Quantitative changes of serum lipoprotein-X after cholestyramine administration in infants with cholestatic biliary tract and liver disease
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Lipoprotein-X (LP-X) in infants with cholestatic jaundice indicates severe cholestasis. A decrease in LP-X after cholestyramine administration helps differentiate biliary atresia from other liver diseases.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Biochemistry
Background:
- Cholestatic jaundice in infants presents diagnostic challenges, often requiring differentiation between intrahepatic and extrahepatic causes.
- Lipoprotein-X (LP-X) is an abnormal lipoprotein found in certain cholestatic conditions.
Purpose of the Study:
- To evaluate the utility of serum Lipoprotein-X (LP-X) levels in differentiating biliary atresia from other causes of cholestatic jaundice in infants.
- To assess the response of LP-X to cholestyramine administration as a diagnostic tool.
Main Methods:
- Serum LP-X levels were measured in 55 infants with persistent cholestatic jaundice before and after cholestyramine administration.
- Infants were categorized into groups with biliary atresia and intrahepatic cholestasis.
Main Results:
- In infants with biliary atresia (n=27), LP-X levels did not significantly decrease after cholestyramine, often increasing.
- In infants with intrahepatic cholestasis (n=28), LP-X levels decreased significantly (average 78%) after cholestyramine treatment.
- The absence of LP-X before or after treatment was noted in some infants, and its presence indicated severe cholestasis.
Conclusions:
- Serum LP-X is a marker of severe cholestasis in infants but not exclusively diagnostic of biliary atresia.
- Cholestyramine administration effectively differentiates biliary atresia from other infantile cholestatic diseases by observing LP-X level changes.
- A marked decrease in LP-X following cholestyramine treatment suggests patent extrahepatic bile ducts, whereas its persistence or increase is indicative of biliary atresia.
Abstract:
Lipoprotein-X (LP-X) was determined before and after the administration of cholestyramine in fifty-five infants with persistent cholestatic jaundice to differentiate between intra- and extrahepatic disease. In twenty-seven infants with biliary atresia, serum LP-X prior to cholestyramine ranged from 0.87 to 11.42 g/l (mean: 3.43 g/l; the average concentration was significantly lower (P less than 0.001) in males. After cholestyramine, LP-X rose in twenty-three, remained the same in two, and decreased slightly in two infants. Serum LP-X was present in twenty of the twenty-eight infants with intrahepatic cholestasis prior to cholestyramine in concentrations from 0.84 to 14.19 g/l (mean: 3.13 g/l). After cholestyramine, LP-X decreased in all by an average of 78% (P less than 0.005). The other eight infants did not have LP-X before or after cholestyramine. This study shows that LP-X in the serum of infants with cholestatic jaundice indicates severe cholestasis, but is not itself diagnostic of biliary atresia. The differentiation of biliary atresia from other diseases is readily achieved, as the administration of cholestyramine for 2-3 weeks causes a marked decrease of serum LP-X in patients with patent extrahepatic bile ducts. The absence of serum LP-X excludes biliary atresia.