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NPD Measurements in PSC Patients

The recruitment status of this study is unknown. The completion date has passed and the status has not been verified in more than two years.
Verified October 2007 by Hadassah Medical Organization.
Recruitment status was:  Not yet recruiting
Information provided by:
Hadassah Medical Organization Identifier:
First received: May 21, 2007
Last updated: October 30, 2007
Last verified: October 2007
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

Primary Sclerosing Cholangitis

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

Resource links provided by NLM:

Further study details as provided by Hadassah Medical Organization:

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.


Ages Eligible for Study:   10 Years and older   (Child, Adult, Senior)
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
  Contacts and Locations
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, see Learn About Clinical Studies.

Please refer to this study by its identifier: NCT00476814

Contact: Michael Wilschanski, Dr. 972-2-5844922

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
Principal Investigator: Michael Wilschanski, Dr Hadassah Medical Organization
  More Information Identifier: NCT00476814     History of Changes
Other Study ID Numbers: will002-HMO-CTIL
Study First Received: May 21, 2007
Last Updated: October 30, 2007

Keywords provided by Hadassah Medical Organization:
cystic fibrosis,
nasal potential difference,
primary sclerosing cholangitis

Additional relevant MeSH terms:
Cystic Fibrosis
Cholangitis, Sclerosing
Pancreatic Diseases
Digestive System Diseases
Lung Diseases
Respiratory Tract Diseases
Genetic Diseases, Inborn
Infant, Newborn, Diseases
Bile Duct Diseases
Biliary Tract Diseases processed this record on September 21, 2017