Childhood interstitial lung disease (chILD) Respiratory Other specified interstitial pulmonary diseases
Conditions
Interventions
Observations will start from time of presentation at hospital during which the diagnosis is made and participants will continue in the trial for 12 months. Participants will be given the usual treatme
Sponsors
Academic and Clinical Centre Office for Research and Development (ACCORD) (UK)
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Infants and children presenting to hospital with clinician-suspected interstitial lung disease or at least three of the following four criteria present: 1. Respiratory symptoms for at least 14 days 1.1. Cough 1.2. Rapid and/or difficult breathing 1.3. Exercise intolerance 2. Respiratory signs 2.1. Tachypnea 2.2. Adventitious sounds 2.3. Retractions 2.4. Digital clubbing 2.5. Failure to thrive 2.6. Respiratory failure 3. Hypoxemia 4. Diffuse abnormalities on a chest radiograph or computerised tomography (CT) scan
Exclusion criteria
Exclusion criteria: A participant would be excluded from the database if ineligible to participate in the ChILD-EU Minimal Dataset observation and follow-up study. Exclusion criteria are common causes of diffuse lung disease, including but not exclusively: 1. Cystic fibrosis 2. Respiratory distress syndrome 3. Bronchopulmonary dysplasia 4. Acute infection (viral or bacterial) 5. Inherited or acquired immune deficiency
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 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) | — |
Secondary
| Measure | Time frame |
|---|---|
| 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) | — |
Countries
United Kingdom
Contacts
Public ContactMorag MacLean
Outcome results
None listed