Categorizing diffuse parenchymal lung disease in children

Orphanet Journal of Rare Diseases

Carolin Kröner²  Frank Brasch¹  Peter Lohse³  Thomas Wittmann²  Daniela Rauch²  Traudl Wesselak²  Andrea Schams²  Matthias Kappler²  Florian Gothe²  Ingo Pawlita²  Maria Feilcke²  Jan Ripper²  Felicitas Nagel²  Helen Burmester²  Meike Hengst²  Armin Irnstetter²  Matthias Griese² 

[1] Department of Pathology, Academic Teaching Hospital Bielefeld, Bielefeld, Germany;Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, Munich, Germany;Praxis für Humangenetik, CeGaT GmbH, Tübingen, Germany

关键词: Registry;    Register;    Categorization;    Rare pediatric lung disease;    Diffuse parenchymal lung disease;    chILD;    Childhood interstitial lung disease;   

Others  :  1228438
DOI  :  10.1186/s13023-015-0339-1

 received in 2015-05-09, accepted in 2015-09-07,  发布年份 2015

【 摘 要 】

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.

【 授权许可】

   
2015 Griese et al.

【 图 表 】

Fig. 1.

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