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J Pediatr Gastroenterol Nutr. 2014 Dec;59(6):702-7. doi: 10.1097/MPG.0000000000000547.

Total serum bilirubin predicts fat-soluble vitamin deficiency better than serum bile acids in infants with biliary atresia.

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*Department of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA †Department of Surgery and Department of Biostatistics, University of Michigan, Ann Arbor, MI ‡Department of Pediatrics, Washington University, St Louis, MO §Department of Pediatrics, Mount Sinai Medical Center, New York, NY ||Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH ¶Department of Pediatrics, Texas Children's Hospital, Houston #Department of Pediatrics, Children's Health Care of Atlanta, Atlanta, GA **Department of Gastroenterology, Children's Hospital Los Angeles, Los Angeles, CA ††Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA ‡‡Department of Pediatrics, Riley Children's Hospital, Indianapolis, IN §§Department of Pediatrics, Seattle Children's Hospital, Seattle, WA ||||Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada ¶¶School of Public Health, University of Michigan, Ann Arbor ##Department of Pediatrics, University of California San Francisco, San Francisco ***Department of Pediatrics, Johns Hopkins University, Baltimore †††National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD ‡‡‡Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora §§§Department of Pediatrics, Saint Louis University School of Medicine, St Louis, MO ||||||Department of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA ¶¶¶Department of Pediatrics, Children's Memorial Hospital, Chicago, IL.



Fat-soluble vitamin (FSV) deficiency is a well-recognized consequence of cholestatic liver disease and reduced intestinal intraluminal bile acid. We hypothesized that serum bile acid (SBA) would predict biochemical FSV deficiency better than serum total bilirubin (TB) level in infants with biliary atresia.


Infants enrolled in the Trial of Corticosteroid Therapy in Infants with Biliary Atresia after hepatoportoenterostomy were 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 nonparametric correlations were made between specific vitamin measurement levels and either TB or SBA.


The degree of correlation for any particular vitamin at a specific time point was higher with TB than with SBA (higher for TB in 31 circumstances vs 3 circumstances for SBA). Receiver operating characteristic curve shows that TB performed better than SBA (area under the curve 0.998 vs 0.821). Including both TB and SBA did not perform better than TB alone (area under the curve 0.998).


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 progressive familial intrahepatic cholestasis, α-1-antitrypsin deficiency, and Alagille syndrome in which the pathophysiology is dominated by intrahepatic cholestasis, warrants further study.


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