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. 2015 Sep 25:10:122.
doi: 10.1186/s13023-015-0339-1.

Categorizing diffuse parenchymal lung disease in children

Matthias Griese  1 , Armin Irnstetter  2 , Meike Hengst  3 , Helen Burmester  4 , Felicitas Nagel  5 , Jan Ripper  6 , Maria Feilcke  7 , Ingo Pawlita  8 , Florian Gothe  9 , Matthias Kappler  10 , Andrea Schams  11 , Traudl Wesselak  12 , Daniela Rauch  13 , Thomas Wittmann  14 , Peter Lohse  15 , Frank Brasch  16 , Carolin Kröner  17
Affiliations

Categorizing diffuse parenchymal lung disease in children

Matthias Griese et al. Orphanet J Rare Dis. .

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.

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Figures

Fig. 1
Fig. 1
Overview on study design. The upper part (black) of the figure shows the patients collected in the kids lung register (KLR) and categorized according to the KLR algorithm between 1997 and 2012. Patients received a working diagnosis and were categorized into DPLD categories and subcategories; the latter process is described in more detail in Addditional file 2: Figure S1. The lower part of the figure (red) describes the workflow used for the re-categorization of 100 cases selected randomly and in proportion of their occurrence in the KLR. Two reviewers (AI, MG) re-assessed those cases blinded and independently and obtained a working diagnosis, categorization and sub-categorization according to the workflow in the lower part of Addditional file 2: Figure S1 (red)
See this image and copyright information in PMC

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