Annual cholangitis more than twice predicts liver transplant in biliary atresia patients who achieve jaundice-free after Kasai portoenterostomy

Background: Biliary atresia (BA) is a progressive liver disease even after Kasai portoenterostomy (KPE), and the most common cause of liver transplant (LT) in the pediatric population. This study aimed to unveil the risk factors for LT in BA patients post-KPE.

Methods: We conducted a retrospective study of BA patients in a northern Taiwan Children's Medical Center from Jan 2000 to Oct 2020.

Results: A total of 65 BA patients (32 males, 33 females) were included. Seventeen (26%) patients received LT. Multivariate analysis showed higher serum direct bilirubin (cutoff ≥1.1 mg/dL) 3 months post-KPE (OR 8.17, p = 0.037) and higher peak yearly cholangitis episodes (OR 3.32, p = 0.008) independently predicted LT. In patients achieving jaundice-free (JF), yearly cholangitis frequency (OR 2.64, p = 0.032) independently predicted LT (cutoff ≥3). Concerning high cholangitis frequency, the independent predictor was the first cholangitis occurring 61-120 days post-KPE (OR 6.61, p = 0.034). In patients who achieved JF, bacteremia (OR 20.06, p = 0.037) and higher AST level 6 months post-KPE (OR 1.02, p = 0.016) independently predicted higher peak annual cholangitis frequency.

Conclusions: Higher direct bilirubin 3 months post-KPE (≥1.1 mg/dL) and higher peak annual cholangitis frequency predict LT in BA patients. The first cholangitis occurring 61-120 days post-KPE predicts higher peak annual cholangitis frequency. In patients who achieved JF, peak annual cholangitis episodes with a threshold of ≥3 independently predict LT. Bacteremia and higher serum AST levels at six months post-KPE predict higher peak annual cholangitis frequency.