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Cystic Fibrosis Liver Disease clinical trials

View clinical trials related to Cystic Fibrosis Liver Disease.

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NCT ID: NCT05229640 Withdrawn - Clinical trials for Cystic Fibrosis-related Diabetes

Relationship Between the Development of Impaired Glucose Tolerance, the Phenotype of CFLD, and the Risk of Liver Fibrosis

Start date: March 31, 2022
Phase: N/A
Study type: Interventional

This study proposes to examine the relationship between the development of impaired glucose tolerance, the phenotype of CFLD, and risk of liver fibrosis.

NCT ID: NCT05037643 Completed - Clinical trials for Cystic Fibrosis Liver Disease

Pre-emptive Transjugular Intrahepatic Portosystemic Shunt (TIPS) in Cystic Fibrosis Related Liver Disease

Start date: June 2007
Phase: N/A
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)

NCT ID: NCT04602468 Active, not recruiting - Cystic Fibrosis Clinical Trials

Real World Clinical Outcomes With Novel Modulator Therapy Combinations in People With CF (RECOVER)

RECOVER
Start date: September 3, 2020
Phase: Phase 4
Study type: Interventional

RECOVER is a prospective, multicenter observational study designed to measure the real world clinical effectiveness of elexacaftor, tezacaftor and ivacaftor triple combination therapy (Kaftrio) in people with cystic fibrosis over a two year period. Measured outcomes include measures of lung function, lung inflammation, lung imaging, abdominal symptoms, gut inflammation, liver function, pancreatic exocrine function, nasal inflammation, quality of life and adherence to therapy. The study will examine outcomes in children aged six years and above over a period of two years. The first phase of the study will commence in 2020, recruiting children 12 years and older who have started on clinical treatment with Kaftrio.

NCT ID: NCT04277819 Completed - Cystic Fibrosis Clinical Trials

The Use of Novel Diagnostic Tools to Increase Detection of Early Fibrosis in Cystic Fibrosis Related Liver Disease to Improve Clinical Management

Start date: February 15, 2019
Phase:
Study type: Observational [Patient Registry]

Cystic Fibrosis (CF) is a genetic condition which affects 1 in 2500 newborn infants and is the commonest genetic condition in the UK. 1 in 25 of the white population carry the mutation. The genetic defect prevents the movement of fluids from cells, leading to thickened secretions and injury. With improvements in treatments from the commonest organ affected, the lungs, patients born with CF now can expect to live into their 40s with more than 60% living past 16. Though better, more can be done. As treatments from lung complications have improved, the management of liver disease (second commonest organ involved) remains unchanged for a considerable time. Treatment options are limited with liver transplant the only curative option. Though potentially life-saving, it has risks and an organ shortage means alternative treatment options are desperately needed. Identifying those with or at risk of Cystic Fibrosis related liver disease is difficult due to inadequate diagnostic tools. Routine blood tests are unreliable; therefore specific blood tests to identify scarring of the liver (biomarkers) are urgently needed. Ultrasound scan, the recommended diagnostic investigation, is only accurate in identifying the late stages of liver disease. For new therapies to be most effective we need to be able to identify patients at a much earlier stage. This study will use multi-modality testing, including imaging techniques such as FibroScan, MRI scan and blood tests (biomarkers), to diagnose those with liver scarring and use this to better categorise disease.

NCT ID: NCT03961516 Completed - Cystic Fibrosis Clinical Trials

Glycemic Characterization and Pancreatic Imaging Correlates in Cystic Fibrosis

CFCGM
Start date: May 1, 2019
Phase:
Study type: Observational

The purpose of this study is to investigate the utility of a continuous glucose monitor device (CGM) in screening for cystic fibrosis related diabetes. The investigators will also study how fat deposition in the pancreas and liver impacts insulin production and response, as measured by a frequently sampled oral glucose tolerance test.

NCT ID: NCT03312140 Completed - Clinical trials for Cystic Fibrosis Liver Disease

Examination of the Lipid Metabolism of the Liver After Choline Substitution in Cystic Fibrosis

Start date: November 6, 2014
Phase: N/A
Study type: Interventional

Pilot study to investigate the effect of choline chloride in cystic fibrosis patients with liver steatosis by comparing their status before and after the intervention

NCT ID: NCT03264950 Not yet recruiting - Cystic Fibrosis Clinical Trials

Utility of Point Shear-wave Elastography to Assess for Hepatic & Pancreatic Fibrosis in Pediatric CF Patients

CFALD
Start date: October 1, 2017
Phase: N/A
Study type: Interventional

Diagnosis of hepatic fibrosis is challenging as specific tests for detection of fibrosis in pediatric Cystic Fibrosis associated liver disease (CFALD) have not been developed and existing investigations do not correlate well with presence or severity of disease. Using a Liver biopsy it is difficult to diagnose this condition because of the patchy nature of the disease. Investigators intend to identify hepatic and pancreatic fibrosis in Cystic Fibrosis patients using Elastography and correlate this with their biochemical markers as well as histological findings of patients who have undergone liver biopsy for diagnosis of CFALD.

NCT ID: NCT03001388 Completed - Clinical trials for Cystic Fibrosis Liver Disease

Longitudinal Assessment of Transient Elastography in Cystic Fibrosis

Start date: March 8, 2017
Phase:
Study type: Observational

To determine if transient elastography (TE), when combined with ultrasound (US) pattern characterization can improve the prediction of progression to a nodular pattern on US. To confirm the feasibility of obtaining TE measurements in children with Cystic Fibrosis (CF) To prospectively assess whether TE data are associated with conventional laboratory markers of hepatic fibrosis To determine the variability of TE measurements taken at different sites in the same patient

NCT ID: NCT02979340 Completed - Clinical trials for Pancreatic Insufficiency

MRI to Characterize and Predict CF Liver Disease in PUSH Cohort

Start date: January 27, 2017
Phase:
Study type: Observational

Specific Aim 1. Determine if valid results of non-sedated MRI based assessments of liver stiffness and lipid content can be obtained in more than 90% of children and young adults with cystic fibrosis. Specific Aim 2. Determine hepatic lipid content using the HepaFat sequence and liver stiffness using MRE. Investigators will compare the results obtained by MRI with PUSH study grayscale ultrasounds in CF patients with normal, heterogeneous, homogeneous or nodular (cirrhotic) pattern on ultrasound. Specific Aim 3. Creation of an imaging core lab to centralize evaluation of MR imaging data, allow for remote image upload, electronic data storage, and remote image viewing/interpretation. This infrastructure will be utilized to standardize image post processing. Specific Aim 4. Using the longitudinal PUSH study, determine if MRI based imaging improves discrimination of subjects at risk for progression to advanced CF liver disease (development of cirrhosis) compared to using US imaging alone