International management platform for children's interstitial lung disease (chILD-EU)

Matthias Griese1, Elias Seidl1, Meike Hengst1

  • 1Department of Pediatric Pneumology, Dr von Hauner Children's Hospital, Ludwig-Maximilians-University, German Center for Lung Research, Munich, Germany.

Thorax
|October 24, 2017
PubMed

Insights

An international platform for children's interstitial lung diseases (chILD) improved diagnosis accuracy. Multidisciplinary review confirmed diagnoses in many cases, highlighting the need for standardized subclassification training.

Area of Science:

  • Pediatric Pulmonology
  • Rare Diseases
  • Medical Informatics

Background:

  • Children's interstitial lung diseases (chILD) encompass rare, often undiagnosed conditions.
  • There is a critical need for expert advice and standardized classification of chILD entities.
  • An international registry is essential for comprehensive chILD research and management.

Purpose of the Study:

  • To implement an international platform for chILD management and multidisciplinary case review.
  • To assess the platform's ability to improve diagnostic accuracy in chILD.
  • To evaluate the quality and reproducibility of a diagnostic subclassification system for chILD.

Main Methods:

  • Development and implementation of a web-based chILD management platform with registry and biobank.
  • Inclusion of 575 patients over 3 years, with multidisciplinary reviews conducted at five international sites.
  • Assessment of diagnostic subclassification accuracy by nine expert clinicians using the chILD-EU classification.

Main Results:

  • The platform facilitated the inclusion of 575 chILD patients, with 346 undergoing multidisciplinary review.
  • Peer review led to diagnostic confirmation or refinement in 13% of cases initially diagnosed by referring teams.
  • Expert clinicians achieved 64% inter-rater agreement in subcategorization after training, indicating usefulness but need for further standardization.

Conclusions:

  • The chILD-EU platform provides a valuable tool for enhancing the clinical assessment of children's interstitial lung diseases.
  • International collaboration and standardized review processes are crucial for accurate chILD diagnosis and management.
  • Further training is recommended to optimize the implementation and consistency of chILD subclassification.
Abstract

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