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Thorax. 2018 Mar;73(3):231-239. doi: 10.1136/thoraxjnl-2017-210519. Epub 2017 Oct 22.

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

Author information

1
Department of Pediatric Pneumology, Dr von Hauner Children's Hospital, Ludwig-Maximilians-University, German Center for Lung Research, Munich, Germany.
2
Department of Pathology, LMU Munich, Munich, Germany.
3
Department of Neurology, University of Marburg, Central Information Office, Marburg, Germany.
4
Division of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
5
Department of Pediatrics, University of Padova, Padova, Italy.
6
Child and Adolescent Psychiatry, University of Ulm, Clinic for Child and Adolescent Psychiatry/Psychotherapy, Ulm, Germany.
7
Helmholtz Zentrum München, Deutsches Forschungszentrum für Gesundheit und Umwelt, Munich, Germany.
8
Department of Pediatric Radiology, Dr von Hauner Children's Hospital, Ludwig-Maximilians-University, Munich, Germany.
9
Department of Respiratory and Sleep Medicine, Royal Hospital for Sick Children, Edinburgh, UK.
10
Department of Paediatrics, Royal Brompton Hospital, London, UK.
11
Department of Paediatrics, Imperial College London, NHLI, London, UK.
12
Department of Paediatrics, University Pierre und Marie Curie, Paris, France.
13
Department of Paediatrics, Great Ormond Street Hospital For Children NHS Trust, London, UK.
14
Department of Pediatric Pulmonology, Hannover Medical School, Hannover, Germany.
#
Contributed equally

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

PMID:
29056600
DOI:
10.1136/thoraxjnl-2017-210519
[Indexed for MEDLINE]
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