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.
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
OBSERVATIONAL
Enrollment
61
Assessed through the raised volume rapid thoracoabdominal compression technique and plethysmography
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.
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.
Riley Hospital for Children
Indianapolis, Indiana, United States
Lung function
Forced expiratory volume in 0.5 seconds
Time frame: 24 months
Lung function - FRC
Functional Residual Capacity
Time frame: 24 months
Lung function - MBW
Multiple breath washout result
Time frame: 24 months
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