Informations générales (source: ClinicalTrials.gov)
Portal Hypertension and Systemic Endothelial Function: Investigation of Systemic Endothelial Dysfunction in Case of Portal Hypertension Associated With Cystic Fibrosis. (ENDOTH-MUCO)
Interventional
N/A
Hopital Foch (Voir sur ClinicalTrials)
avril 2016
décembre 2021
29 juin 2024
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.
Etablissements
| Les établissements d'Île-de-France dont les données sont issues de ClinicalTrials.gov Origine et niveau de fiabilité des données | |||||
|---|---|---|---|---|---|
| HOPITAL FOCH | Dominique Grenet, MD | vendredi 11 mars 2022 | Contact (sur clinicalTrials) | ||
Critères
Tous
Inclusion Criteria:
- Patients over 18 years.
- Patients affiliated to a social security scheme
- Patients who have given their written consent
- Four study groups:
- Group A: Patients with cystic fibrosis (CF) with liver damage and diagnosis of CF is
based on sweat test and genetic analysis). PHT diagnosis is based on tomographic
criteria portal vein width superior to> 15 mm, portosystemic shunt and / or
splenomegaly
- Group B: cystic fibrosis patients without PHT diagnosis is based on sweat test and
genetic analysis). Absence of PHTP is predicated on tomographic of Scanner.
- Group C: Patients free of CF with PHT related to another cause. Patients followed
for viral liver disease (hepatitis B or C) or idiopathic portal venous system
disorder, with or without cirrhosis. The diagnosis of PHT is based on tomographic
criteria portal vein width superior to> 15 mm, highlighting porto-systemic shunt,
splenomegaly) and / or indirect signs namely ascitis or esophageal varices.
- Group D: Healthy controls.
- Patients over 18 years.
- Patients affiliated to a social security scheme
- Patients who have given their written consent
- Four study groups:
- Group A: Patients with cystic fibrosis (CF) with liver damage and diagnosis of CF is
based on sweat test and genetic analysis). PHT diagnosis is based on tomographic
criteria portal vein width superior to> 15 mm, portosystemic shunt and / or
splenomegaly
- Group B: cystic fibrosis patients without PHT diagnosis is based on sweat test and
genetic analysis). Absence of PHTP is predicated on tomographic of Scanner.
- Group C: Patients free of CF with PHT related to another cause. Patients followed
for viral liver disease (hepatitis B or C) or idiopathic portal venous system
disorder, with or without cirrhosis. The diagnosis of PHT is based on tomographic
criteria portal vein width superior to> 15 mm, highlighting porto-systemic shunt,
splenomegaly) and / or indirect signs namely ascitis or esophageal varices.
- Group D: Healthy controls.
- Patients suffering from uncontrolled hypertension despite treatment (systolic BP>
160 mmHg);
- Patient with uncontrolled diabetes (glycated Hb measurement done during the last 3
months > 7%);
- Patients with uncorrected dyslipidemia;
- Patient suffering from a sleep apnea syndrome;
- Patients with severe coagulation disorders: PR< 50%, platelets < 30,000 / microL,
current anticoagulant treatment;
- Patient with contra-indication to the injection of iodinated contrast material,
including history of hypersensitivity to iodinated contrast media or renal clearance
failure <50 ml / min Modification of Diet in Renal Disease (MDRD) formula
- Patients allergic to latex which contra-indicates endothelial function measurement;
- Acute pathology unresolved at the time of inclusion: respiratory exacerbation,
ongoing infection, recent thrombosis;
- Smoking history> 10 pack-years;
- Vasoactive therapy that may interfere with the measurement of endothelial function
and cannot be stopped 24 hours before the measurement: nitrates, beta-blockers,
angiotensin converting enzyme inhibitors, calcium channel blockers, inhibitors of
endothelin receptors, similar prostacyclin analog, inhibitors of phosphodiesterases;
- Pregnant and lactating women (all patients with childbearing potential will only be
included if their β-human chorionic gonadotropin (β-HCG) urine test is negative;
- Patient unable to provide written consent. Patient under guardianship.