http://www.ncbi.nlm.nih.gov/pubmed/27429424

Nightingale S, Stormon MO, O'Loughlin EV, Shun A, Thomas G, Benchimol EI, Day AS, Adams S, Shi E, Ooi CY, Kamath BM, Fecteau A, Langer JC, Roberts EA, Ling SC, Ng VL. J Pediatr Gastroenterol Nutr. 2016 Jul 16.

Abstract

OBJECTIVES:
Most infants with biliary atresia (BA) require liver transplantation (LT) following hepatoportoenterostomy (HPE), including those who initially clear jaundice. The aim of this study was to identify clinical and routine laboratory factors in BA infants post-HPE that predict native liver survival at 2 years.

METHODS:
A retrospective cohort study was conducted with 217 BA patients undergoing HPE in Sydney, Australia and Toronto, Canada between January 1986-July 2009. Univariate and multivariate logistic regression using backwards-stepwise elimination identified variables at 3 months post-HPE most associated with 2-year native liver survival.

RESULTS:
Significant variables (p < 0.05) on univariate analysis included serum total bilirubin (TB) and albumin at 3 months post-HPE, bridging fibrosis or cirrhosis on initial liver biopsy, ascites<3 months post-HPE, type 3 BA anatomy, age at HPE>45 days, change in length z-scores within 3 months of HPE and center. On multivariate analysis, TB (p < 0.0001) and albumin (p = 0.02) at 3 months post-HPE, and center (p = 0.0003) were independently associated with native liver survival. ROC analysis revealed an optimal cut-off value of TB <74 μmol/L (4.3 mg/dL; AUROC 0.8990) and serum albumin level>35 g/L (3.5 mg/dL; AUROC 0.7633) to predict 2-year native liver survival. TB and albumin levels 3 months post-HPE defined three Groups (1: TB≤74 μmol/L, albumin>35 g/L; 2: TB≤74 μmol/L, albumin≤35 g/L; 3: TB>74 μmol/L) with distinct short- and long-term native liver survival rates (log-rank p < 0.001). Length z-scores 3 months post-HPE were poorer for Group 2 than Group 1 (-0.91 vs -0.30, p = 0.0217) with similar rates of coagulopathy.

CONCLUSIONS:
Serum total bilirubin and albumin levels 3 months post-HPE independently predicted native liver survival in BA when controlling for center. Serum albumin level <35 g/L in BA infants who were no longer jaundiced at 3 months post-HPE was a poor prognostic indicator. Poorer linear growth and absence of significant coagulopathy suggests a role for early aggressive nutritional therapy in this group.