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

• Published in: Thorax Vol. 73; no. 3; pp. 231 - 239
• Main Authors: Griese, Matthias, Seidl, Elias, Hengst, Meike, Reu, Simone, Rock, Hans, Anthony, Gisela, Kiper, Nural, Emiralioğlu, Nagehan, Snijders, Deborah, Goldbeck, Lutz, Leidl, Reiner, Ley-Zaporozhan, Julia, Krüger-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
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 01.03.2018; BMJ Publishing Group
• Subjects: Adolescent; Adult; Child; Child, Preschool; Children & youth; Clinical trials; Cystic fibrosis; Ethics; Experts; Female; Human health and pathology; Humans; Infant; Life Sciences; Lung diseases; Lung Diseases, Interstitial - diagnosis; Male; Pediatrics; Pulmonology and respiratory tract; Registries; Reproducibility of Results; Teams; Thorax; Young Adult; United Kingdom > UK; Ireland; Europe; Germany; rare lung diseases; paediatric interstitial lung disease; paediatric lung disaese
• ISSN: 0040-6376; 1468-3296; 1468-3296
• DOI: 10.1136/thoraxjnl-2017-210519

BackgroundChildren’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.MethodsA web-based chILD management platform with a registry and biobank was successfully designed and implemented.ResultsOver 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.ConclusionsWe have shown that chILD-EU has generated a platform to help the clinical assessment of chILD.Trial registration numberResults, NCT02852928.