Cystic Fibrosis Clinical Trial
Official title:
Portal Hypertension and Systemic Endothelial Function: Investigation of Systemic Endothelial Dysfunction in Case of Portal Hypertension Associated With Cystic Fibrosis.
Cystic fibrosis can affect organs other than the lungs. Liver disease affects about 30% of
patients: its main manifestation is the development of portal hypertension (PHT). The
pathophysiology of this comorbidity is still poorly understood. It was previously considered
secondary to the formation of biliary cirrhosis but another hypothesis would be that of a
primitive pathology of venous vessels may cause the gradual emergence of portal hypertension
without cirrhosis. Evidence indiscutly suggest that cystic fibrosis is associated with a
specific endothelial dysfunction, especially as the CFTR (Cystic Fibrosis Transmembrane
conductance Regulator) protein is expressed on the surface of endothelial cells. The
investigators hypothesize that liver disease related to PHT−associated cystic fibrosis is
associated with systemic endothelial dysfunction.
The aim is:
To demonstrate a systemic endothelial dysfunction in patients with cystic fibrosis when
associated with PHT.
To study the correlations between measures of systemic endothelial function and serum markers
of endothelial dysfunction and between measures of liver stiffness and systemic endothelial
function.
Prospective , monocentric study, with four groups of patients:
- Patients with cystic fibrosis and PHT
- Cystic fibrosis patients without PHT
- Patients free from cystic fibrosis with PHT from other causes
- Healthy controls. One study visit, no follow-up.
During the visit the following examinations will be performed:
- Collection of a blood sample of 21 mL.
- Liver eElastography achieved through hardware FibroScan® -
- Measurement of endothelial function with Endopat®
- Contrast−enhanced tomography. Abdominal CT scan will not be performed in healthy
volunteers.
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