- ICH GCP
- US Clinical Trials Registry
- Clinical Trial NCT00476814
NPD Measurements in PSC Patients
Cystic Fibrosis Transmembrane Conductance Regulator Function as Measured by Nasal Potential Difference Measurements in Patients With Primary Sclerosing Cholangitis
Study Overview
Status
Conditions
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
Contacts and Locations
Study Contact
- Name: Michael Wilschanski, Dr.
- Phone Number: 972-2-5844922
- Email: michaelwil@hadassah.org.il
Study Locations
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Jerusalem, Israel, 91240
- Hadassah Medical Organization
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Contact:
- Michael Wilschanski, Dr
- Phone Number: 972-2-5844922
- Email: michaelwil@hadassah.org.il
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Principal Investigator:
- Michael Wilschanski, Dr
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Participation Criteria
Eligibility Criteria
Ages Eligible for Study
Accepts Healthy Volunteers
Genders Eligible for Study
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?
Design Details
Collaborators and Investigators
Sponsor
Investigators
- Principal Investigator: Michael Wilschanski, Dr, Hadassah Medical Organization
Study record dates
Study Registration Dates
First Submitted
First Submitted That Met QC Criteria
First Posted (Estimate)
Study Record Updates
Last Update Posted (Estimate)
Last Update Submitted That Met QC Criteria
Last Verified
More Information
Terms related to this study
Additional Relevant MeSH Terms
Other Study ID Numbers
- will002-HMO-CTIL
This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.
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