NCT02850692

Portal Hypertension and Systemic Endothelial Function

Portal Hypertension and Systemic Endothelial Function: Investigation of Systemic Endothelial Dysfunction in Case of Portal Hypertension Associated With Cystic Fibrosis.

• Status: Unknown
• Phase: N/A
• Study type: Interventional
• Enrollment: 60 (estimated)
• Sponsor: Hopital Foch · Academic / Other
• Sex: All
• Age: 18 Years
• Healthy volunteers: Accepted

Summary

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.

Detailed 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® * Contrast-enhanced tomography. Abdominal CT scan will not be performed in healthy volunteers.

Conditions

• Cystic Fibrosis
• Portal Hypertension

Interventions

Type	Name	Description
OTHER	measure of endothelial function	Arterial tone index measured by EndoPAT®. Patient should neither eat nor drink at least 4 hours before exam and should neither smoke 3 hours before the exam.
BIOLOGICAL	Blood sample	21 ml of blood to measure : hepatic workup, complete blood count (CBC), platelets, prothrombin time (PT), activated partial thromboplastin time (aPTT), C-reactive protein (CRP), lipid test, ionograms, creatinine, tissue plasminogen activator factor (tPA), plasminogen activator inhibitor-1 (PAI-1), tissue factor pathway inhibitor (TFPI), Willebrand factor, soluble thrombomodulin, blood levels of endoglin and syndecan. And level beta human chorionic gonadotropin (beta-HCG) for woman only.
OTHER	Hepatic elastography	Hepatic elastography by Fibrocan®. Patient should neither eat nor drink at least 2 hours before exam. 10 successive measurements are made.
DIAGNOSTIC_TEST	Injected abdominal CT	Patient should neither eat nor drink at least 4 hours before exam. The examination is not realized if an abdominal scan or an MRI was performed in the five years prior to the day of the visit.

Timeline

• Start date: 2016-04-18
• Primary completion: 2021-12-01
• Completion: 2021-12-01
• First posted: 2016-08-01
• Last updated: 2020-05-26

Locations

1 site across 1 country: France