Ursodeoxycholic acid improves cholestasis in infants with cystic fibrosis.
OBJECTIVE
To describe two infants with cholestatic jaundice treated with ursodeoxycholic acid (UDCA).


METHODS
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.


CONCLUSIONS
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.
Scher
H
H
Department of Pediatrics, College of Medicine, University of Iowa, Iowa City 52242, USA.
Bishop
W P
WP
McCray
P B
PB
D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D002779:Cholestasis / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008111:Liver Function Tests; D008297:Male; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid