title: Categorizing diffuse parenchymal lung disease in children.
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.
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. matthias.griese@med.uni-muenchen.de.
authors.forename: Matthias
authors.initials: M
authors.lastname: Griese
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Armin.Irnstetter@med.uni-muenchen.de.
authors.forename: Armin
authors.initials: A
authors.lastname: Irnstetter
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Meike.Hengst@med.uni-muenchen.de.
authors.forename: Meike
authors.initials: M
authors.lastname: Hengst
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Helen.Burmester@med.uni-muenchen.de.
authors.forename: Helen
authors.initials: H
authors.lastname: Burmester
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Felicitas.Nagel@med.uni-muenchen.de.
authors.forename: Felicitas
authors.initials: F
authors.lastname: Nagel
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Jan.Ripper@med.uni-muenchen.de.
authors.forename: Jan
authors.initials: J
authors.lastname: Ripper
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Maria.Feilcke@med.uni-muenchen.de.
authors.forename: Maria
authors.initials: M
authors.lastname: Feilcke
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Ingo.Pawlita@med.uni-muenchen.de.
authors.forename: Ingo
authors.initials: I
authors.lastname: Pawlita
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Florian.Gothe@med.uni-muenchen.de.
authors.forename: Florian
authors.initials: F
authors.lastname: Gothe
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Matthias.Kappler@med.uni-muenchen.de.
authors.forename: Matthias
authors.initials: M
authors.lastname: Kappler
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Andrea.Schams@med.uni-muenchen.de.
authors.forename: Andrea
authors.initials: A
authors.lastname: Schams
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Traudl.Wesselak@med.uni-muenchen.de.
authors.forename: Traudl
authors.initials: T
authors.lastname: Wesselak
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Daniela.Rauch@med.uni-muenchen.de.
authors.forename: Daniela
authors.initials: D
authors.lastname: Rauch
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Thomas.Wittmann@med.uni-muenchen.de.
authors.forename: Thomas
authors.initials: T
authors.lastname: Wittmann
authors.affiliation: Praxis für Humangenetik, CeGaT GmbH, Tübingen, Germany. Peter.lohse@cegat.de.
authors.forename: Peter
authors.initials: P
authors.lastname: Lohse
authors.affiliation: Department of Pathology, Academic Teaching Hospital Bielefeld, Bielefeld, Germany. Frank.Brasch@klinikumbielefeld.de.
authors.forename: Frank
authors.initials: F
authors.lastname: Brasch
authors.affiliation: Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstraße 4, 80337, Munich, Germany. Carolin.Kroener@med.uni-muenchen.de.
authors.forename: Carolin
authors.initials: C
authors.lastname: Kröner
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D017563:Lung Diseases, Interstitial / Q000145:classification* / Q000175:diagnosis*; D008297:Male; D016037:Single-Blind Method; D055815:Young Adult