Cystic Fibrosis, Death, Lung Transplantation
Conditions
Keywords
Multiple-breath washout, MBW, Lung clearance index, LCI, Prediction, Survival, Cystic Fibrosis, CF
Brief summary
Retrospective cohort study using routinely collected annual data on lung clearance index (LCI) in combination with clinical data to predict survival in patients with Cystic Fibrosis. The primary study endpoint is the association of LCI with the compound outcome survival or lung transplantation in patients with CF.
Detailed description
Retrospective analysis of longitudinal data collected during clinically routine from approximately 200 patients with CF aged 3 years or more between 01.01.1980 and 31.12.2005. The overall aim of this project is to retrospectively assess whether LCI measured for clinical purposes during school age is predictive for survival or lung transplantation in adulthood. For this, the investigators will assess in a systematic way clinically measured LCI and relate them with the compound outcome survival or lung transplantation retrospectively.
Interventions
DIAGNOSTIC_TESTLung function testing
Multiple-breath washout (MBW) is a safe, easy and sensitive lung function test using inert gases such as Nitrogen (N2) to assess efficiency of ventilation distribution. The latter can be estimated by the lung clearance index (LCI), an established study end-point in patients with CF
Sponsors
Lindenhofspital
Insel Gruppe AG, University Hospital Bern
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
3 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patients treated at the Children's Hospital Bern * Confirmed CF Diagnosis * Age \>= 3 years
Exclusion criteria
* Uncertain diagnosis of CF according to current standards * Existing written or otherwise documented refusal to participate in research
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Lung clearance index (LCI) | Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | Lung function outcome derived by MBW and the compound Outcomes mortality or lung transplantation |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Forced expired Volume in 1 second (FEV1) | Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | Lung function outcome derived by Spirometry |
| Body weight | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | Anthropometric characteristics |
| Body length | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | Anthropometric characteristics |
| Functional residual capacity (FRC) | Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | Lung function outcome derived by MBW |
| Presence of Pseudomonas aeruginosa infection | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | Clinical characteristics |
| Presence of Staphylococcus aureus infection | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | Clinical characteristics |
| Gender | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | Anthropometric characteristics |
Countries
Switzerland
Outcome results
None listed