International management platform for children's interstitial lung disease (chILD-EU). Issue 3 (22nd October 2017)

• Record Type: Journal Article
• 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;
• Other Names: author non-byline.; Eber E author non-byline.; Pfleger A author non-byline.; Basek P author non-byline.; Proesmans M author non-byline.; Silva filho Lv Ribeiro author non-byline.; Rochat I author non-byline.; Fontana M author non-byline.; Freihorst J author non-byline.; Brinkmann F author non-byline.; Koerner-rettberg C author non-byline.; Becker S author non-byline.; Stehling F author non-byline.; Heinzmann A author non-byline.; Kusserow S author non-byline.; Nährlich L author non-byline.; Lex C author non-byline.; Berthold D author non-byline.; Kaiser-labusch P author non-byline.; Hämmerling S author non-byline.; Ahrens F author non-byline.; Müller-stöver S author non-byline.; Tutdibi E author non-byline.; Seidemann K author non-byline.; Prenzel F author non-byline.; Reiter K author non-byline.; Feilcke M author non-byline.; Ripper J author non-byline.; Pawlita I author non-byline.; Fischer I author non-byline.; Forstner M author non-byline.; Weichert S author non-byline.; Schaible T author non-byline.; Kern I author non-byline.; Seidenberg J author non-byline.; Zeidler S author non-byline.; Baden W author non-byline.; Niemitz M author non-byline.; Gappa M author non-byline.; Liese J author non-byline.; Rubak S author non-byline.; Buchvald F author non-byline.; Almario P author non-byline.; Escribano A author non-byline.; Lopez J author non-byline.; Coulomb A author non-byline.; Le pointe H Ducou author non-byline.; Nathan N author non-byline.; Reiter J author non-byline.; Rossi G author non-byline.; Barbarto A author non-byline.; Cogo P author non-byline.; Terheggen-largo S author non-byline.; Glowacka E author non-byline.; Lange J author non-byline.; Katarzyna K author non-byline.; Cobanoglu E author non-byline.; Sismanlar T author non-byline.; Aslan At author non-byline.; Orhan D author non-byline.; Berna O author non-byline.; Cinel G author non-byline.; Yalçın E author non-byline.; Sair Fn author non-byline.; Bal Cm author non-byline.; Yuksel H author non-byline.; Turner S author non-byline.; Michael S author non-byline.; Kenia P author non-byline.; Hilliard T author non-byline.; Ross-russell R author non-byline.; Forton J author non-byline.; Redmond F author non-byline.; Gibson N author non-byline.; Wallis C author non-byline.; Pabry R author non-byline.; Owens C author non-byline.; Gupta A author non-byline.; Mayell S author non-byline.; Chetcuti P author non-byline.; Child F author non-byline.; Moss S author non-byline.; Bhatt J author non-byline.; Hull J author non-byline.; Hansel D author non-byline.; Nwokoro C author non-byline.; O'reilly R author non-byline.; Walker W author non-byline.; Schwarzkopf I author non-byline.; Seidl H author non-byline.; Szentes B author non-byline.; Brasch F author non-byline.; Gilbert C author non-byline.; Zampoli M author non-byline.; Kritzinger F author non-byline.; Rosewich M author non-byline.;
• Is Part Of: Thorax. Volume 73:Issue 3(2018)
• Page Start: 231
• Page End: 239
• Publication Date: 2017-10-22
• Subjects: paediatric interstitial lung disease -- rare lung diseases -- paediatric lung disaese; Chest -- Diseases -- Periodicals; Thorax; Chest -- Diseases; Periodicals; Periodicals; 617.54
• DOI: 10.1136/thoraxjnl-2017-210519
• Languages: English
• ISSNs: 0040-6376

Abstract

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 .