Ursodeoxycholic Acid Improves Cholestasis in Infants with Cystic Fibrosis

Objective: To describe two infants with cholestatic jaundice treated with ursodeoxycholic acid (UDCA).

Case Summary: Two infants with cystic fibrosis (CF)-associated hepatobiliary disease, manifesting as cholestatic jaundice and elevated liver enzymes within the first 6 weeks of life, had improved biochemical indices of liver function following treatment with UDCA 20-40 mg/kg/d.

Discussion: To our knowledge, this is the first report of UDCA treatment in infants with CF-associated cholestatic jaundice. Infants and children require treatment with increased doses of UDCA to compensate for reduced intestinal absorption of bile acid and immaturity of the enterohepatic circulation.

Conclusions: UDCA appears to be a cost-effective treatment for CF-associated hepatobiliary disease in infants and children.