Urinary bile alcohol profile in infants with intrahepatic cholestasis: identification of 5ß-cholestane-3a,7a,24,25-tetrol
| Autor: | Shinji Takenaka, Michiko Nakagawa, Taiju Kuramoto, K Iinuma, D Abukawa, Mizuho Une |
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| Rok vydání: | 1999 |
| Předmět: |
Adult
Male medicine.medical_specialty Chromatography Gas medicine.drug_class Urinary system Alcohol Cholestasis Intrahepatic Urine Sensitivity and Specificity digestive system Gastroenterology Gas Chromatography-Mass Spectrometry Bile Acids and Salts chemistry.chemical_compound Liver Function Tests Cholestasis Reference Values Internal medicine Chenodeoxycholic acid Humans Medicine medicine.diagnostic_test Bile acid business.industry Age Factors Infant Newborn Infant General Medicine medicine.disease chemistry Pediatrics Perinatology and Child Health Female Gas chromatography–mass spectrometry business Liver function tests Cholestanols |
| Zdroj: | Acta Paediatrica. 88:1078-1082 |
| ISSN: | 0803-5253 |
| DOI: | 10.1080/08035259950168126 |
| Popis: | Urinary bile acids and bile alcohols were examined in six infants aged between 1 and 6 mo who had intrahepatic cholestasis. Following extraction, hydrolysis and solvolysis, cholanoids were analysed by gas-liquid chromatography and gas-liquid chromatography-mass spectrometry. The relative ratio of the urinary excretion of bile alcohols to bile acids was very low (0.07-0.22) in three patients with mild to severe cholestasis, whereas the urinary excretion of bile alcohols was 2-4 times greater than that of the total bile acids in three patients with slight cholestasis. The urinary bile alcohol spectrum in infants appears to be quite different from that in adults. Although the major bile alcohol was 27-nor-5beta-cholestane-3alpha,7alpha,12alpha,24 ,25-pentol, comprising more than 50% of total urinary bile alcohols in healthy adults, it accounted for only 35% of total urinary bile alcohols in our patients. In addition, bile alcohols carrying chenodeoxycholic acid type nucleus were detected in our patients by comparison of the retention times and mass spectra with those of authentic standards. The presence of 5beta-cholestane-3alpha,7alpha,24,25-tetrol confirmed for the first time in this study may represent an alternative pathway for chenodeoxycholic acid biosynthesis via a "25-hydroxylation pathway" in early life. |
| Databáze: | OpenAIRE |
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