Last updated on August 2019

Portal Hypertension and Systemic Endothelial Function


Brief description of study

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 PHTassociated 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.

Detailed Study Description

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
  • Contrastenhanced tomography. Abdominal CT scan will not be performed in healthy volunteers.

Clinical Study Identifier: NCT02850692

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Hopital Foch

Suresnes, France
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