Categorizing diffuse parenchymal lung disease in children
Open Access
- 25 September 2015
- journal article
- research article
- Published by Springer Science and Business Media LLC in Orphanet Journal of Rare Diseases
- Vol. 10 (1), 1-6
- https://doi.org/10.1186/s13023-015-0339-1
Abstract
Aim of this study was to verify a systematic and practical categorization system that allows dynamic classification of pediatric DPLD irrespective of completeness of patient data. The study was based on 2322 children submitted to the kids-lung-register between 1997 and 2012. Of these children 791 were assigned to 12 DPLD categories, more than 2/3 belonged to categories manifesting primarily in infancy. The work-flow of the pediatric DPLD categorization system included (i) the generation of a final working diagnosis, decision on the presence or absence of (ii) DPLD and (iii) a systemic or lung only condition, and (iv) the allocation to a category and subcategory. The validity and inter-observer dependency of this workflow was re-tested using a systematic sample of 100 cases. Two blinded raters allocated more than 80 % of the re-categorized cases identically. Non-identical allocation was due to lack of appreciation of all available details, insufficient knowledge of the classification rules by the raters, incomplete patient data, and shortcomings of the classification system itself. This study provides a suitable workflow and hand-on rules for the categorization of pediatric DPLD. Potential pitfalls were identified and a foundation was laid for the development of consensus-based, international categorization guidelines.Keywords
Funding Information
- Seventh Framework Programme (305653)
- Else Kröner-Fresenius-Stiftung (EKS 2013_A72)
This publication has 9 references indexed in Scilit:
- Childhood Interstitial Lung Diseases: An 18-year Retrospective AnalysisPediatrics, 2013
- An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in InfancyAmerican Journal of Respiratory and Critical Care Medicine, 2013
- Diffuse lung disease in infancy and childhood: expanding the chILD classificationHistopathology, 2013
- Incidence and classification of pediatric diffuse parenchymal lung diseases in GermanyOrphanet Journal of Rare Diseases, 2009
- Diffuse Lung Disease in Infancy: A Proposed Classification Applied to 259 Diagnostic BiopsiesPediatric and Developmental Pathology, 2009
- Diffuse Lung Disease in Young ChildrenAmerican Journal of Respiratory and Critical Care Medicine, 2007
- Task force on chronic interstitial lung disease in immunocompetent childrenEuropean Respiratory Journal, 2004
- Idiopathic interstitial pneumonitis in children: A national survey in the United Kingdom and IrelandPediatric Pulmonology, 2002
- The Diagnosis, Assessment and Treatment of Diffuse Parenchymal Lung Disease in AdultsThorax, 1999