Categorizing diffuse parenchymal lung disease in children
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RESEARCH
Open Access
Categorizing diffuse parenchymal lung disease in children Matthias Griese1*, Armin Irnstetter1, Meike Hengst1, Helen Burmester1, Felicitas Nagel1, Jan Ripper1, Maria Feilcke1, Ingo Pawlita1, Florian Gothe1, Matthias Kappler1, Andrea Schams1, Traudl Wesselak1, Daniela Rauch1, Thomas Wittmann1, Peter Lohse2, Frank Brasch3 and Carolin Kröner1
Abstract 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. Keywords: Childhood interstitial lung disease, chILD, Diffuse parenchymal lung disease, Rare pediatric lung disease, Categorization, Register, Registry
Background Childhood interstitial lung diseases (ILD) represent a large spectrum of individually rare diffuse parenchymal lung diseases (DPLD), prevalent in children of all ages [1–3]. They comprise more than 200 different disease entities which are treated by pediatricians and general practitioners in general and specialized (children´s) hospitals. Due to the similarity of symptoms it is often difficult to differentiate these rare patients from children with more common respiratory diseases [4]. Clinical presentation of the disease may further be blurred by recurrent infections or allergies. Childhood DPLD may thus easily be underdiagnosed. * Correspondence: [email protected] 1 Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany Full list of author information is available at the end of the article
Correct classification of all patients is however indispensable for the appropriate treatment, for a better understanding of the underlying pathophysiology, for the identification of biomarkers and for long-term stu
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