Abstract
Lithocholic acid (LCA) is a hepatotoxic compound. Fetal LCA might play a role in the pathogenesis of neonatal cholestasis/EHBA. Recent results show that fetal liver has capacity for hydroxylations of LCA (Ped. Res. 21:99, 1987).
If the pathogenesis of EHBA involves increased fetal levels of LCA, the condition could be due to impaired fetal liver metabolism of LCA. This should lead to increased levels of LCA at birth.
LCA, cholic acid (CA) and chenodeoxycholic acid (CDCA) were quantitated in stored dried blood from six newborn infants with EHBA and fourteen controls. The blood was collected at neonatal metabolic screening. The bile acids were quantitated by gas chromatography-mass spectrometry using selected ion monitoring.
Results: Mean blood levels (±SD) of LCA were 0.11±0.10 μM in the infants with EHBA and 0.08±0.06 μM in the controls. Mean blood levels (±SD) of CA and CDCA were 15.6±8.7 μM and 7.4±6.1 μM in the EHBA infants and 1.7±1.2 μM and 1.8±1.5 μM in the controls.
Conclusion: The low blood levels of LCA found indicate a normal metabolism of this bile acid in fetuses with EHBA. The increased levels of CA and CDCA in the infants with this disease can be due to cholestasis.
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Gustafsson, J., Alvelius, G., Björkhem, I. et al. BILE ACID METABOLISM IN EXTRAHEPATIC BILIARY ATRESIA (EHBA): LITHOCHOLIC ACID IN STORED DRIED BLOOD COLLECTED AT NEONATAL SCREENING. Pediatr Res 32, 625 (1992). https://doi.org/10.1203/00006450-199211000-00122
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DOI: https://doi.org/10.1203/00006450-199211000-00122