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Urinary bile alcohol profile in infants with intrahepatic cholestasis: identification of

M Nakagawa1, M Une, S Takenaka

  • 1Department of Pediatrics, Sendai Tokushukai Hospital, Japan.

Acta Paediatrica (Oslo, Norway : 1992)
|November 24, 1999
PubMed

Insights

Infants with cholestasis show altered urinary bile alcohol to bile acid ratios. Unique bile alcohol profiles in infants suggest alternative biosynthesis pathways, differing from adults.

Area of Science:

  • Biochemistry
  • Pediatric Gastroenterology
  • Metabolomics

Background:

  • Intrahepatic cholestasis in infants disrupts normal bile acid metabolism.
  • Understanding bile acid and bile alcohol profiles is crucial for diagnosing and managing pediatric liver conditions.

Purpose of the Study:

  • To analyze urinary bile acids and bile alcohols in infants with intrahepatic cholestasis.
  • To compare infant bile metabolite profiles with those of healthy adults.
  • To investigate potential alternative bile acid biosynthesis pathways in early life.

Main Methods:

  • Analysis of urinary cholanoids from six infants using gas-liquid chromatography and gas-liquid chromatography-mass spectrometry.
  • Extraction, hydrolysis, and solvolysis of samples.
  • Comparison with authentic standards for identification and quantification.

Main Results:

  • Urinary bile alcohol to bile acid ratios varied significantly based on cholestasis severity.
  • Infant urinary bile alcohol spectrum differed from adults, with a lower proportion of 27-nor-5beta-cholestane-3alpha,7alpha,12alpha,24,25-pentol.
  • Detection of bile alcohols with a chenodeoxycholic acid nucleus, including 5beta-cholestane-3alpha,7alpha,24,25-tetrol, suggesting a novel 25-hydroxylation pathway.

Conclusions:

  • Urinary bile acid and bile alcohol profiles are altered in infants with intrahepatic cholestasis.
  • Infant bile metabolism exhibits distinct characteristics compared to adults.
  • The findings suggest a potential alternative pathway for chenodeoxycholic acid biosynthesis in early life.

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