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.

journal_name

Thromb Haemost

authors

Romano M,Collura M,Lapichino L,Pardo F,Falco A,Chiesa PL,Caimi G,Davì G

keywords:

subject

Has Abstract

pub_date

2001-12-01 00:00:00

pages

1363-7

issue

6

eissn

0340-6245

issn

2567-689X

pii

01121363

journal_volume

86

pub_type

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