BioEnergetics and Metabolomics in Cystic Fibrosis

Study Overview
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Brief Summary
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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
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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.

Official Title
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Integration of Nutritional Metabolomics With Bioenergetics in Cystic Fibrosis

Conditions
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Cystic Fibrosis

Participation Criteria
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Eligibility Criteria
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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 Exclusion criteria: current pregnancy current active malignant neoplasm or history of malignancy (other than localized basal cell cancer of the skin) during the previous 5 years current respiratory disease including asthma, chronic obstructive pulmonary disorder, or emphysema current chronic autoimmune or pro-inflammatory disease history of tuberculosis, HIV, or other chronic infection previous diagnosis of type 1 or type 2 diabetes with active treatment with insulin or other glucose lowering medication advanced (≥ stage 3) renal disease body mass index (BMI) ≥ 30 kg/m2 acute illness (such as a viral infection) within the past 2 weeks current use of any prescription medications that would indicate presence of an acute or chronic medical condition that may influence study results drug (recreational or prescription) or alcohol abuse weight instability (± 10% body weight within the last 6 months) or current participation in weight loss or weight gain program inability to provide informed consent.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 50 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Subjects with Cystic Fibrosis<br>Cross-sectional, observational study | |
| healthy volunteers<br>Cross-sectional, observational study | |
| Subjects with CF in a vitamin D study<br>This is a longitudinal observational study in subjects enrolled in a high-dose vitamin D study. The investigator (Jessica Alvarez) does not assign the intervention to the subjects of the study. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Acylcarnitine analysis by high-resolution liquid chromatography mass spectrometry | This will pe performed using targeted metabolomics methods | Baseline for cross-sectional study and 1 year for longitudinal study | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| quantitative plasma levels of specific plasma acylcarnitines | This will be measured in fasted blood | Baseline for cross-sectional study and 1 year for longitudinal study |