Prediction of Survival in Children With Cystic Fibrosis Using the Multiple-breath Washout

Multiple-breath Washout for Lung Function Testing in Children With Cystic Fibrosis: Predictive for Survival?

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.

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.

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