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

2019; Elsevier BV; Volume: 71; Issue: 1 Linguagem: Inglês

10.1016/j.jhep.2019.03.005

1600-0641

Vandana Jain, Charlotte Burford, Emma Alexander, Harry Sutton, Anil Dhawan, Deepak Joshi, Mark Davenport, Nigel Heaton, Nedim Hadžić, Marianne Samyn,

Gallbladder and Bile Duct Disorders

•Patients with biliary atresia are at risk of needing a liver transplant when >16 years old. •Higher bilirubin and lower creatinine at 16 years of age are predictors of the need for liver transplant. •Cholangitis and varices in adolescence increase the risk of needing a liver transplant when >16 years old. 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 to portal hypertension (PHT) and cholangitis are common in adulthood. For those requiring liver transplantation (LT), the timing can be challenging. The aim of this study was to identify variables that could predict whether young people with BA would require LT when they are >16 years of age. Methods This study was a single-centre retrospective analysis of 397 patients who underwent Kasai portoenterostomy (KP) between 1980–96 in the UK. After KP, 111/397 (28%) demonstrated NLS until 16 years of age. At final follow-up, 67 showed NLS when >16 years old (Group 1) and 22 required LT when >16 years old (Group 2). Laboratory, clinical and radiological parameters were collected for both groups at a median age of 16.06 years (13.6–17.4 years). Results The need for LT when >16 years old was associated with higher total bilirubin (hazard ratio 1.03, p = 0.019) and lower creatinine (hazard ratio 0.95, p = 0.040), at 16 years, on multivariate analysis. Receiver-operating characteristic curve analysis demonstrated that a total bilirubin level of ≥21 µmol/L at 16 years old (AUROC = 0.848) predicted the need for LT when >16 years old, with 85% sensitivity and 74% specificity. Cholangitis episode(s) during adolescence were associated with a 5-fold increased risk of needing LT when >16 years old. The presence of PHT or gastro-oesophageal varices in patients 16 years of age. This study was a single-centre retrospective analysis of 397 patients who underwent Kasai portoenterostomy (KP) between 1980–96 in the UK. After KP, 111/397 (28%) demonstrated NLS until 16 years of age. At final follow-up, 67 showed NLS when >16 years old (Group 1) and 22 required LT when >16 years old (Group 2). Laboratory, clinical and radiological parameters were collected for both groups at a median age of 16.06 years (13.6–17.4 years). The need for LT when >16 years old was associated with higher total bilirubin (hazard ratio 1.03, p = 0.019) and lower creatinine (hazard ratio 0.95, p = 0.040), at 16 years, on multivariate analysis. Receiver-operating characteristic curve analysis demonstrated that a total bilirubin level of ≥21 µmol/L at 16 years old (AUROC = 0.848) predicted the need for LT when >16 years old, with 85% sensitivity and 74% specificity. Cholangitis episode(s) during adolescence were associated with a 5-fold increased risk of needing LT when >16 years old. The presence of PHT or gastro-oesophageal varices in patients <16 years old was associated with a 7-fold and 8.6-fold increase in the risk of needing LT, respectively. BA in adulthood requires specialised management. Adult liver disease scoring models are not appropriate for this cohort. Bilirubin ≥21 µmol/L, PHT or gastro-oesophageal varices at 16 years, and cholangitis in adolescence, can predict the need for future LT in young people with BA. Low creatinine at 16 years also has potential prognostic value.