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Griese, Matthias; Irnstetter, Armin; Hengst, Meike; Burmester, Helen; Nagel, Felicitas; Ripper, Jan; Feilcke, Maria; Pawlita, Ingo; Gothe, Florian; Kappler, Matthias; Schams, Andrea; Wesselak, Traudl; Rauch, Daniela; Wittmann, Thomas; Lohse, Peter; Brasch, Frank; Kröner, Carolin (2015): Categorizing diffuse parenchymal lung disease in children. In: Orphanet Journal of Rare Diseases 10:122


Background 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. Methods 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. Results 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. Conclusions 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.