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.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
PREVENTION
Masking
NONE
Enrollment
60
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.
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.
Hepatic elastography by Fibrocan®. Patient should neither eat nor drink at least 2 hours before exam. 10 successive measurements are made.
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.
Hopital Foch
Suresnes, France
RECRUITINGEndothelial systemic function measured by EndoPAT®
To compare the systemic endothelial function between the four study groups, the arterial tone index is measured by EndoPAT®. The EndoPAT® is the leading medical device for noninvasive endothelial function assessment. It quantifies the endothelium-mediated changes in vascular tone, elicited by a 5-minute occlusion of the brachial artery (using a standard blood pressure cuff). When the cuff is released, the surge of blood flow causes an endothelium-dependent. Flow Mediated Dilatation (FMD). The dilatation, manifested as Reactive Hyperemia, is captured by EndoPAT® as an increase in the PAT Signal amplitude. A post-occlusion to pre-occlusion ratio is calculated by the EndoPAT® software, providing the arterial tone index.
Time frame: 30 minutes
Serum levels of markers of endothelial dysfunction (tPA, thrombomodulin, Willebrandt factor, PAI1)
Blood sample (21 ml) is collected from each patients to dose the markers of endothelial dysfunction (tPA, thrombomodulin, Willebrandt factor, PAI1). The objective is to compare for the 4 groups the relations between: * the serum levels of markers of endothelial dysfunction and peripheral endothelial function; * And the serum levels of markers of endothelial dysfunction and the presence of portal hypertension (HTP).
Time frame: 30 minutes
Endogline/Syndecan-4 ratio measurement
Blood sample (21 ml) is collected from each patients to measure the blood levels of endoglin and syndecan. Endoglin and syndecan-4 are transmembrane glycoproteins present on endothelial cells. The objective is to compare for the 4 group the relation between : * endoglin/syndecan-4 ratio and peripheral endothelial function; * and, endoglin/syndecan-4 ratio and presence of HTP.
Time frame: 30 minutes
Measurement of hepatic elasticity by Fibroscan®
The objective is to compare for the 4 groups the relations between hepatic elasticity and endothelial systemic function. The Fibroscan measures the velocity of the sound wave passing through the liver from the device and then converts that measurement into a liver elasticity measurement. The result reveals the health of the liver: more the liver is hard, more his state is pathologic.
Time frame: 30 minutes
Hepatic abnormalities observed on injected abdominal CT.
All hepatic abnormalities observed on injected abdominal CT for groups A, B and C are analyzed. The objective is to compare the hepatic radiologic abnormalities associated with HTP in cystic fibrosis with hepatic radiologic abnormalities associated with HTP from other causes.
Time frame: 30 minutes
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