Cystic Fibrosis
Conditions
Brief summary
With better medical care, patients with cystic fibrosis (CF), a life-threatening disease, are enjoying longer lives. As the CF life expectancy increases, conditions such as CF-related diabetes (CFRD) become more prevalent. Nutrition plays a major role in maintaining optimal health in cystic fibrosis (CF). This project is designed to investigate nutrition-related factors, such as diet and body composition, on outcomes in patients with CF. The data generated from this study will be used to inform future nutrition intervention studies in adults with CF and CFRD.
Detailed description
Research methods include blood draws for plasma markers of oxidative stress and metabolomics (the measurement of thousands of small chemicals in the blood), magnetic resonance imaging (MRI, to assess energy capacity and thigh fat), dual energy X-ray absorptiometry (DEXA, to assess total and regional body fat and muscle), and indirect calorimetry (to assess resting energy expenditure and whole body fat oxidation). Clinically-relevant data, such as lung function, CF genotype, and diabetes status will be obtained from the electronic medical record and the CF Registry.
Interventions
None listed
Sponsors
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Emory University
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 50 Years
Healthy volunteers
Yes
Inclusion criteria
Cystic Fibrosis Inclusion Criteria: * have confirmed CF diagnosis with at least one Class I to III CFTR mutations * age ≥ 18 yrs * on a clinically-stable medical regimen for 3 wks * no intravenous or oral antibiotics for at least 3 wks prior to study visit
Exclusion criteria
* current pregnancy * unwilling or unable to discontinue enteral tube feeds for one night before the study visit, if applicable * most recent FEV1% \<40% * drug (recreational or prescription) or alcohol abuse * pacemaker or any electronic implantable device * unable to give informed consent Healthy volunteers Inclusion criteria: * 18-50 yrs of age * ambulatory status * absence of hospitalization in the previous year except for accidents
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Acylcarnitine analysis by high-resolution liquid chromatography mass spectrometry | Baseline for cross-sectional study and 1 year for longitudinal study | This will pe performed using targeted metabolomics methods |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| quantitative plasma levels of specific plasma acylcarnitines | Baseline for cross-sectional study and 1 year for longitudinal study | This will be measured in fasted blood |
Other
| Measure | Time frame | Description |
|---|---|---|
| whole-body fasting fat oxidation | Baseline for cross-sectional study and 1 year for longitudinal study | This will be assessed using indirect calorimetry |
| 31P-magnetic resonance spectroscopy (MRS)-derived measures of resting thigh skeletal muscle bioenergetic metabolism | Baseline for cross-sectional study | This will be assessed using MRI |
Countries
United States
Outcome results
None listed