Study of Tauroursodeoxycholic Acid for Hepatobiliary Disease in Cystic Fibrosis

PROTOCOL ENTRY CRITERIA: --Disease Characteristics-- Cystic fibrosis-associated liver disease, defined by at least one of the following criteria: (1) Documented increase in serum concentrations of any of the liver enzymes (at least once in the preceding year) ALT at least twice normal AST at least 1.5 times normal Alkaline phosphatase at least 1.5 times normal GGT at least 1.5 times normal (2) Persistent hepatomegaly of more than 6 months duration defined by percussed liver span greater than 1 SEM for age (3) Splenomegaly, defined as a palpable spleen greater than 2.0 cm below the left costal margin (4) Abnormalities of ultrasound scan (increased size, dishomogeneous echogenicity, nodular liver, irregular margins, splenomegaly) within 6 months prior to study entry Patients enrolled in the first part of the study (objective I) are eligible to participate in the second part (objective II) --Prior/Concurrent Therapy-- At least 3 months since prior ursodiol At least 3 months since treatment with drug with choleretic properties or effects that influence bile acid metabolism --Patient Characteristics-- Hepatic: No decompensated cirrhosis No hepatic neoplasm or cholelithiasis Pulmonary: No significantly impaired pulmonary function with FEV1 less than 50% Other: At least 15 kg body weight No severely compromised clinical or nutritional state