Publications

Total Serum Bilirubin Predicts Fat-Soluble Vitamin Deficiency Better Than Serum Bile Acids in Infants with Biliary Atresia.

Objective: Fat soluble vitamin (FSV) deficiency is a well-recognized consequence of cholestatic liver disease and reduced intestinal intraluminal bile acids. We hypothesized that serum bile acids (SBA) would predict biochemical FSV deficiency better than serum total bilirubin level (TB) in infants with biliary atresia. Methods: Infants enrolled in the Trial of Corticosteroid Therapy in Infants with Biliary Atresia (START)after hepatoportoenterostomywere the subjects of this investigation. Infants received standardized FSV supplementation and monitoring of TB, SBA and vitamin levels at 1, 3 and 6 months. A logistic regression model was used with the binary indicator variable insufficient/sufficient as the outcome variable. Linear and non-parametric correlations were made between specific vitamin measurement levels and either TB or SBA.
Results: The degree of correlation for any particular vitamin at a specific time point was higher with TB than SBA (higher for TB in 31 circumstances versus 3 circumstances for SBA). Receiver operating characteristic (ROC) shows that TB performed better than SBA (AUC 0.998 vs. 0.821). Including both TB and SBA did not perform better than TB alone (AUC 0.998).
Conclusion: We found that TB was a better predictor of FSV deficiency than SBA in infants with biliary atresia. The role of SBA as a surrogate marker of FSV deficiency in other cholestatic liver diseases, such as PFIC, alpha-one antitrypsin deficiency and Alagille syndrome where the pathophysiology is dominated by intrahepatic cholestasis, warrants further study.