Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and longstanding cholestasis

Cystic fibrosis (CF) is an incurable inherited disease characterized by chronic respiratory infection and an inability of the pancreas to produce sufficient enzymes to properly digest food. With improvements in therapies directed against the respiratory infections, patients suffering from CF now survive into their twenties (compared with an average life span of only 14 years in 1969). As more patients live longer, liver and biliary tract problems are seen more frequently. In particular, cholestasis, or impaired bile flow, can produce nutritional deficits. In other conditions involving cholestasis, administration of the bile salt ursodeoxycholic acid (UDC) has been shown to stimulate bile flow and improve nutrition. To determine whether these beneficial effects would also be seen in CF patients, eight patients (aged 10 to 25) were given a six-month course of treatment with UDC. Following the treatment, bile flow was significantly improved. Liver function improved significantly on most indices in all patients; there was a slight deterioration of functional liver cell mass as measured by the galactose elimination test. Nutritional status of the patients increased, and they gained an average of four pounds, with a corresponding increase in muscle mass. Steatorrhea (the passing of fatty stools) was not affected by UDC treatment. (Consumer Summary produced by Reliance Medical Information, Inc.)