NPD Measurements in PSC Patients

Primary Sclerosing Cholangitis Clinical Trial

Official title:

Cystic Fibrosis Transmembrane Conductance Regulator Function as Measured by Nasal Potential Difference Measurements in Patients With Primary Sclerosing Cholangitis

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT00476814
• Other study ID #: will002-HMO-CTIL
• Status: Not yet recruiting
• Phase: N/A
• First received: May 21, 2007
• Last updated: October 30, 2007

Study information

• Verified date: October 2007
• Source: Hadassah Medical Organization
• Contact: Michael Wilschanski, Dr.
• Phone: 972-2-5844922
• Email: michaelwil[@]hadassah.org.il
• Is FDA regulated: No
• Health authority: Israel: Ministry of Health
• Study type: Observational

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

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.

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 0
• Gender: Both
• Age group: 10 Years and older

Eligibility

Inclusion Criteria:

• Age > 10 years

• Patients diagnosed as Non-IBD PSC

• Patients diagnosed as IBD PSC

Exclusion Criteria:

• Age < 10 years

Study Design

Time Perspective: Prospective

Related Conditions & MeSH terms

• Cholangitis
• Cholangitis, Sclerosing
• Primary Sclerosing Cholangitis

Locations

Country	Name	City	State
Israel	Hadassah Medical Organization	Jerusalem

Sponsors (1)

Lead Sponsor	Collaborator
Hadassah Medical Organization

Country where clinical trial is conducted

Israel,