International Management Platform for Children'S Interstitial Lung Disease (Child-Eu)
Date
2018Author
Griese, Matthias
Seidl, Elias
Hengst, Meike
Reu, Simone
Rock, Hans
Anthony, Gisela
Kiper, Nural
Emiralioglu, Nagehan
Snijders, Deborah
Goldbeck, Lutz
Leidl, Reiner
Ley-Zaporozhan, Julia
Krueger-Stollfuss, Ingrid
Kammer, Birgit
Wesselak, Traudl
Eismann, Claudia
Schams, Andrea
Neuner, Doerthe
MacLean, Morag
Nicholson, Andrew G.
Lauren, McCann
Clement, Annick
Epaud, Ralph
de Blic, Jacques
Ashworth, Michael
Aurora, Paul
Calder, Alistair
Wetzke, Martin
Kappler, Matthias
Cunningham, Steve
Schwerk, Nicolaus
Bush, Andy
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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.