Very low bilirubin after portoenterostomy improves survival of the native liver in patients with biliary atresia by deferring liver fibrogenesis

Abstract

Background: Progression of fibrosis and ensuing complications determine the postoperative course of patients operated on for biliary atresia. We evaluated predictors of the progression of fibrosis in the native liver after operative treatment. Methods: Among patients whose bilirubin decreased to Results: After median follow-up of 5.2 years (interquartile range 1.6-10.2) after portoenterostomy, liver biopsies showed cirrhosis in 53% of patients, and the Metavir stage remained stable or decreased in 38%. The development of cirrhosis was predicted by total or conjugated bilirubin >= 170/120 mu mol/L at the time of portoenterostomy (P = 12.5/7.5 mu mol/L (P=.002) and aspartate aminotransferase-to-platelet ratio >= 0.55 at 3 months postoperatively (P=.001); and total or conjugated bilirubin >= 7.5/2.5 mu mol/L (P = 0.63 (P=.004), and gamma glutamyl transferase >= 266 U/L (P=.007) at 6 months postoperatively. In multiple regression analysis, conjugated bilirubin >= 2.5 mu mol/L at 6 months increased the risk of cirrhosis 35-fold (P=.020), and other predictors were not predictive. Total or conjugated bilirubin <12.5/7.5 mu mol/L (P = 0.55 at 3 months (P=.006), and total or conjugated bilirubin <7.5/2.5 mu mol/L at 6 months postoperatively (P Conclusion: Among patients whose serum bilirubin normalizes after portoenterostomy, its rapid decrease to very low levels prolongs the survival of their native liver by delaying the progression of fibrosis. (C) 2018 Elsevier Inc. All rights reserved.