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  • 1
    ISSN: 1437-9813
    Schlagwort(e): Congenital biliary atresia ; 1β-hydroxy bile acids ; Fetal bile acids
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The 1β-hydroxy bile acids have been said to be fetal bile acids. These bile acids were evaluated in eight patients with congenital biliary atresia (CBA) using gas chromatography-mass spectrometry. At the time of operation 1β,3α,7α,12α-tetrahydroxy-5β-cholan-24-oic acid (CA-1β-ol) and 1β,3α,7α-trihydroxy-5β-cholan-24-oic acid (CDCA-1β-ol) were 0.46 ± 34 μg/ml and 0.72 ± 0.45 μg/ml, respectively, which was significantly higher than in the control group. The percentage CA-1β-ol of total bile acids showed a tendency to decrease as age advanced. The grade of hepatic fibrosis ranged from F2 to F3 and the values and percentages of CA-1β-ol and CDCA-1β-ol were relatively higher in F2 than F3 patients. The percentage of total bile acids gradually increased in patients without sufficient bile flow but fell sharply after Kasai's procedure in the patients with sufficient bile flow. It appears that fetal bile acids are produced in the livers of CBA patients in the same way as in fetal liver, and that production continues in patients without good bile secretion even after Kasai's procedure. These results suggest that these hydroxylases are reactivated in CBA patients.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    ISSN: 1432-1076
    Schlagwort(e): Key words 3-Oxo-Δ4-steroid 5β-reductase deficiency ; 3-oxo-Δ4 bile acid ; Immunoblot analysis
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract A 3-oxo-Δ4-steroid 5β-reductase (5β-reductase) deficiency is difficult to diagnose because severe liver damage can result in a similar pattern of metabolite excretion. We investigated the usefulness of immunoblot analysis for diagnosis of 5β-reductase deficiency and quantitatively analysed urinary bile acids by gas chromatography-mass spectrometry in a 5-month-old Japanese boy with severe neonatal cholestasis associated with hypertyrosinaemia. A liver sample was examined by immunoblot analysis using monoclonal antibodies against 5β-reductase. Urinary 3-oxo-Δ4 bile acids accounted for 88.3% of total bile acids, 5α-bile acids for 0.9%, and primary bile acids for 9.1%. Immunoblot analysis of the liver tissue showed an indistinct band of 5β-reductase. Conclusions These findings suggest that this patient had a secondary 5β-reductase deficiency due to severe liver damage, even though 3-oxo-Δ4 bile acids constituted more than 70% of total urinary bile acids. However, the patient may possibly have had an inherited 5β-reductase deficiency.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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