BioEnergetics and Metabolomics in Cystic Fibrosis (BEAM-CF)

Integration of Nutritional Metabolomics With Bioenergetics in Cystic Fibrosis

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.

Study Overview

• Status: Completed
• Conditions: Cystic Fibrosis

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.

• Study Type: Observational
• Enrollment (Actual): 50

Contacts and Locations

Study Locations

• United States
  • Georgia
    • Atlanta, Georgia, United States, 30322
      • Emory University Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 50 years (Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Adults with cystic fibrosis and healthy volunteers from the Atlanta community

Description

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.

Study Plan

How is the study designed?

Number of groups / cohorts

3

Cohorts and Interventions

Group / Cohort
Subjects with Cystic Fibrosis; Cross-sectional, observational study
healthy volunteers; Cross-sectional, observational study
Subjects with CF in a vitamin D study; 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?

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

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
whole-body fasting fat oxidation	This will be assessed using indirect calorimetry	Baseline for cross-sectional study and 1 year for longitudinal study
31P-magnetic resonance spectroscopy (MRS)-derived measures of resting thigh skeletal muscle bioenergetic metabolism	This will be assessed using MRI	Baseline for cross-sectional study

Collaborators and Investigators

• Sponsor: Emory University
• Collaborators: National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
• Investigators: Principal Investigator: Jessica A Alvarez, PhD, RD, Emory University

Study record dates

Study Major Dates

• Study Start: September 1, 2014
• Primary Completion (Actual): November 7, 2018
• Study Completion (Actual): November 7, 2018

Study Registration Dates

• First Submitted: August 21, 2014
• First Submitted That Met QC Criteria: August 24, 2014
• First Posted (Estimate): August 26, 2014

Study Record Updates

• Last Update Posted (Actual): November 29, 2019
• Last Update Submitted That Met QC Criteria: November 27, 2019
• Last Verified: November 1, 2019

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Digestive System Diseases; Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Infant, Newborn, Diseases; Genetic Diseases, Inborn; Pancreatic Diseases; Fibrosis; Cystic Fibrosis
• Other Study ID Numbers: IRB00073559; K01DK102851 (U.S. NIH Grant/Contract)