Clinical Trials Logo

Clinical Trial Summary

Portal hypertension (PHT) and its sequelae are the most clinically important manifestation in cystic fibrosis related liver disease (CFLD), with end-stage liver failure as a late and rare manifestation. The aim is to evaluate the safety and efficacy of a pre-emptive Transjugular Intrahepatic Portosystemic Shunt (TIPS) for the prophylaxis of variceal bleeding in pediatric CFLD patients with subclinical non-cirrhotic portal hypertension (NCPH)


Clinical Trial Description

Cystic fibrosis (CF) is the most frequent autosomal recessive disorder in Caucasians caused by a mutation in the cystic fibrosis transmembrane conductance regulator gene. Cystic Fibrosis associated Liver Disease (CFLD) is a well-known complication and includes a wide range of hepatobiliary diseases. The clinical outcome in CFLD is largely determined by PHT and its sequelae. Variceal bleeding is the most feared complication. Currently, there is no medical therapy to delay or reverse clinically established CFLD.Treatment focuses on screening for and managing the complications of portal hypertension and optimizing nutritional status. Eventually, liver transplantation (LT) is an effective therapeutic option for CF patients with end-stage liver failure, treatment resistant, and complicated portal hypertension. TIPS placement is a well-established procedure for portal vein decompression in adults. In retrospective case studies of severe CFLD cirrhosis in pediatric populations, TIPS has been proven a feasible option in acute or refractory variceal bleeding and as a bridge to LT. In CFLD patients, little data are available on the long-term outcome of treatments to mitigate PHT. In this trial, the primary aim was to evaluate the safety and efficacy of a pre-emptive TIPS for the prophylaxis of variceal bleeding in non-cirrhotic CFLD with early PHT. The secondary aim was to investigate the long-term clinical outcome of a pre-emptive TIPS and in more particular, whether TIPS might effectively postpone LT. ;


Study Design


Related Conditions & MeSH terms


NCT number NCT05037643
Study type Interventional
Source University Hospital, Ghent
Contact
Status Completed
Phase N/A
Start date June 2007
Completion date December 2020

See also
  Status Clinical Trial Phase
Active, not recruiting NCT04602468 - Real World Clinical Outcomes With Novel Modulator Therapy Combinations in People With CF (RECOVER) Phase 4
Not yet recruiting NCT03264950 - Utility of Point Shear-wave Elastography to Assess for Hepatic & Pancreatic Fibrosis in Pediatric CF Patients N/A
Completed NCT03312140 - Examination of the Lipid Metabolism of the Liver After Choline Substitution in Cystic Fibrosis N/A
Completed NCT03961516 - Glycemic Characterization and Pancreatic Imaging Correlates in Cystic Fibrosis
Completed NCT03001388 - Longitudinal Assessment of Transient Elastography in Cystic Fibrosis
Withdrawn NCT05229640 - Relationship Between the Development of Impaired Glucose Tolerance, the Phenotype of CFLD, and the Risk of Liver Fibrosis N/A
Completed NCT02979340 - MRI to Characterize and Predict CF Liver Disease in PUSH Cohort
Completed NCT04277819 - The Use of Novel Diagnostic Tools to Increase Detection of Early Fibrosis in Cystic Fibrosis Related Liver Disease to Improve Clinical Management