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

Background/Aims: Urinary 3-oxo-Δ4 bile acids have been detected in infants who ultimately died of liver disease. We used qualitative and quantitative methods to compare urinary 3-oxo-Δ4 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-Δ4 bile acids in the urine of patients and healthy controls. Results: Patients with a deficiency of 3-oxo-Δ4-steroid 5β-reductase and acute hepatic failure exhibited a significantly higher percentage of 3-oxo-Δ4 bile acids in total bile acids in urine than the healthyc ontrols or other patient groups, including those with neonatal cholestasis or biliary atresia (p<0.0001). The urinary 3-oxo-Δ4 bile acids in patients with 3-oxo-Δ4-steroid 5β-reductase deficiency who had a poor prognosis were mainly 7α-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 7α-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.