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

Study Overview
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Brief Summary
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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
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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.

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

Conditions
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Cystic Fibrosis, Death, Lung Transplantation

Intervention / Treatment
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* Diagnostic Test: Lung function testing


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 3 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Diagnostic Test: Lung 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|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Lung clearance index (LCI) | Lung function outcome derived by MBW and the compound Outcomes mortality or lung transplantation | Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Functional residual capacity (FRC) | Lung function outcome derived by MBW | Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | 
| Forced expired Volume in 1 second (FEV1) | Lung function outcome derived by Spirometry | Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | 
| Body weight | Anthropometric characteristics | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | 
| Body length | Anthropometric characteristics | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | 
| Gender | Anthropometric characteristics | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | 
| Presence of Pseudomonas aeruginosa infection | Clinical characteristics | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | 
| Presence of Staphylococcus aureus infection | Clinical characteristics | Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Multiple-breath washout, MBW, Lung clearance index, LCI, Prediction, Survival, Cystic Fibrosis, CF