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Thromb Haemost 2001; 86(06): 1363-1367
DOI: 10.1055/s-0037-1616736
Review Article
Schattauer GmbH

Endothelial Perturbation in Cystic Fibrosis

Mario Romano
1   Department of Human Pathology, University of Messina
,
Mirella Collura
2   Cystic Fibrosis Center, Di Cristina Hospital, Palermo, and Department of Medicine, University of Palermo
,
Luciana Iapichino
2   Cystic Fibrosis Center, Di Cristina Hospital, Palermo, and Department of Medicine, University of Palermo
,
Francesca Pardo
2   Cystic Fibrosis Center, Di Cristina Hospital, Palermo, and Department of Medicine, University of Palermo
,
Angela Falco
3   Department of Medicine and Aging, University of Chieti “G. D’Annunzio” School of Medicine, Italy
,
Pierluigi Lelli Chiesa
3   Department of Medicine and Aging, University of Chieti “G. D’Annunzio” School of Medicine, Italy
,
Gregorio Caimi
2   Cystic Fibrosis Center, Di Cristina Hospital, Palermo, and Department of Medicine, University of Palermo
,
Giovanni Davì
3   Department of Medicine and Aging, University of Chieti “G. D’Annunzio” School of Medicine, Italy
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Publikationsverlauf

Received 24. März 2001

Accepted after resubmission 06. August 2001

Publikationsdatum:
12. Dezember 2017 (online)

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Summary

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- (TNF-) and interleukin (IL)-6 levels were also increased in CF patients and significant direct correlations were found between TNF- 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.

Keywords

Endothelium - cystic fibrosis - von Willebrand factor - tissue plasminogen activator - P-selectin
 

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