Author:
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
Abstract
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.
Subject
Pulmonary and Respiratory Medicine
Cited by
63 articles.
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