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.