title: Evidence of vascular endothelial dysfunction in young patients with cystic fibrosis.
abstract: BACKGROUND
  Cystic fibrosis (CF) is a genetic disorder that affects not only pulmonary function but also multiple organ systems. The fl ow-mediated dilation (FMD) test is a noninvasive assessment of endothelial function and nitric oxide bioavailability. Thus, the purpose of this study was to determine (1) whether endothelial dysfunction is present in young patients with CF and (2) whether endothelial function is associated with pulmonary function and exercise capacity.
  
  
  METHODS
  Fifteen patients with CF and 15 demographically matched control subjects participated in this study. Spirometry, brachial-artery FMD, and a maximal exercise capacity test on a cycle ergometer were performed on all subjects to determine pulmonary function, endothelial function, and exercise capacity, respectively.
  
  
  RESULTS
  No differences ( P . .05) in age, height, or BMI were observed between patients with CF and control subjects. FEV 1 (% predicted), FEV 1 /FVC, and forced expiratory fl ow between 25% and 75% of vital capacity were lower in patients with CF. Volume of oxygen consumption peak (absolute and relative) was similar between groups; however, volume of oxygen consumption (% predicted and mL/kg fat-free mass/min) and peak workload were significantly ( P , .05) lower in patients with CF. FMD (4.9% 2.6% vs 7.5% 3.1%; P 5 .018) was lower in patients compared with control subjects, respectively. Relationships between FMD and both pulmonary function and exercise capacity were identified.
  
  
  CONCLUSIONS
  For the fi rst time to our knowledge, these data provide evidence of vascular endothelial dysfunction in a fairly healthy cohort of young patients with CF. In addition, our data demonstrate the complex relationships between endothelial function and both pulmonary function and exercise capacity in young patients with CF.
authors.affiliation: Georgia Prevention Center, Georgia Health Sciences University, Augusta, GA; These data were presented in abstract form. Flow-mediated dilation is attenuated in young patients with cystic fibrosis. FASEB J. 2012;26:1130.13.
authors.forename: Spencer
authors.initials: S
authors.lastname: Poore
authors.affiliation: Georgia Prevention Center, Georgia Health Sciences University, Augusta, GA; These data were presented in abstract form. Flow-mediated dilation is attenuated in young patients with cystic fibrosis. FASEB J. 2012;26:1130.13.
authors.forename: Breana
authors.initials: B
authors.lastname: Berry
authors.affiliation: Pediatric Pulmonology, Georgia Health Sciences University, Augusta, GA; These data were presented in abstract form. Flow-mediated dilation is attenuated in young patients with cystic fibrosis. FASEB J. 2012;26:1130.13.
authors.forename: Dabney
authors.initials: D
authors.lastname: Eidson
authors.affiliation: Pediatric Pulmonology, Georgia Health Sciences University, Augusta, GA; These data were presented in abstract form. Flow-mediated dilation is attenuated in young patients with cystic fibrosis. FASEB J. 2012;26:1130.13.
authors.forename: Kathleen T
authors.initials: KT
authors.lastname: McKie
authors.affiliation: Georgia Prevention Center, Georgia Health Sciences University, Augusta, GA; These data were presented in abstract form. Flow-mediated dilation is attenuated in young patients with cystic fibrosis. FASEB J. 2012;26:1130.13. Electronic address: ryharris@georgiahealth.edu.
authors.forename: Ryan A
authors.initials: RA
authors.lastname: Harris
mesh_terms: D000293:Adolescent; D001916:Brachial Artery / Q000502:physiology; D016022:Case-Control Studies; D002648:Child; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000503:physiopathology*; D004730:Endothelium, Vascular / Q000503:physiopathology*; D005080:Exercise Test; D017079:Exercise Tolerance / Q000502:physiology*; D005260:Female; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D009569:Nitric Oxide / Q000378:metabolism; D010101:Oxygen Consumption / Q000502:physiology; D012129:Respiratory Function Tests; D014664:Vasodilation / Q000502:physiology
chemical_list: D009569:Nitric Oxide