title: Endothelial perturbation in cystic fibrosis.
abstract: Cystic fibrosis (CF) is characterized by a persistent inflammatory state, which can be secondary to chronic pulmonary infection and may affect vascular endothelium. We measured circulating levels of von Willebrand factor (vWF), tissue-plasminogen activator (t-PA), and P-selectin in 20 CF patients and 20 healthy subjects. vWF, t-PA and P-selectin levels were significantly higher in CF patients. Endothelial perturbation (>2 SD increase in both vWF and t-PA) was present in 65% of CF patients. These patients displayed lower FEV1 values compared to individuals without endothelial perturbation and an inverse correlation between FEV1 and P-selectin levels was observed. Tumor necrosis factor-alpha (TNF-alpha) and interleukin (IL)-6 levels were also increased in CF patients and significant direct correlations were found between TNF-alpha and vWF, t-PA or P-selectin levels. These results indicate that CF patients exhibit signs of endothelial dysfunction/perturbation, which are likely to be related to a persistent inflammatory state due to chronic pulmonary infection, and may play a role in the progression of this disease.
authors.affiliation: Department of Human Pathology, University of Messina, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Romano
authors.forename: M
authors.initials: M
authors.lastname: Collura
authors.forename: L
authors.initials: L
authors.lastname: Lapichino
authors.forename: F
authors.initials: F
authors.lastname: Pardo
authors.forename: A
authors.initials: A
authors.lastname: Falco
authors.forename: P L
authors.initials: PL
authors.lastname: Chiesa
authors.forename: G
authors.initials: G
authors.lastname: Caimi
authors.forename: G
authors.initials: G
authors.lastname: Davì
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000097:blood / Q000150:complications / Q000473:pathology*; D004198:Disease Susceptibility; D004730:Endothelium, Vascular / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D007249:Inflammation; D015850:Interleukin-6 / Q000097:blood; D008297:Male; D019007:P-Selectin / Q000032:analysis*; D018410:Pneumonia, Bacterial / Q000097:blood / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D011552:Pseudomonas Infections / Q000097:blood / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D012008:Recurrence; D012129:Respiratory Function Tests; D010959:Tissue Plasminogen Activator / Q000032:analysis*; D014409:Tumor Necrosis Factor-alpha / Q000032:analysis; D014841:von Willebrand Factor / Q000032:analysis*
chemical_list: D015850:Interleukin-6; D019007:P-Selectin; D014409:Tumor Necrosis Factor-alpha; D014841:von Willebrand Factor; D010959:Tissue Plasminogen Activator