International management platform for children's interstitial lung disease (chILD-EU)
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 MacLean, Andrew 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, the other chILD-EU collaborators, E Eber, A Pfleger, P Basek, M Proesmans, Lv Ribeiro Silva Filho, I Rochat, M Fontana, J Freihorst, F Brinkmann, C Koerner-Rettberg, S Becker, F Stehling, A Heinzmann, S Kusserow, L Nährlich, C Lex, D Berthold, P Kaiser-Labusch, S Hämmerling, F Ahrens, S Müller-Stöver, E Tutdibi, K Seidemann, F Prenzel, K Reiter, M Feilcke, J Ripper, I Pawlita, I Fischer, M Forstner, S Weichert, T Schaible, I Kern, J Seidenberg, S Zeidler, W Baden, M Niemitz, M Gappa, J Liese, S Rubak, F Buchvald, P Almario, A Escribano, J Lopez, A Coulomb, H Ducou Le Pointe, N Nathan, J Reiter, G Rossi, A Barbarto, P Cogo, S Terheggen-Largo, E Glowacka, J Lange, K Katarzyna, N Cobanoglu, T Sismanlar, At Aslan, D Orhan, O Berna, G Cinel, E Yalçın, Fn Sair, Cm Bal, H Yuksel, S Turner, S Michael, P Kenia, T Hilliard, R Ross-Russell, J Forton, F Redmond, N Gibson, C Wallis, R Pabry, C Owens, A Gupta, S Mayell, P Chetcuti, F Child, S Moss, J Bhatt, J Hull, D Hansel, C Nwokoro, R O'reilly, W Walker, I Schwarzkopf, H Seidl, B Szentes, F Brasch, C Gilbert, M Zampoli, F Kritzinger, M Rosewich, 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 MacLean, Andrew 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, the other chILD-EU collaborators, E Eber, A Pfleger, P Basek, M Proesmans, Lv Ribeiro Silva Filho, I Rochat, M Fontana, J Freihorst, F Brinkmann, C Koerner-Rettberg, S Becker, F Stehling, A Heinzmann, S Kusserow, L Nährlich, C Lex, D Berthold, P Kaiser-Labusch, S Hämmerling, F Ahrens, S Müller-Stöver, E Tutdibi, K Seidemann, F Prenzel, K Reiter, M Feilcke, J Ripper, I Pawlita, I Fischer, M Forstner, S Weichert, T Schaible, I Kern, J Seidenberg, S Zeidler, W Baden, M Niemitz, M Gappa, J Liese, S Rubak, F Buchvald, P Almario, A Escribano, J Lopez, A Coulomb, H Ducou Le Pointe, N Nathan, J Reiter, G Rossi, A Barbarto, P Cogo, S Terheggen-Largo, E Glowacka, J Lange, K Katarzyna, N Cobanoglu, T Sismanlar, At Aslan, D Orhan, O Berna, G Cinel, E Yalçın, Fn Sair, Cm Bal, H Yuksel, S Turner, S Michael, P Kenia, T Hilliard, R Ross-Russell, J Forton, F Redmond, N Gibson, C Wallis, R Pabry, C Owens, A Gupta, S Mayell, P Chetcuti, F Child, S Moss, J Bhatt, J Hull, D Hansel, C Nwokoro, R O'reilly, W Walker, I Schwarzkopf, H Seidl, B Szentes, F Brasch, C Gilbert, M Zampoli, F Kritzinger, M Rosewich
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.
Keywords: paediatric interstitial lung disease; paediatric lung disaese; rare lung diseases.
Conflict of interest statement
Competing interests: None declared.
© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
Source: PubMed