role of body fat distribution in metabolic and pulmonary decline in cystic fibrosis (orbit-cf)

Conditions: Cystic Fibrosis

Summary: nutrition and body composition, the amount of muscle and fat in the body, has a role in overall health. this study wants to learn more about how nutrition and body composition affects health outcomes like glucose tolerance and lung function in patients with cystic fibrosis (cf) who are ages 16-30 years old. 60 adolescents and young adults with cf will be recruited, and 30 volunteers without cystic fibrosis. a total of 40 of these study participants with cf will be asked to return for annual study visits for 2 years after the first visit. the long-term goal of this study is to use the information collected to make decisions about future nutrition monitoring and interventions which help maintain optimal health for individuals with cf.

this is a prospective, observation study to test the central hypothesis that individuals with cystic fibrosis (cf) have a higher propensity to increased visceral adipose tissue (vat) accumulation and decreased lean body mass (lbm) compared to healthy controls, and this dysregulation in adipose and protein deposition exacerbates glucose intolerance and lung function decline. a sub-set of participants with cf will be followed longitudinally for two years (n=40). the investigators will conduct detailed body composition, fat distribution, metabolic, and nutritional phenotyping in this cohort. body fat distribution will be assessed with mri. whole body composition will be assessed with dexa. glucose tolerance will be assessed with an oral glucose tolerance test (ogtt) and mathematical modeling of the c-peptide and insulin response to glucose. lung health will be assessed by objective clinical data and self-reported symptoms.

Eligibility criteria:
inclusion criteria: cf inclusion criteria 1. confirmed cf diagnosis based on sweat testing by pilocarpine iontophoresis and/or cftr genotyping with two disease causing mutations 2. be aged ≥ 16 yrs 3. clinically stable, defined as no changes in medical regimen (including medications) for at least 21 days prior to study visit 4. participation in the cff patient registry longitudinal study inclusion: cf participants who have normal glucose tolerance results after their initial study oral glucose tolerance test (ogtt). healthy controls inclusion criteria: 1. male or female ages 16 years and older 2. clinically stable. healthy controls will be recruited who are similar in age, gender, and bmi as the participants with cf. exclusion criteria: cf exclusion criteria: 1. diagnosis of cf-related diabetes (cfrd) 2. nocturnal tube feeds 3. life expectancy \<6 months 4. history of or on waiting list for lung transplant 5. un-removable metal that is incompatible with mri 6. inability or unwillingness to perform major study activities (ogtt, dexa, mri) due to claustrophobia, fear of blood draw, or other reasons 7. current pregnancy or lactation 8. study visit falls between window of 1 week to 8 weeks of initiation of cftr modulator 9. use of chronic oral corticosteroids, 10. in the opinion of the cf care team or study physician, participant should not participate in the study, or 11. inability to provide informed consent or assent. healthy controls exclusion criteria: 1. malignant neoplasm (other than localized basal cell cancer of the skin) during the previous 5 years 2. respiratory (including asthma), endocrine (including diabetes), autoimmune, or other chronic disease 3. hiv or other chronic infection 4. current use of any medications to treat an acute or chronic disease or illness (anti-depressants, anti-anxiety medications are acceptable), 5. acute illness within the past 3 weeks 6. intravenous or oral antibiotics or use of systemic corticosteroids within the past 3 weeks 7. inability or unwillingness to perform major study activities due to claustrophobia, fear of blood draw, or other reasons 8. current pregnancy or lactation, or 9. inability to provide informed consent or assent.