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Ursodeoxycholic acid improves cholestasis in infants with cystic fibrosis

H Scher1, W P Bishop, P B McCray

  • 1Department of Pediatrics, College of Medicine, University of Iowa, Iowa City 52242, USA.

The Annals of Pharmacotherapy
|September 20, 1997
PubMed

Insights

Ursodeoxycholic acid (UDCA) effectively treated cholestatic jaundice in two infants with cystic fibrosis-associated liver disease. This approach offers a cost-effective solution for pediatric hepatobiliary conditions.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Genetic Disorders

Background:

  • Cystic Fibrosis (CF) can lead to hepatobiliary complications, including cholestatic jaundice, in infants.
  • Early identification of liver dysfunction is crucial for managing CF-associated liver disease.

Observation:

  • Two infants diagnosed with CF-associated cholestatic jaundice within the first six weeks of life were studied.
  • These infants presented with elevated liver enzymes and jaundice.

Findings:

  • Treatment with ursodeoxycholic acid (UDCA) at doses of 20-40 mg/kg/d resulted in improved liver function biochemical indices.
  • This marks the first reported use of UDCA for cholestatic jaundice in infants with CF.

Implications:

  • UDCA demonstrates potential as a safe and effective therapeutic agent for infants and children with CF-associated hepatobiliary disease.
  • Higher UDCA doses may be necessary in pediatric populations due to absorption and enterohepatic circulation factors.
  • UDCA represents a potentially cost-effective treatment strategy for this specific pediatric population.
Abstract

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