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

Background / Aims: Urinary 3-oxo-Delta(4) bile acids have been detected in infants wh ultimately died of liver disease. We used qualitative and quantitative methods to compare urinary 3-oxo-Delta(4) 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-Delta(4) bile acids in the urine of patients and healthy controls. Results: Patients with a deficiency of 3-oxo-Delta(4)-steroid 5 beta-reductase and acute hepatic failure exhibited a significantly higher percentage of 3-oxo-Delta(4) 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-Delta(4) bile acids in patients 3-oxo-Delta(4)-steroid 5 beta-reductase deficiency who had a poor prognosis were mainly 7 alpha-hydroxy-3-oxochol-4-en-25-oic acid and 3-oxochola-4,6-dien-24-oic acid. Conclusions: Our results indicate that an increase in the 7 alpha-hydroxy-3-oxochol-4-en-24-oic acid and 3-ox-ochola-4,6-dien-24-oic acid in the urine of patients with hepatobiliary disease indicates a poor prognosis.