European Management Platform for Childhood Interstitial Lung Diseases - chILD-EU Register and Biobank

Objective 1: Generation of a common European database and biobank. The existing national programmes to collect data on chILD in three countries (France, Germany, UK) will enable the consortium to swiftly adapt current frameworks to a functionally appropriate pan-European web-based database and biobank. Importantly, compatibility with ongoing United States chILD data base developments will be factored in. Objective 2: Continuous assessment and implementation of guidelines and treatment protocols. Our Standards Working Group will convene regularly. Initial tasks will establish (a) specific diagnostic pathways, including detailed protocols for gathering clinical information, blood testing, imaging and pathology ("Best Practice Checklist"); (b) international panels of clinicians, geneticists, radiologists and pathologists who will review every diagnosis to quality control the data; and (c) detailed protocols for follow up?follow up The period after treatment or enrolment during which participants are observed or contacted, for example to record side effects or outcomes. to generate natural history?natural history A study that follows how a condition changes over time without assigning an experimental treatment. It may involve study visits and tests, and is often used to plan future trials. data. Objective 3: Recruitment of a carefully characterized cohort?cohort A group of people who share a characteristic, such as a diagnosis, and are studied together. 'Prospective' means they are followed forward from enrolment; 'retrospective' means existing records are reviewed. of chILD patients. European wide recruitment and interdisciplinary critical peer review of all diagnoses submitted from across Europe is imperative. Each case will be given a diagnosis independently; if no firm diagnosis is possible, we will review the case periodically as new information becomes available. During the first year of the study, clinicians´ decisions according to local practice and outcomes will be independently monitored and assessed. Objective 4: Determine the value of outcomes used in chILD. We will systematically optimize and clarify the relative weight of a large spectrum of single and composite clinical outcomes (using both clinician and carer scoring), sequential limited chest CT?CT An X-ray scan that builds detailed cross-section images of the body. 'High-resolution' CT (HRCT) is used to look closely at the lungs. (to minimise radiation exposure), lung function testing, histopathological categorization of lung biopsies, serum markers and genetic tests. Variability, reproducibility and the effects of training on reading images will be investigated. Objective 5: Assess treatment variations used, deliver data from defined protocols and linked outcomes. This project will analyse in detail treatment and outcomes within and between subjects using data collected. Analysis of the collected data will enable us to support the definition of trial protocols planned in the future.