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

and the other chILD-EU collaborators, Matthias Griese, Elias Seidl, Meike Hengst, Simone Reu, Hans Rock, Gisela Anthony, Nural Kiper, Nagehan Emiralioğlu, Deborah Snijders, Lutz Goldbeck, Reiner Leidl, Julia Ley-Zaporozhan, Ingrid Krüger-Stollfuss, Birgit Kammer, Traudl Wesselak, Claudia Eismann, Andrea Schams, Doerthe Neuner, Morag MacLeanAndrew G Nicholson, McCann Lauren, Annick Clement, Ralph Epaud, Jacques de Blic, Michael Ashworth, Paul Aurora, Alistair Calder, Martin Wetzke, Matthias Kappler, Steve Cunningham, Nicolaus Schwerk, Andy Bush

Research output: Contribution to journalArticlepeer-review

Abstract

BACKGROUND: Children's interstitial lung diseases (chILD) cover many rare entities, frequently not diagnosed or studied in detail. There is a great need for specialised advice and for internationally agreed subclassification of entities collected in a register.Our objective was to implement an international management platform with independent multidisciplinary review of cases at presentation for long-term follow-up and to test if this would allow for more accurate diagnosis. Also, quality and reproducibility of a diagnostic subclassification system were assessed using a collection of 25 complex chILD cases.

METHODS: A web-based chILD management platform with a registry and biobank was successfully designed and implemented.

RESULTS: Over a 3-year period, 575 patients were included for observation spanning a wide spectrum of chILD. In 346 patients, multidisciplinary reviews were completed by teams at five international sites (Munich 51%, London 12%, Hannover 31%, Ankara 1% and Paris 5%). In 13%, the diagnosis reached by the referring team was not confirmed by peer review. Among these, the diagnosis initially given was wrong (27%), imprecise (50%) or significant information was added (23%).The ability of nine expert clinicians to subcategorise the final diagnosis into the chILD-EU register classification had an overall exact inter-rater agreement of 59% on first assessment and after training, 64%. Only 10% of the 'wrong' answers resulted in allocation to an incorrect category. Subcategorisation proved useful but training is needed for optimal implementation.

CONCLUSIONS: We have shown that chILD-EU has generated a platform to help the clinical assessment of chILD.

TRIAL REGISTRATION NUMBER: Results, NCT02852928.

Original languageEnglish
JournalThorax
Early online date22 Oct 2017
DOIs
Publication statusPublished - 3 Nov 2017

Keywords

  • Journal Article

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