Evidence of vascular endothelial dysfunction in young patients with cystic fibrosis

Spencer Poore, Breana Berry, Dabney Eidson, Kathleen T McKie, Ryan A. Harris

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

Background: Cystic fibrosis (CF) is a genetic disorder that affects not only pulmonary function but also multiple organ systems. The flow-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. FEV1 (% predicted), FEV1 /FVC, and forced expiratory flow 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 =.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 first 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.

Original languageEnglish (US)
Pages (from-to)939-945
Number of pages7
JournalChest
Volume143
Issue number4
DOIs
StatePublished - Jan 1 2013

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Cystic Fibrosis
Blood Vessels
Dilatation
Lung
Exercise
Oxygen Consumption
Inborn Genetic Diseases
Brachial Artery
Spirometry
Vital Capacity
Workload
Exercise Test
Biological Availability
Nitric Oxide
Fats

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Evidence of vascular endothelial dysfunction in young patients with cystic fibrosis. / Poore, Spencer; Berry, Breana; Eidson, Dabney; McKie, Kathleen T; Harris, Ryan A.

In: Chest, Vol. 143, No. 4, 01.01.2013, p. 939-945.

Research output: Contribution to journalArticle

Poore, Spencer ; Berry, Breana ; Eidson, Dabney ; McKie, Kathleen T ; Harris, Ryan A. / Evidence of vascular endothelial dysfunction in young patients with cystic fibrosis. In: Chest. 2013 ; Vol. 143, No. 4. pp. 939-945.
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