Cystic Fibrosis, Respiratory Disease
Conditions
Brief summary
There are many techniques that can allow for the quantification of lung function in children; some are being used clinically and others are under development. Many of these tools are available at BC Children's Hospital. This registry study will act as a central repository for the results of traditional and novel pulmonary function tests done at BC Children's Hospital to allow for future analysis.
Detailed description
The scope of the registry is to collect data on small airways function in healthy children, children with cystic fibrosis (CF) and children with non-CF respiratory disease using the multiple breath washout (MBW) system. These data will be stored along with demographic data, clinical data, and traditional pulmonary function testing (PFT) data. The MBW data will be collected with concurrent with clinically indicated PFT testing. The purpose of the registry is to 1/ establish a locally collected normal range of small airways function across the paediatric age range and 2/ to collect these measurements in CF and non-CF respiratory disease. Ultimately, the objective of this registry is to validate MBW technology as a tool that can be utilized in clinical care.
Interventions
None listed
Sponsors
University of British Columbia
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
0 Years to 99 Years
Healthy volunteers
No
Inclusion criteria
Participants with CF Inclusion Criteria: -Diagnosis of CF as evidenced by one or more clinical feature consistent with the CF phenotype or positive CF newborn screen AND one or more of the following criteria: i. A documented sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis (QPIT). ii. A documented genotype with two disease-causing mutations in the CFTR gene. \- In the opinion of the investigator, the participant will likely have the ability to perform the lung function test of interest on the day of testing
Exclusion criteria
* Physical findings at the screening that would compromise the safety of the participant or the quality of the data (e.g. respiratory distress or work of breathing) * Requirement of supplementary oxygen to maintain oxygen saturation above 95% Participants with other Respiratory Disease Inclusion criteria: * Physician-diagnosed lung disease * Informed consent by participant, parent, or legal guardian * In the opinion of the investigator, the participant will likely have the ability to perform the lung function test of interest on the day of testing
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| To validate new lung function testing technologies | 10 years | The ultimate objective of this registry is to validate new lung function testing technologies as tools that can be utilized in clinical care. |
Countries
Canada
Contacts
Primary ContactRodrigo Sandoval, MSc
Backup ContactAlam Lakhani
Outcome results
None listed