Determinants of Early Cystic Fibrosis Lung Disease
| Status: | Recruiting |
|---|---|
| Conditions: | Pulmonary, Pulmonary, Pulmonary |
| Therapuetic Areas: | Pulmonary / Respiratory Diseases |
| Healthy: | No |
| Age Range: | Any - 5 |
| Updated: | 9/5/2018 |
| Start Date: | May 8, 2018 |
| End Date: | June 2020 |
| Contact: | Heather Muston, MD |
| Email: | hmuston@iupui.edu |
| Phone: | 317-948-7208 |
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.
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.
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.
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
We found this trial at
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Riley Hospital for Children Riley Hospital for Children at IU Health is a place of...
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