PMID- 11776300
OWN - NLM
STAT- MEDLINE
DCOM- 20020712
LR  - 20041117
IS  - 0340-6245 (Print)
IS  - 0340-6245 (Linking)
VI  - 86
IP  - 6
DP  - 2001 Dec
TI  - Endothelial perturbation in cystic fibrosis.
PG  - 1363-7
AB  - 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.
FAU - Romano, M
AU  - Romano M
AD  - Department of Human Pathology, University of Messina, Italy.
FAU - Collura, M
AU  - Collura M
FAU - Lapichino, L
AU  - Lapichino L
FAU - Pardo, F
AU  - Pardo F
FAU - Falco, A
AU  - Falco A
FAU - Chiesa, P L
AU  - Chiesa PL
FAU - Caimi, G
AU  - Caimi G
FAU - Davi, G
AU  - Davi G
LA  - eng
PT  - Journal Article
PL  - Germany
TA  - Thromb Haemost
JT  - Thrombosis and haemostasis
JID - 7608063
RN  - 0 (Interleukin-6)
RN  - 0 (P-Selectin)
RN  - 0 (Tumor Necrosis Factor-alpha)
RN  - 0 (von Willebrand Factor)
RN  - EC 3.4.21.68 (Tissue Plasminogen Activator)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Cystic Fibrosis/blood/complications/*pathology
MH  - Disease Susceptibility
MH  - Endothelium, Vascular/metabolism/*pathology
MH  - Female
MH  - Humans
MH  - Inflammation
MH  - Interleukin-6/blood
MH  - Male
MH  - P-Selectin/*analysis
MH  - Pneumonia, Bacterial/blood/etiology/pathology/physiopathology
MH  - Pseudomonas Infections/blood/etiology/pathology/physiopathology
MH  - Recurrence
MH  - Respiratory Function Tests
MH  - Tissue Plasminogen Activator/*analysis
MH  - Tumor Necrosis Factor-alpha/analysis
MH  - von Willebrand Factor/*analysis
EDAT- 2002/01/05 10:00
MHDA- 2002/07/13 10:01
CRDT- 2002/01/05 10:00
PHST- 2002/01/05 10:00 [pubmed]
PHST- 2002/07/13 10:01 [medline]
PHST- 2002/01/05 10:00 [entrez]
AID - 01121363 [pii]
PST - ppublish
SO  - Thromb Haemost. 2001 Dec;86(6):1363-7.