HospitalDepartment of Respiratory Paediatrics, Starship Children’s HospitalAustralian Paediatric Surveillance Unit, Kids Research InstituteSchool of Women’s and Children’s Health, Faculty of Medicine, University of New South WalesAbstract 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 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. 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.http://link.springer.com/article/10.1186/s13023-017-0637-xInterstitial lung diseasechILD syndromeAustraliaNew Zealand |
| spellingShingle | Vishal Saddi Sean Beggs Bruce Bennetts Joanne Harrison Neil Hime Nitin Kapur Jill Lipsett Lawrence M. Nogee Amy Phu Sadasivam Suresh André Schultz Hiran Selvadurai Stephanie Sherrard Roxanne Strachan Julian Vyas Yvonne Zurynski Adam Jaffé Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade’s experience Orphanet Journal of Rare Diseases Interstitial lung disease chILD syndrome Australia New Zealand |
| title | Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade’s experience |
| title_full | Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade’s experience |
| title_fullStr | Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade’s experience |
| title_full_unstemmed | Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade’s experience |
| title_short | Childhood interstitial lung diseases in immunocompetent children in Australia and New Zealand: a decade’s experience |
| title_sort | childhood interstitial lung diseases in immunocompetent children in australia and new zealand a decade s experience |
| topic | Interstitial lung disease chILD syndrome Australia New Zealand |
| url | http://link.springer.com/article/10.1186/s13023-017-0637-x |
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