CF Cystic fibrosis
Conditions
Interventions
None listed
Sponsors
Erasmus MC, Universitair Medisch Centrum Rotterdam
Eligibility
Age
2 Years to 11 Years
Inclusion criteria
Inclusion criteria: Subjects are eligible for this study when: - They are diagnosed with CF, confirmed with 2 mutations found by genetic analysis, either from heel prick screening or diagnosed later in life. - Aged 1, 3 or 5 years, when they will undergo bronchoscopy and chest CT scan as part of the routine monitoring program for CF. - Authorized by an informed consent from parents to undergo a possible extra rinse during bronchoscopy to retrieve sufficient BALF, an extra blood sample of 2 ml during the routine venous puncture, and permission to use excess biomaterials and coded clinical data for research.
Exclusion criteria
Exclusion criteria: Absence of previously given informed consent for use of encoded clinical data for scientific purposes
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1. Correlation of lipid profiles with early lung disease in CF: Lipidomics endpoints. The primary end-points are the different bioactive lipid levels in BALF of infants with CF using liquid chromatography (LC) coupled to Mass spectrometry (MS). Lipid profiles will be derived of the BALF supernatant 2. Association early lung disease in CF with airway neutrophils reprogramming: PMN endpoints. The primary endpoint for BALF cells flow cytometry analysis is surface CD63 on airway PMNs (exocytosis of NE-rich granules), which was shown to correlate with lung function in chronic CF disease | — |
Secondary
| Measure | Time frame |
|---|---|
| Other study parameters: We will assess various correlations, between: * Bioactive lipid levels and chest CT score * Bioactive lipid levels and past exacerbations / past antibiotic courses * Bioactive lipid levels and future (after moment of samples) exacerbations / future antibiotic courses (Bioactive lipid levels as riskfactors for later problems) * Bioactive lipid levels and microbiome (microorganisms from BALF / nasopharynx) * PMN endpoints and chest CT score * PMN endpoints and past exacerbations / past antibiotic courses * PMN endpoints and future exacerbations / future antibiotic courses (PMN endpoints as risk factors for later problems) * PMN endpoints and microbiome * PMN endpoints and bioactive lipid levels | — |
Countries
Netherlands
Outcome results
None listed