Paediatric Liver, GI and Nutrition Centre and Mowatlabs, Kings College Hospital, London, UK.
Faculty of Life Sciences and Medicine, Kings College London, London, UK.
J Hepatol. 2019 Jul;71(1):71-77. doi: 10.1016/j.jhep.2019.03.005. Epub 2019 Mar 13.
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.
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.
Patients with biliary atresia commonly require liver transplantation before reaching adulthood. Those who reach adulthood with their own liver are still at risk of needing a transplant. This study aimed to identify tests that could help clinicians predict which patients with biliary atresia who reach the age of 16 without a transplant will require one in later life. The study found that the presence of bilirubin ≥21 µmol/L, lower creatinine levels, and a history of portal hypertension or gastro-oesophageal varices at 16 years, as well as cholangitis in adolescence, could predict the future likelihood of needing a liver transplant for young people with biliary atresia.
在胆道闭锁（BA）患者中，全球范围内报告的自体肝存活率（NLS）至成年期的比例为 14%-44%。与门静脉高压（PHT）和胆管炎相关的并发症在成年期很常见。对于需要肝移植（LT）的患者，时机可能具有挑战性。本研究的目的是确定可预测 BA 年轻人在 16 岁以上时是否需要 LT 的变量。
这是一项在英国进行的回顾性单中心研究，纳入了 1980-96 年间接受 Kasai 门腔分流术（KP）的 397 例患者。KP 后，111/397（28%）在 16 岁之前显示出 NLS。在最终随访时，67 例在 16 岁以上时显示出 NLS（第 1 组），22 例在 16 岁以上时需要 LT（第 2 组）。在中位数为 16.06 岁（13.6-17.4 岁）时，收集了两组的实验室、临床和影像学参数。
在多变量分析中，16 岁时需要 LT 的因素与较高的总胆红素（危险比 1.03，p=0.019）和较低的肌酐（危险比 0.95，p=0.040）相关。受试者工作特征曲线分析表明，16 岁时总胆红素水平≥21µmol/L（AUROC=0.848）可预测 16 岁以上需要 LT，其敏感性为 85%，特异性为 74%。青少年时期发生胆管炎发作与 16 岁以上需要 LT 的风险增加 5 倍相关。16 岁以下患者存在 PHT 或胃食管静脉曲张与需要 LT 的风险分别增加 7 倍和 8.6 倍相关。
BA 进入成年期需要专门的管理。成人肝病评分模型不适合该队列。16 岁时胆红素≥21µmol/L、PHT 或胃食管静脉曲张和青少年时期的胆管炎可预测 BA 年轻患者未来需要 LT 的情况。16 岁时肌酐较低也具有潜在的预后价值。
胆道闭锁患者在成年前通常需要进行肝移植。那些在成年期仍保留自体肝脏的患者仍有需要进行移植的风险。本研究旨在确定能够帮助临床医生预测哪些在成年期前未接受移植的胆道闭锁患者在以后的生活中需要接受移植。该研究发现，16 岁时存在胆红素≥21µmol/L、肌酐水平较低、存在门静脉高压或胃食管静脉曲张以及青少年时期发生胆管炎，这些因素可能预测胆道闭锁患者在成年后需要进行肝移植的可能性。
