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
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.
PROTOCOL ENTRY CRITERIA:
Chronic cholestatic liver disease
Cystic fibrosis-associated liver disease
Usual and customary diet maintained throughout study, e.g., medium-chain triglyceride oil
Pulmonary: No serious respiratory deficiency
No acute illness
No inability to swallow
No fertile women