ChILD-EU database and observational study

For the database and biobank study - collate detailed information on clinical cases of possible ChILD on a central database and biobank.

For the observational study - describe outcomes at 1, 2, 3, 6 and12 months in infants and children with ChILD.
Outcomes measured will be:
1. Death
2. Survival on artificial ventilatory support (invasive or non-invasive)
3. Survival in supplemental oxygen
4. Survival breathing room air
5. Quality of life (QoL)

For the database and biobank study:
1. To review each case by an experienced international interdisciplinary peer review team to provide diagnostic oversight and feedback
2. To provide annual updates of diagnosis and outcome in a feedback loop via peer review
3. To store for future research, blood samples for genetic analysis of cases and parents
4. To support paediatricians and families caring for children with ChILD

For the observational study:
To describe variance in outcome at 1, 2, 3, 6 and 12 months in infants and children with ChILD according to:
1. Diagnosis and presentation
1.1. Diagnosis (peer review)
1.2. Diagnostic certainty (peer review)
1.3. Computed tomography (CT) score – by component radiologist (peer review)
1.4. Blood oxygen saturation (SpO2) at rest in room air at presentation
1.5. SpO2 asleep in room air at presentation (nadir)
1.6. Respiratory rate (RR) (z score) at rest in air at presentation
1.7. Heart rate (HR) (z score) in air at presentation
1.8. Blood pressure at rest for 5 minutes at presentation
1.9. Weight (z-score) at presentation
1.10. Leland Fan 5 point severity score (nil, symptoms, SpO2 <90% air asleep, SpO2 at rest,
pulmonary hypertension).
2. Time to treatment and improvement
2.1. Time from onset of symptoms/signs of ChILD to first treatment
2.2. Time from onset of symptoms/signs of ChILD to diagnosis (local clinical)
2.3. Time from onset of symptoms/signs of ChILD to normoxia whilst awake (SpO2 ≥94% breathing room air at rest)
2.4. Time from onset of symptoms/signs of ChILD to respiratory rate in normal range for
age (Fleming, Thompson et al. 2011)
2.5. Time from onset of first treatment to reduction in RR by 10%
2.6. Time from onset of first treatment to reduction in HR by 20%
2.7. Time from onset of symptoms/signs of ChILD to normoxia whilst asleep (SpO2 ≥94% breathing room air at rest)
2.8. Time from onset of symptoms/signs of ChILD to weight appropriate for age/height without use of calorie supplementation
2.9. Time from onset of treatment to improvement in weight by 10%
3. Treatments
3.1. Steroids: use of steroids, dose, route and frequency of steroid use, time from first presentation to initiation of steroids, number of concomitant ChILD treatments at time of starting steroids
3.2. Hydroxychloroquine: use of hydroxychloroquine, dose and frequency of hydroxychloroquine, time from first presentation to initiation of hydroxychloroquine, number of concomitant ChILD treatments at time of starting hydroxychloroquine
3.3. Azithromycin: use of azithromycin, dose and frequency of azithromycin, time from first presentation to initiation of azithromycin, number of concomitant ChILD treatments at time of starting azithromycin
4. Concomitant medicines
5. Follow-up review
5.1. SpO2 in room air measured 4 weeks after commencing initial treatment
5.2. RR at rest measured 4 weeks after commencing initial treatment
5.3. Heart rate at rest measured 4 weeks after commencing initial treatment
6. Quality of Life score - PEDS QL Generic Core Scales at 0 and 12 months
7. Questionnaire for health care utilisation and costs
7.1. Utilisation of inpatient and outpatient care to calculate direct costs gathered at 0, 3, 6 and 12 months
7.2. Loss of productivity of parents and children to calculate indirect costs gathered at 0, 3, 6 and 12 months