Prognostic markers at adolescence in patients requiring liver transplantation for biliary atresia in adulthood.

摘要: Background & Aims In patients with biliary atresia (BA), the rate of native liver survival (NLS) to adulthood has been reported as 14–44% worldwide. Complications related portal hypertension (PHT) and cholangitis are common in adulthood. For those requiring transplantation (LT), timing can be challenging. The aim this study was identify variables that could predict whether young people BA would require LT when they >16 years age. Methods This a single-centre retrospective analysis 397 who underwent Kasai portoenterostomy (KP) between 1980–96 UK. After KP, 111/397 (28%) demonstrated NLS until 16 years At final follow-up, 67 showed old (Group 1) 22 required 2). Laboratory, clinical radiological parameters were collected for both groups at median age 16.06 years (13.6–17.4 years). Results need associated higher total bilirubin (hazard ratio 1.03, p = 0.019) lower creatinine 0.95, p = 0.040), 16 years, on multivariate analysis. Receiver-operating characteristic curve level ≥21 µmol/L (AUROC = 0.848) predicted old, 85% sensitivity 74% specificity. Cholangitis episode(s) during adolescence 5-fold increased risk needing old. presence PHT or gastro-oesophageal varices Conclusions requires specialised management. Adult disease scoring models not appropriate cohort. Bilirubin ≥21 µmol/L, adolescence, future BA. Low also potential prognostic value. Lay summary Patients commonly before reaching Those reach their own still transplant. aimed tests help clinicians which 16 without transplant will one later life. found levels, history well likelihood atresia.