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

Insel Gruppe AG, University Hospital Bern200 enrolled

Overview

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.

Study Type

OBSERVATIONAL

Enrollment

200

Conditions

Cystic Fibrosis Death Lung Transplantation

Interventions

Lung function testingDIAGNOSTIC_TEST

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

Eligibility

Sex: ALLMin age: 3 Years

Medical Language ↔ Plain English

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

Locations (2)

University Children's Hospital Bern

Bern, Switzerland

Lindenhof Spital, Quartier Bleu

Bern, Switzerland

Outcomes

Primary Outcomes

Lung clearance index (LCI)

Lung function outcome derived by MBW and the compound Outcomes mortality or lung transplantation

Time frame: Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached

Secondary Outcomes

Functional residual capacity (FRC)

Lung function outcome derived by MBW

Time frame: 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

Time frame: Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached

Body weight

Anthropometric characteristics

Time frame: Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached

Body length

Anthropometric characteristics

Time frame: Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached

Gender

Anthropometric characteristics

Time frame: 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

Time frame: Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached

Data from ClinicalTrials.gov

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