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

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.

Exogenous lipoid pneumonia in children: A systematic review

“…Diagnostic certainty levels have been proposed for randomized control trials in other conditions in children such as wheezing, where standardized definitions are lacking [51]. Additionally, standardized reporting could be adapted from international registries such as the international management platform for ChILD [52] or other global registries. Preferably data collection should be prospective to prevent pitfalls of bias and missing data.…”

Health‐related quality of life in infants and children with interstitial lung disease

“…With the collaboration of patient associations, efforts need to be pursued in creating and validating new specific chILD QoL questionnaire that could reach high correlations in all the dimensions with validated pediatric questionnaires. A dedicated working group of the European Network for Translational research in children and adult ILD (ENTeR‐chILD) (European Cooperation in Science and Technology (COST) action CA16125) has recently been dedicated to these crucial issues . Already, regarding the patient's information on the disease, a booklet for the chILD has been set up in English and is now available in five other languages (http://www.klinikum.uni-muenchen.de/Child-EU/en/child-eu-register/services/booklet/index.html).…”

“…Other chILD can be related to various aetiologies such as environmental or toxic exposure‐related ILD (hypersensitivity pneumonitis, medication), autoimmune diseases (connective tissue disease, or pulmonary vasculitis), metabolic disorders, and developmental disorders . However, depending on the recruitment strategy, 8% to 27% of chILD remain of unknown etiology . Nearly 16% of cases appear to be familiar .…”

Chronic interstitial lung disease in children

“…Engagement in registries for single diseases such as idiopathic pulmonary fibrosis or cystic fibrosis is superior, probably related to the availability of research coordinators entering data and shipping biomaterials [15]. The workload to enter a case is ∼5 h, and includes obtaining consent, collection of clinical data, burning of radiology imagines onto compact discs, collecting pathology specimens and biomaterials, shipping to a central site and writing a case description [16].…”

“…As the main purpose of this system is greatest possible grouping of similar diseases to build cohorts, constant refinement in subcategories is in progress. The system is widely used by the chILD-EU management platform (www.childeu.net) and the reliability and precision of categorisation has been assessed [14,16]. The publications reported in this review are ordered according to this classification system.…”