Cystic Fibrosis Liver Disease Clinical Trial
Official title:
Pre-emptive Transjugular Intrahepatic Portosystemic Shunt (TIPS) in Pediatric Patients With Cystic Fibrosis Related Liver Disease and Non-cirrhotic Portal Hypertension
NCT number | NCT05037643 |
Other study ID # | B67020071504 |
Secondary ID | |
Status | Completed |
Phase | N/A |
First received | |
Last updated | |
Start date | June 2007 |
Est. completion date | December 2020 |
Verified date | August 2021 |
Source | University Hospital, Ghent |
Contact | n/a |
Is FDA regulated | No |
Health authority | |
Study type | Interventional |
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)
Status | Completed |
Enrollment | 5 |
Est. completion date | December 2020 |
Est. primary completion date | December 2020 |
Accepts healthy volunteers | No |
Gender | All |
Age group | N/A to 18 Years |
Eligibility | Inclusion Criteria: - Cystic fibrosis - Liver disease detected by an abnormal physical examination (hepatomegaly or splenomegaly, confirmed on US), abnormalities of liver function tests (increase of AST, ALT, GGT levels above the upper normal limits) or ultrasonographic evidence of liver involvement (US liver score = 5). - Indirect signs of portal hypertension on Doppler US - Progressive portal hypertension / liver disease on bi-monthly follow-up, evaluated by physical examination, blood analysis and US Exclusion Criteria: - Cirrhosis on biopsy - Symptomatic portal hypertension (Portosytemic pressure gradient > 10 mmHg) |
Country | Name | City | State |
---|---|---|---|
n/a |
Lead Sponsor | Collaborator |
---|---|
University Hospital, Ghent |
Type | Measure | Description | Time frame | Safety issue |
---|---|---|---|---|
Other | Liver transplant/splenectomy-free survival | Transplant/splenectomy-free survival time was calculated from date of TIPS to the following event: transplant, splenectomy and death from any cause. | Through study completion, an average of 10 year | |
Other | Model for end-stage liver disease (MELD) score | The Model for End-Stage Liver Disease, or MELD, is a scoring system for assessing the severity of chronic liver disease. | Through study completion, an average of 10 year | |
Other | Body Mass Index (BMI) for age Z-score | BMI-for age Z-scores are measures of relative weight adjusted for child age and sex defined by the Flemish growth charts | Through study completion, an average of 10 year | |
Other | Length for age Z-score | Length-for age Z-scores are measures of relative length adjusted for child age and sex defined by the Flemish growth charts | Through study completion, an average of 10 year | |
Primary | Variceal bleeding | Variceal bleeding, diagnosed on endoscopy, is regarded as the main complication of portal hypertension from cystic fibrosis liver disease | Through study completion, an average of 10 year | |
Secondary | Hypersplenism | Thrombocytopenia causes an increased bleeding tendency. Increasing splenomegaly causes earlier concerns with abdominal distension and decreased appetite by gastric compression. In symptomatic hypersplenism, a surgical splenorenal shunt or splenectomy might be indicated. | Through study completion, an average of 10 year |
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
|