NPD Measurements in PSC Patients

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

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

Study Overview

• Status: Unknown
• Conditions: Primary Sclerosing Cholangitis

Detailed 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 Type: Observational

Contacts and Locations

Study Locations

• Israel
  • Jerusalem, Israel, 91240
    • Hadassah Medical Organization

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 10 years and older (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: N/A
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Age > 10 years
• Patients diagnosed as Non-IBD PSC
• Patients diagnosed as IBD PSC

Exclusion Criteria:

• Age < 10 years

Study Plan

How is the study designed?

Collaborators and Investigators

• Sponsor: Hadassah Medical Organization
• Investigators: Principal Investigator: Michael Wilschanski, Dr, Hadassah Medical Organization

Study record dates

Study Registration Dates

• First Submitted: May 21, 2007
• First Submitted That Met QC Criteria: May 21, 2007
• First Posted (Estimate): May 22, 2007

Study Record Updates

• Last Update Posted (Estimate): October 31, 2007
• Last Update Submitted That Met QC Criteria: October 30, 2007
• Last Verified: October 1, 2007

More Information

Terms related to this study

• Keywords: cystic fibrosis,; primary sclerosing cholangitis; nasal potential difference,
• Additional Relevant MeSH Terms: Digestive System Diseases; Biliary Tract Diseases; Bile Duct Diseases; Cholangitis; Cholangitis, Sclerosing
• Other Study ID Numbers: will002-HMO-CTIL