Status:

ACTIVE_NOT_RECRUITING

Genetic Modifiers of Cystic Fibrosis (CF) Liver Disease

Lead Sponsor:

University of North Carolina, Chapel Hill

Collaborating Sponsors:

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

Cystic Fibrosis Foundation

Conditions:

Cystic Fibrosis

Liver Disease

Eligibility:

All Genders

2+ years

Brief Summary

This study examines "modifier genes" that may play a role in the development of CF liver disease. Modifier genes are genes, other than the CF gene (CFTR), which may directly or indirectly have an affe...

Detailed Description

The clinical heterogeneity in cystic fibrosis (CF) is only partly explained by mutations in the CFTR gene. Most CF patients have evidence of liver dysfunction and focal biliary cirrhosis (fibrosis), a...

Eligibility Criteria

Inclusion

  • Volunteers with CF, regardless of genotype and age, who have "severe" liver disease with portal hypertension (big spleen, i.e. "splenomegaly" and/or presence of enlarged vessels in the esophagus, i.e. esophageal varices) may be eligible to participate. This would include any person who has been evaluated for a liver transplant, or has already received a liver transplant.

Exclusion

  • History of Alcohol Abuse
  • History of Biliary Atresia
  • History of Clinically Significant Hepatitis Infection
  • History of Hepatic Vein Thrombosis
  • History of Liver Cancer
  • History of Portal Vein Thrombosis
  • History of Clinically Significant use of total parenteral nutrition (TPN)
  • History of Wilson's Disease

Key Trial Info

Start Date :

March 1 2004

Trial Type :

OBSERVATIONAL

Allocation :

ACTUAL

End Date :

July 1 2027

Estimated Enrollment :

154 Patients enrolled

Trial Details

Trial ID

NCT00804583

Start Date

March 1 2004

End Date

July 1 2027

Last Update

September 23 2025

Active Locations (1)

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1

The University of North Carolina at Chapel Hill

Chapel Hill, North Carolina, United States, 27599