Clinical trial NCT04016194: Prediction of Survival in Children With Cystic Fibrosis Using the Multiple-breath Washout
Official title: Multiple-breath Washout for Lung Function Testing in Children With Cystic Fibrosis: Predictive for Survival?
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Cystic Fibrosis
Death
Lung Transplantation
Keywords: Multiple-breath washout, MBW
Lung clearance index, LCI
Prediction
Survival
Cystic Fibrosis, CF
Lead sponsor: Insel Gruppe AG, University Hospital Bern
Sponsor class: OTHER
Collaborators: name: Lindenhofspital
First posted: 2019-07-11
Enrollment: 200
Sex: ALL
Minimum age (years): 3
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: RETROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Non-Probability sample

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.

Eligibility criteria: 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

Primary outcomes:
measure: Lung clearance index (LCI)
timeFrame: Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached
Secondary outcomes:
measure: Functional residual capacity (FRC)
timeFrame: Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached
measure: Forced expired Volume in 1 second (FEV1)
timeFrame: Lung function is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached
measure: Body weight
timeFrame: Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached
measure: Body length
timeFrame: Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached
measure: Gender
timeFrame: Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached
measure: Presence of Pseudomonas aeruginosa infection
timeFrame: Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached
measure: Presence of Staphylococcus aureus infection
timeFrame: Characteristics are collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached
Other outcomes:
Locations:
city: Bern
country: Switzerland
facility: University Children's Hospital Bern
city: Bern
country: Switzerland
facility: Lindenhof Spital, Quartier Bleu
Study officials:
affiliation: Insel Gruppe AG, University Hospital Bern
name: Florian Singer
role: PRINCIPAL_INVESTIGATOR
References:
citation: Kurz JM, Ramsey KA, Rodriguez R, Spycher B, Fischer Biner R, Latzin P, Singer F. Association of lung clearance index with survival in individuals with cystic fibrosis. Eur Respir J. 2022 Mar 3;59(3):2100432. doi: 10.1183/13993003.00432-2021. Print 2022 Mar.