Determinants of Early Cystic Fibrosis Lung Disease
Study Overview
Status
Conditions
Intervention / Treatment
Detailed Description
The overall objective of this study is to determine the impact early nutritional and respiratory indices have on early CF lung disease. This knowledge will guide clinical management of infants with CF, who are now primarily diagnosed through newborn screening. We hypothesize that respiratory and nutritional indices during the first year of life are critical determinants of lung function in infants and preschoolers with CF.
Aim #1 - To examine the relationship between nutritional status (weight-for-age (WFA) and weight-for-length (WFL)) at 6 months of age and lung function at 1-2 years of age in infants with CF.
Hypothesis: Infants with CF with poor nutritional status at 6 months of age will have worse lung function, as assessed through the raised volume rapid thoracoabdominal compression technique and plethysmography, compared to those with better nutritional indices, defined as improved weight-for-age and weight-for-length.
Aim #2 - To examine the relationship between nutritional status (WFA and WFL) in infants with CF at 12 months of age and the lung clearance index (LCI) at 3-5 years of age.
Hypothesis: Infants with CF with poor nutritional indices at 12 months of age will have a higher (worse) LCI at 3-5 years of age compared to those with better nutritional status.
Aim #3 - To delineate the relationship between passive tidal breathing lung function testing in infants with CF at 4-8 weeks of age and subsequent lung function at 6-12 months of age.
Hypothesis: Abnormalities in passive tidal breathing lung function testing will be associated with abnormal infant pulmonary function testing obtained via the raised volume rapid thoracoabdominal compression technique and plethysmography at 6-12 months of age.
Study Type
Enrollment (Actual)
Contacts and Locations
Study Locations
-
-
Indiana
-
Indianapolis, Indiana, United States, 46202
- Riley Hospital for Children
-
-
Participation Criteria
Eligibility Criteria
Ages Eligible for Study
Accepts Healthy Volunteers
Genders Eligible for Study
Sampling Method
Study Population
Description
Inclusion Criteria:
- Informed consent by a parent or legal guardian
- A confirmed diagnosis of CF by newborn screening and either a documented sweat chloride of at least 60 mEq/L by quantitative pilocarpine iontophoresis or a genotype with two CF-causing mutations
- One month to five years of age.
Exclusion Criteria:
- Chronic lung disease not due to CF
- Contraindications to sedation, including structural abnormalities of the upper airway or chest wall and severe gastroesophageal reflux
- Gestational age < 36 weeks
Study Plan
How is the study designed?
Design Details
Cohorts and Interventions
Group / Cohort |
Intervention / Treatment |
|---|---|
|
Nutrition and Infant PFT
To examine the relationship between nutritional status (weight-for-age (WFA) and weight-for-length (WFL)) at 6 months of age and lung function at 1-2 years of age in infants with CF.
|
Assessed through the raised volume rapid thoracoabdominal compression technique and plethysmography
|
|
Nutrition and Lung Clearance Index
To examine the relationship between nutritional status (WFA and WFL) in infants with CF at 12 months of age and the lung clearance index (LCI) at 3-5 years of age.
|
The Lung Clearance Index is derived from the Multiple Breath Washout test.
It is the cumulative exhaled volume (the volume of gas needed to wash out resident gas mixture out of the lungs) divided by the FRC.
The FRC is the amount of air left in the lungs after normal exhalation.
|
|
Passive Tidal Breathing and Infant PFT
To delineate the relationship between passive tidal breathing lung function testing in infants with CF at 4-8 weeks of age and subsequent lung function at 6-12 months of age.
|
Assessed through the raised volume rapid thoracoabdominal compression technique and plethysmography
During quiet sleep, tidal breathing flow-volume curves are obtained.
The tPEF/tE is calculated by taking the time it takes to reach peak expiratory flow divided by the total peak expiratory time.
|
What is the study measuring?
Primary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
|---|---|---|
|
Lung function
Time Frame: 24 months
|
Forced expiratory volume in 0.5 seconds
|
24 months
|
Secondary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
|---|---|---|
|
Lung function - FRC
Time Frame: 24 months
|
Functional Residual Capacity
|
24 months
|
|
Lung function - MBW
Time Frame: 24 months
|
Multiple breath washout result
|
24 months
|
Collaborators and Investigators
Sponsor
Investigators
- Principal Investigator: Heather Muston, MD, Indiana University
Study record dates
Study Major Dates
Study Start (Actual)
Primary Completion (Actual)
Study Completion (Actual)
Study Registration Dates
First Submitted
First Submitted That Met QC Criteria
First Posted (Actual)
Study Record Updates
Last Update Posted (Actual)
Last Update Submitted That Met QC Criteria
Last Verified
More Information
Terms related to this study
Keywords
Additional Relevant MeSH Terms
Other Study ID Numbers
- 1712581512
Plan for Individual participant data (IPD)
Plan to Share Individual Participant Data (IPD)?
Drug and device information, study documents
Studies a U.S. FDA-regulated drug product
Studies a U.S. FDA-regulated device product
This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.