J Pediatr Gastroenterol Nutr. 1994 Aug;19(2):198-203.

Cross-over design.

Single centre. Italy. Inclusion criteria: 51 participants recruited, age range 8 - 32 years, median 14 years. 10 participants had abnormal liver enzymes and of these 2 had cirrhosis and varices and 8 had enlarged liver and fibrosis at ultrasound. 42 participants completed the study, 9 dropouts (5 in UDCA group, 4 in UDCA plus taurine group) not followed-up. diagnosis of CF as documented by raised sweat chloride values and clinical symptoms; evidence of malnutrition as documented by body mass percentile less than or equal to 90%; age over 6 years; good compliance with previous conventional treatment; no previous UDCA treatment.

UDCA: 12 mg/kg/day for 6 months (see Methods for study design). Taurine: 18 - 22 mg/kg/day for 6 months. Control: placebo (glucose) for 6 months.

weight, body mass percentile, triceps skinfold thickness, mid-arm-muscle circumference, albumin, aspartate transferase, alanine transferase, alkaline phosphatase, gamma glutamate transferase were measured at 6 months.

Nine patients dropped out before concluding the study. Liver function tests, nutritional status, and coefficients of fat absorption were determined at entry and after each 6 months of placebo or treatment. Nutritional status and fat absorption were not significantly modified by either treatment. Liver function tests improved after ursodeoxycholic acid administration only in patients with concomitant chronic liver disease.

6 months of therapy with a medium dose of ursodeoxycholic acid, either alone or with taurine, does not improve the nutritional status of young malnourished CF patients. Higher doses given for longer periods might be worth investigating.