Urinary 7alpha-hydroxy-3-oxochol-4-en-24-oic and 3-oxochola-4,6-dien-24-oic acids in infants with cholestasis

  • J Hepatol. 1998 Feb;28(2):270-9. doi: 10.1016/0168-8278(88)80014-x.
A Kimura  1 ,  M Suzuki ,  T Murai ,  T Kurosawa ,  M Tohma ,  M Sata ,  T Inoue ,  A Hoshiyama ,  E Nakashima ,  Y Yamashita ,  T Fujisawa ,  H Kato
Affiliations
  • 1. Department of Pediatrics and Child Health, Kurume University School of Medicine, Fukuoka, Japan.
PMID: 9514540 DOI: 10.1016/0168-8278(88)80014-x
Abstract

Background/aims: Urinary 3-oxo-delta4 Bile Acids have been detected in infants who ultimately died of Liver Disease. We used qualitative and quantitative methods to compare urinary 3-oxo-delta4 Bile Acids in Liver Disease, determining their composition and evaluating the prognostic implication in patients of various ages with various liver diseases.

Methods: Gas chromatography-mass spectrometry was used to measure 3-oxo-delta4 Bile Acids in the urine of patients and healthy controls.

Results: Patients with a deficiency of 3-oxo-delta4-steroid 5beta-reductase and acute hepatic failure exhibited a significantly higher percentage of 3-oxo-delta4 Bile Acids in total Bile Acids in urine than the healthy controls or other patient groups, including those with neonatal cholestasis or biliary atresia (p<0.0001). The urinary 3-oxo-delta4 Bile Acids in patients with 3-oxo-delta4-steroid 5beta-reductase deficiency who had a poor prognosis were mainly 7alpha-hydroxy-3-oxochol-4-en-24-oic acid and 3-oxochola-4,6-dien-24-oic acid.

Conclusions: Our results indicate that an increase in the 7alpha-hydroxy-3-oxochol-4-en-24-oic acid and 3-oxochola-4,6-dien-24-oic acid in the urine of patients with hepatobiliary disease indicates a poor prognosis.

Products