H Scher1, W P Bishop, P B McCray. 1. Department of Pediatrics, College of Medicine, University of Iowa, Iowa City 52242, USA.
Abstract
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.
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.
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