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.
- paediatric interstitial lung disease
- rare lung diseases
- paediatric lung disaese
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SC, NS and AB contributed equally.
Contributors MG designed the project, organised and developed the platform with register and biobank, reviewed the cases and performed calculations and data interpretation. He drafted the manuscript under the corrective action of AB. All authors reviewed the manuscript and agreed with the contents. HR and GA set up and programmed the web-based system. Clinical principal site investigators of the project were NK, DS, LG, RL, AC, RE, JDB, SC, NS, AB, MG. Major contributions for the Pediatric radiology group came from JL-Z, IK-S, BK and AC. Major contributions for the Pediatric pathology group came from SR, AM and AGN. TW, CE, AS, DN, MM and MCL were key clinical trial management staff. Major additional clinical peer-review came from ES, MH, NE, PA, MW and MK. ES and MH were central register physicians. All authors participated in discussions for the development and conclusion of the project. All contributors listed supplied cases and participated in the cases-related discussions.
Funding The research leading to these results has received funding from the European Union’s Seventh Framework Program under grant agreement n°305653-chILD-EU. Funding of the project started in December 2012 and ended in November 2016; the project is continuous under ClinicalTrials.gov identifier: NCT02852928.
Competing interests None declared.
Patient consent Obtained.
Ethics approval Ethics Committee of the University of Munich.
Provenance and peer review Not commissioned; externally peer reviewed.
Collaborators chILD-EU collaborators are: E Eber (Universitätsklinikfür Kinder- und Jugendheilkunde, Graz, Austria), A Pfleger (Universitätsklinik für Kinder- und Jugendheilkunde, Graz, Austria), P Basek (Kinderspital Salzburg, Austria), M Proesmans (UZ Leuven, Belgium), L V Ribeiro Silva Filho (Emergency Instituto daCriança do Hospital das Clínicas da FMUSP, Sao Paulo, Brasil), I Rochat (Centre hospitalier universitaire vaudois, Lausanne, Switzerland), M Fontana (Kinderspital Luzern, Switzerland), J Freihorst (Ostalb Klinikum, Aalen, Germany), F Brinkmann (RuhrUniversity-Bochum, Germany), C Koerner-Rettberg (RuhrUniversity-Bochum, Germany), S Becker (Darmstädter Kinderklinik Prinzessin Margaret, Darmstadt, Germany), F Stehling (University of Essen, Germany), A Heinzmann (Universitätsklinik Freiburg, Germany), S Kusserow (Marienhospital, Gelsenkirchen, Germany), L Nährlich (Universitätsklinikum Gießen und Marburg, Giessen,Germany), C Lex (Universitätsmedizin Göttingen, Germany), D Berthold (Medizinische Hochschule Hannover Zentrum für Kinderheilkunde und Jugendmedizin, Hannover, Germany), P Kaiser-Labusch (Gesundheit Nord Klinikverbund Bremen, Germany), S Hämmerling (Universitätsklinikum Heidelberg, Germany), F Ahrens (Altonaer Kinderkrankenhaus, Hamburg, Germany), S Müller-Stöver (Altonaer Kinderkrankenhaus, Hamburg, Germany), E Tutdibi (Universitätsklinikum des Saarlandes, Homburg, Germany), K Seidemann (Städtisches Klinikum Karlsruhe, Germany), F Prenzel (Klinik und Poliklinik für Kinder- und Jugendmedizin Universitätsklinikum Leipzig, Germany), K Reiter (Dr von Haunersches Kinderspital, München, Germany), M Feilcke (Dr von Haunersches Kinderspital, München, Germany), J Ripper (Dr von Haunersches Kinderspital, München, Germany), I Pawlita (Dr von Haunersches Kinderspital, München, Germany), I Fischer (Dr von Haunersches Kinderspital, München, Germany), S Witt (Helmholtz Zentrum München, Institute of Health Economics and Health Care Management, Neuherberg, Germany), L Schwarzkopf (Helmholtz Zentrum München, Institute of Health Economics and Health Care Management, Neuherberg, Germany), B L Szentes (Helmholtz Zentrum München, Institute of Health Economics and Health Care Management, Neuherberg, Germany), S Weichert (UMM Universitätsmed. Mannheim Klinik für Kinder- und Jugendmedizin, Mannheim, Germany), T Schaible (UMM Universitätsmedizin Mannheim Klinik für Kinder- undJugendmedizin, Mannheim, Germany), I Kern (Elisabeth-Krankenhaus Rheydt, Mönchengladbach, Germany), J Seidenberg (Zentrum fürKinder- und Jugendmedizin, Oldenburg, Germany), S Zeidler (Asklepios Kinderklinik, Sankt Augustin, Germany), W Baden (Universitätsklinik für Kinder- und Jugendmedizin Tübingen, Germany), M Niemitz (Universitätsklinikum Ulm Kinder- undJugendpsychiatrie, Ulm, Germany), M Gappa (Zentrum für Kinder undJugendliche—Marien-Hospital Wesel, Germany), J Liese (Universitätsklinikum Würzburg—Kinderklinik, Würzburg, Germany), S Rubak (Aarhus University, Denmark), F Buchvald (University ofCopenhagen, Denmark), P Almario (Hospital Universitario Barcelona,Spain), A Escribano (SEPAR Sociedad Espanyola de Neumologia yCirurgia Toràcica, Valencia, Spain), J Lopez (Escuela Universitariade Enfermería La Fe, Valencia, Spain), A Coulomb (CHU Paris-EstHôpital d’Enfants Armand Trousseau, Paris, France), H Ducou LePointe (CHU Paris-Est Hôpital d’Enfants Armand Trousseau, Paris, France), N Nathan (CHU Paris-Est Hôpitald’Enfants Armand Trousseau, Paris, France), J Reiter (Hadassah-Hebrew University, Jerusalem, Israel), G Rossi (Giannina Gaslini, Genova, Italy), A Barbarto (University of Padova, Padova, Italy), P Cogo (Ospedale Pediatrico Bambino Gesù, Roma, Italy), S Terheggen-Largo (University Medical Center Utrecht, Netherlands), E Glowacka (Children’s University Hospital, Krakow, Hungary), J Lange (Medical University of Warsaw, Poland), K Katarzyna (Medical University of Warsaw, Poland), N Cobanoglu (Ankara University, Ankara, Turkey), T Sismanlar (Gazi University Hospital, Ankara, Turkey), A T Aslan (Gazi University Hospital, Ankara, Turkey), D Orhan (Hacettepe University, Ankara, Turkey), O Berna (Hacettepe University, Ankara, Turkey), G Cinel (Hacettepe University, Ankara, Turkey), E Yalçın (Hacettepe University, Ankara, Turkey), F N Sair (Zekai Technical Hospital, Ankara, Turkey), C M Bal (Ege Üniversitesi, Izmir, Turkey), H Yuksel (Manisa Celal Bayar Üniversitesi, Turkey), S Turner (Royal Aberdeen Children’s Hospital, UK), S Michael (Queen’s University, Belfast,United Kingdom), P Kenia (Children’s Hospital, Birmingham, UK), T Hilliard (Royal Hopital for Children, Bristol, UK), R Ross-Russell (Addenbrookes, Cambridge, UK), J Forton (University of Wales, Cardiff, Wales), F Redmond (Royal Hospital for Sick Children, Edinburgh, UK), N Gibson (Royal Hospital for Children, Glasgow, UK), C Wallis (Great Ormond Street Hospital, London, UK), R Pabry (Great Ormond Street Hospital, London, UK), C Owens (Great Ormond Street Hospital, London, United Kingdom), A Gupta (King’s Colleage, London, UK), S Mayell (Royal Children’s Hospital, Liverpool, UK), P Chetcuti (Leeds General Infirmary Children’s Hospital, Leeds, UK), F Child (Royal Children’s Hospital, Manchester, UK), S Moss (Royal Victoria Infirmary, Newcastle, UK), J Bhatt (Nottingham Children’s Hospital, Nottingham, United Kingdom), J Hull (John Radcliffe Hospital, Oxford, UK), D Hansel (Royal Brompton Hospital, London, UK), C Nwokoro (Whitechapel Royal London Hospital, London, UK), R O’Reilly (Sheffield Children’s Hospital, UK), W Walker (General Hospital, Southampton, UK), H Seidl (Helmholzzentrum, Munich, Germany), F Brasch (Institute for Pathology, Klinikum Bielefeld, Germany), C Gilbert (UK-chILD-foundation), M Zampoli (Red Cross Children’s Hospital, Kapstadt, South Africa), F Kritzinger (Netcare Christiaan Barnard Memorial Hospital, Kapstadt, South Africa), M Rosewich (Olgahospital, Stuttgart, Germany).
Correction notice This article has been corrected since it was published Online First. Author note was included in error, so it has now been removed. Collaborator, N Cobanoglu’s name was incorrect and is now corrected.
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