NPD Measurements in PSC Patients

The recruitment status of this study is unknown because the information has not been verified recently.
Verified October 2007 by Hadassah Medical Organization.
Recruitment status was  Not yet recruiting
Sponsor:
Information provided by:
Hadassah Medical Organization
ClinicalTrials.gov Identifier:
NCT00476814
First received: May 21, 2007
Last updated: October 30, 2007
Last verified: October 2007

May 21, 2007
October 30, 2007
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Complete list of historical versions of study NCT00476814 on ClinicalTrials.gov Archive Site
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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

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.

Observational
Time Perspective: Prospective
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Primary Sclerosing Cholangitis
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*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Not yet recruiting
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Inclusion Criteria:

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

Exclusion Criteria:

  • Age < 10 years
Both
10 Years and older
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Contact: Michael Wilschanski, Dr. 972-2-5844922 michaelwil@hadassah.org.il
Israel
 
NCT00476814
will002-HMO-CTIL
No
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Hadassah Medical Organization
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Principal Investigator: Michael Wilschanski, Dr Hadassah Medical Organization
Hadassah Medical Organization
October 2007

ICMJE     Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP