Cystic Fibrosis, Gastrointestinal Diseases, Cholestasis
Conditions
Keywords
cardiovascular and respiratory diseases, cholestasis, cystic fibrosis, gastrointestinal disorders, genetic diseases and dysmorphic syndromes, rare disease
Brief summary
OBJECTIVES: I. Compare the bioavailability of polymer-coated and buffered ursodiol (ursodeoxycholic acid) to unmodified ursodiol in patients with cystic fibrosis-associated liver disease or chronic cholestatic liver disease. II. Compare the differences in pruritus, weight gain, and liver function for both treatments.
Detailed description
PROTOCOL OUTLINE: Patients are sequentially treated with 2 formulations of ursodeoxycholic acid: unmodified ursodiol (Actigall) and buffered, enteric-coated ursodiol (Ursocarb). There is a 24-hour washout between each 4-week course of therapy.
Interventions
DRUGursodiol
Sponsors
Children's Hospital Medical Center, Cincinnati
National Center for Research Resources (NCRR)
Study design
Primary purpose
TREATMENT
Eligibility
Sex/Gender
ALL
Age
4 Months to No maximum
Healthy volunteers
No
Inclusion criteria
PROTOCOL ENTRY CRITERIA: --Disease Characteristics-- Chronic cholestatic liver disease Cystic fibrosis-associated liver disease --Prior/Concurrent Therapy-- Usual and customary diet maintained throughout study, e.g., medium-chain triglyceride oil --Patient Characteristics-- Pulmonary: No serious respiratory deficiency No acute illness No inability to swallow No fertile women
Countries
United States
Outcome results
None listed