Cystic Fibrosis Primary Ciliary Dyskinesia
Conditions
Interventions
None listed
Sponsors
Academisch Medisch Centrum
Eligibility
Age
2 Years to 99 Years
Inclusion criteria
Inclusion criteria: * CF diagnosis is based on: clinical symptoms in combination with an abnormal sweat test (chloride > 60 mmol/l) and/or identification of mutations in both alleles of the CFTR gene. * PCD diagnosis is based on: a combination of clinical symptoms, abnormal movement of cilia on microscopic evaluation of respiratory epithelial biopsies and epithelial cell cultures, or identification of an ultra structural defect in the cilia by electron microscopy. * * 6 years of age * Stable respiratory disease for at least 6 weeks ( as determined by the treating physician) * Ability to perform lung function measurement
Exclusion criteria
Exclusion criteria: * Mental retardation * Diabetes Mellitus (CF complication) * Technical unsatisfactory performance of measurements * On the waiting list for lung transplantation * Participation in the PREVEC or VERTEX study (AMC)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Primary outcome measures: relative changes in electronic nose sensors, retention time, abundance and mass to charge ratio from GC-MS analysis. | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary outcomes: (change in) bacterial diversity. | — |
Countries
Netherlands
Outcome results
None listed