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

NCT04016194 | Completed

• 1 other identifier: 3840
• Study Type: observational
• Target: 200
• Locations: 1 country
• Sites: 2

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.

Conditions

Cystic Fibrosis; Death; Lung Transplantation

Keywords

Multiple-breath washout, MBW; Lung clearance index, LCI; Prediction; Survival; Cystic Fibrosis, CF

Outcome Measures

Primary Outcomes (1)

• 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 Outcomes (7)

• Functional residual capacity (FRC)

  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 is collected quarterly until outcome (death or lung transplantation) or end of Study (12/2005) is reached

• Body weight

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

• Body length

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

• Gender

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

Interventions

Lung function testing DIAGNOSTIC_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

Also known as: Multiple-breath washout, MBW

Eligibility Criteria

• Age: 3 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Non-Probability sample

You may qualify if:

• Patients treated at the Children's Hospital Bern
• Confirmed CF Diagnosis
• Age \>= 3 years

You may not qualify if:

• Uncertain diagnosis of CF according to current standards
• Existing written or otherwise documented refusal to participate in research

Sponsors & Collaborators

• Insel Gruppe AG, University Hospital Bern: lead
• Lindenhofspital: collaborator

Study Sites (2)

University Children's Hospital Bern

Bern, 3007, Switzerland

Location

Lindenhof Spital, Quartier Bleu

Bern, 3012, Switzerland

Location

Related Publications (1)

• 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.

  PMID: 34289977 DERIVED

MeSH Terms

Conditions

Cystic Fibrosis; Death

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases; Lung Diseases; Respiratory Tract Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Infant, Newborn, Diseases; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Study Officials

• Florian Singer

  Insel Gruppe AG, University Hospital Bern

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: May 7, 2019
• First Posted: July 11, 2019
• Study Start: January 1, 1990
• Primary Completion: December 1, 2005
• Study Completion: December 1, 2005
• Last Updated: July 11, 2019

Record last verified: 2019-07

Data Sharing

• IPD Sharing: Will share
• Shared Documents: STUDY PROTOCOL, SAP, ICF, CSR
• Time Frame: Until 12/2020
• Access Criteria: Researchers involved in data collection

Locations

CH (2)