NPD Measurements in PSC Patients

Status Not yet recruiting

Clinical Trial Summary

Primary Sclerosing Cholangitis (PSC) is a chronic disorder of the liver causing jaundice and liver damage. When Cystic Fibrosis affects the liver, the damaged liver looks like the liver in PSC. This study is designed to answer the question whether isolated PSC may be a form of CF only in the liver

Clinical Trial Description

Cystic Fibrosis (CF) is a progressive autosomal recessive disorder caused by defects in the cystic fibrosis transmembrane conductance regulator gene (CFTR). CFTR mutations cause loss or impairment of CFTR-mediated ion transport across epithelial cell membranes. CF affects many organs including the respiratory tract, pancreas, intestine, liver and the male reproductive tract. Liver disease occurs less frequently than pulmonary disease. CFTR is expressed in the bile duct epithelial cells and is responsible for the hydration of biliary secretions.

CF was until recently, thought to be a multi-organ disease. However, there are now recognized non-classical presentations of CF involving a few organs only and now there is well established data on single -organ involvement including Congenital Absence of the Vas Deferens and recurrent pancreatitis.

CF and PSC have several features in common. They both affect intrahepatic bile ducts by inspisated biliary secretions, chronic inflammation and fibrosis. Is PSC the "single-organ presentation" of CF? Patients with PSC will undergo testing of the CF protein channel by nasal potential testing. This test is performed by insering a thin plastic tube 2 to 3 cms into the nostril and chloride transport can be measured. This test may shows that the patient has an abnormality in the CF protein channel. ;

Study Design

Time Perspective: Prospective

Related Conditions & MeSH terms

Clinical Trial Details

NCT number	NCT00476814
Study type	Observational
Source	Hadassah Medical Organization
Contact	Michael Wilschanski, Dr.
Phone	972-2-5844922
Email	michaelwil@hadassah.org.il
Status	Not yet recruiting
Phase	N/A

View Details

See also
Status	Clinical Trial	Phase
Completed	`NCT02239211` - A Trial of BTT1023 in Patients With Primary Sclerosing Cholangitis	Phase 2
Withdrawn	`NCT03216876` - A Study Of Ursolic Acid For Primary Sclerosing Cholangitis	Phase 1
Recruiting	`NCT02605213` - Effect and Safety of Oral Vancomycin in Primary Sclerosing Cholangitis Patients	Phase 4
Recruiting	`NCT01688024` - Mitomycin C Therapy for Patients With Primary Sclerosing Cholangitis	Phase 2
Completed	`NCT03041662` - Surveillance Study for Early Detection of Cholangiocarcinoma (CCA) in Primary Sclerosing Cholangitis (PSC)
Completed	`NCT05866809` - Evaluation of the Safety and Efficacy of HK-660S in Patients With Primary Sclerosing Cholangitis	Phase 2
Recruiting	`NCT05618145` - National Database on Primary Sclerosing Cholangitis (PSC)
Active, not recruiting	`NCT02446665` - Disease Status in Primary Sclerosing Cholangitis by Elastography	N/A
Completed	`NCT02247934` - Development of a Patient-Reported Outcome Measure to Assess Symptoms in Patients With Primary Sclerosing Cholangitis (PSC)	N/A
Terminated	`NCT01142323` - Pilot Study of Fenofibrate for PSC	Phase 1/Phase 2
Completed	`NCT01088607` - Safety and Efficacy Study of Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis	Phase 1
Terminated	`NCT04060147` - Safety and Tolerability of Cilofexor in Participants With Primary Sclerosing Cholangitis (PSC) and Compensated Cirrhosis	Phase 1
Recruiting	`NCT04133792` - Effect of Simvastatin on the Prognosis of Primary Primary Sclerosing Cholangitis (PSC)	Phase 3
Active, not recruiting	`NCT04595825` - CM-101 in PSC Patients -The SPRING Study	Phase 2
Recruiting	`NCT03183570` - Detection of Integrin avb6 in IPF, PSC, and COVID19 Using PET/CT	Early Phase 1
Completed	`NCT02943460` - Study to Evaluate the Safety, Tolerability, and Efficacy of Cilofexor in Adults With Primary Sclerosing Cholangitis Without Cirrhosis	Phase 2
Completed	`NCT00951327` - Cholangioscopy Using Narrow Band Imaging (NBI) in Patients With Primary Sclerosing Cholangitis (PSC) Undergoing Endoscopic Retrograde Cholangiopancreatogram (ERCP)	N/A
Completed	`NCT04024813` - A Study to Evaluate the Safety, and Tolerability, and Efficacy of Seladelpar in Patients With PSC	Phase 2
Recruiting	`NCT05912387` - Statin Therapy in Primary Sclerosing Cholangitis (PSC): a Multi-omics Study	Early Phase 1
Completed	`NCT02884557` - NKT Role in the Regulation of the Inflammatory Bowel Disease	N/A