Association between post-Kasai cholangitis episodes, ursodeoxycholic acid regimen, and portal hypertension risk in biliary atresia: a multicenter cross-sectional study

To investigate the association between cholangitis and portal hypertension (PH) risk, and the efficacy of UDCA in native liver survivors after successful Kasai portoenterostomy (KPE).

A multicenter, retrospective cross-sectional study was conducted at nine Chinese pediatric centers. Patient grouping was based on cumulative cholangitis episodes and UDCA regimens.

After a median follow-up of 56 months, the overall incidence of PH was 23.63% (198/838). Recurrent cholangitis (≥ 3 episodes) was an independent risk factor for PH (OR = 4.25, 95% CI 2.61–6.92, P < 0.001), associated with poorer liver function recovery, greater spleen thickness, and lower peak velocity of the main portal vein. Continuous UDCA therapy significantly attenuated the PH risk induced by recurrent cholangitis (RERI: − 7.66). Among this high-risk subgroup, low-dose UDCA (≤ 10 mg/kg/day) constituted an independent protective factor (OR = 0.17, 95%CI 0.04–0.67, P = 0.015) and showed superior efficacy in reducing total bilirubin. These benefits were evident in children under 48 months, but no clear advantage observed beyond this age.

Among native liver survivors with jaundice clearance, recurrent cholangitis (≥ 3 episodes) warrants long-term monitoring for PH. Continuous low-dose UDCA until 48 months is recommended for high-risk patients to improve outcomes.