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Serum bile acid patterns determined by an enzymatic method and high-performance liquid chromatography in young
Insights
Infant cholestasis significantly elevates total serum bile acids. Intrahepatic cholestasis shows altered taurine-conjugated bile acid levels, suggesting specific metabolic changes in infants with liver disease.
Area of Science:
- Biochemistry
- Pediatric Gastroenterology
- Hepatology
Background:
- Cholestasis in infants, including idiopathic neonatal hepatitis syndrome and extra-hepatic biliary atresia, presents diagnostic challenges.
- Bile acid metabolism is crucial for liver function and can be altered in pediatric cholestatic conditions.
Purpose of the Study:
- To investigate serum bile acid profiles in infants with intrahepatic versus extra-hepatic cholestasis.
- To identify potential biomarkers for differentiating types of infant cholestasis based on bile acid composition.
Main Methods:
- Enzymatic procedures and high-performance liquid chromatography (HPLC) were used to analyze serum unconjugated and conjugated bile acids.
- Serum samples from infants with intrahepatic cholestasis (n=8), extra-hepatic cholestasis (n=8), and healthy controls were compared.
Main Results:
- Total serum bile acid levels were markedly increased in both cholestatic groups compared to controls.
- Unconjugated bile acid levels remained largely unchanged across all groups.
- While glycine- to taurine-conjugated bile acid ratios did not differ significantly between cholestatic groups, half of the intrahepatic cholestasis patients exhibited very low taurine-conjugated cholate and chenodeoxycholate levels.
Conclusions:
- Elevated total serum bile acids are characteristic of infant cholestasis.
- Specific alterations in taurine-conjugated bile acids may indicate intrahepatic cholestasis, suggesting a distinct metabolic pathway involvement.
- Bile acid profiling offers potential for diagnosing and understanding infant cholestatic liver diseases.
Abstract:
Serum unconjugated and conjugated bile acids in young infants with intrahepatic cholestasis (idiopathic neonatal hepatitis syndrome, n = 8) or extra-hepatic cholestasis (preoperative extrahepatic biliary atresia, n = 8) were examined by an enzymatic procedure and high-performance liquid chromatography. In comparison with the mean level of total serum bile acid of controls having no liver or gastrointestinal diseases, those of each group markedly increased (15.6 +/- 5.1 vs. 120.9 +/- 64.0 and 161.8 +/- 54.2 nmol/ml), but those of unconjugated bile acid were almost unchanged (1.4 +/- 0.5 vs. 1.0 +/- 0.6 and 0.6 +/- 0.2 nmol/ml). The ratios of cholate to chenodeoxycholate and glycine- to taurine-conjugated bile acids (G/T) were not significantly different between the groups of intrahepatic and extrahepatic cholestasis. However, in the patients with intrahepatic cholestasis, the G/T ratio varied greatly and the quantitative determination of individual conjugated bile acids in serum revealed that a half of the patients examined had very low levels of taurine-conjugated cholate and chenodeoxycholate, suggesting a bile acid metabolism alternation specific for underlying intrahepatic cholestasis.