Urinary 7alpha-hydroxy-3-oxochol-4-en-24-oic and 3-oxochola-4,6-dien-24-oic acids in infants with cholestasis.
OBJECTIVE
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.
Kimura
A
A
Department of Pediatrics and Child Health, Kurume University School of Medicine, Fukuoka, Japan.
Suzuki
M
M
Murai
T
T
Kurosawa
T
T
Tohma
M
M
Sata
M
M
Inoue
T
T
Hoshiyama
A
A
Nakashima
E
E
Yamashita
Y
Y
Fujisawa
T
T
Kato
H
H
D000293:Adolescent; D000328:Adult; D000368:Aged; D001656:Biliary Atresia / Q000150:complications / Q000652:urine*; D016022:Case-Control Studies; D002635:Chenodeoxycholic Acid / Q000031:analogs & derivatives* / Q000652:urine; D002648:Child; D002675:Child, Preschool; D002779:Cholestasis / Q000209:etiology / Q000652:urine*; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000150:complications / Q000652:urine*; D008297:Male; D008875:Middle Aged; D010088:Oxidoreductases / Q000172:deficiency*
D002635:Chenodeoxycholic Acid; C003019:3-oxo-7-hydroxychol-4-enoic acid; D010088:Oxidoreductases; C021936:3-oxo-5 beta-steroid delta 4-dehydrogenase