Working… Menu

NPD Measurements in PSC Patients

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT00476814
Recruitment Status : Unknown
Verified October 2007 by Hadassah Medical Organization.
Recruitment status was:  Not yet recruiting
First Posted : May 22, 2007
Last Update Posted : October 31, 2007
Information provided by:
Hadassah Medical Organization

Brief 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

Condition or disease
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.

Layout table for study information
Study Type : Observational
Time Perspective: Prospective
Official Title: Cystic Fibrosis Transmembrane Conductance Regulator Function as Measured by Nasal Potential Difference Measurements in Patients With Primary Sclerosing Cholangitis

Information from the National Library of Medicine

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.

Layout table for eligibility information
Ages Eligible for Study:   10 Years and older   (Child, Adult, Older Adult)
Sexes Eligible for Study:   All

Inclusion Criteria:

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

Exclusion Criteria:

  • Age < 10 years

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT00476814

Layout table for location contacts
Contact: Michael Wilschanski, Dr. 972-2-5844922

Layout table for location information
Hadassah Medical Organization Not yet recruiting
Jerusalem, Israel, 91240
Contact: Michael Wilschanski, Dr    972-2-5844922   
Principal Investigator: Michael Wilschanski, Dr         
Sponsors and Collaborators
Hadassah Medical Organization
Layout table for investigator information
Principal Investigator: Michael Wilschanski, Dr Hadassah Medical Organization

Layout table for additonal information Identifier: NCT00476814     History of Changes
Other Study ID Numbers: will002-HMO-CTIL
First Posted: May 22, 2007    Key Record Dates
Last Update Posted: October 31, 2007
Last Verified: October 2007
Keywords provided by Hadassah Medical Organization:
cystic fibrosis,
nasal potential difference,
primary sclerosing cholangitis
Additional relevant MeSH terms:
Layout table for MeSH terms
Cholangitis, Sclerosing
Digestive System Diseases
Bile Duct Diseases
Biliary Tract Diseases