Division of Digestive and Liver Diseases, UT Southwestern Medical Center, Dallas, Texas, USA.
Department of Surgery, UT Southwestern Medical Center, Dallas, Texas, USA.
Liver Int. 2023 Oct;43(10):2198-2209. doi: 10.1111/liv.15689. Epub 2023 Aug 7.
Biliary atresia (BA) remains the number one indication for paediatric liver transplantation (LT) worldwide but is an uncommon indication for older LT recipients. The impact of recent donor allocation changes, pervasive organ shortage and evolving LT practices on the BA LT population is unknown.
We identified patients who underwent LT between January 2010 and December 2021 using the UNOS database. We compared clinical outcomes between patients with BA and those with non-BA cholestatic liver disease. Groups were stratified by age, <12 years (allocated via PELD system) and ≥12 years (allocated via MELD system). Waitlist outcomes were compared using competing-risk regression analysis, graft survival rates were compared using Kaplan-Meier time-to-event analysis and Cox proportional hazards modelling provided adjusted estimates.
There were 2754 BA LT waitlist additions and 2206 BA LTs (1937 <12 years [younger], 269 ≥12 years [older]). There were no differences in waitlist mortality between BA and non-BA cholestatic patients. Among BA LT recipients, there were 441 (20.0%) living-donor liver transplantations (LDLT) and 611 (27.7%) split deceased-donor LTs. Five-year graft survival was significantly higher among BA versus non-BA cholestatic patients in the older group (88.3% vs. 79.5%, p < .01) but not younger group (89.3% vs. 89.5%). Among BA LT recipients, improved graft outcomes were associated with LDLT (vs. split LT: HR: 2, 95% CI: 1.03-3.91) and higher transplant volume (volume >100 vs. <40 BA LTs: HR: 3.41, 95% CI: 1.87-6.2).
Liver transplant outcomes among BA patients are excellent, with LDLT and higher transplant centre volume associated with optimal graft outcomes.
胆道闭锁（BA）仍然是全世界小儿肝移植（LT）的首要适应证，但在老年 LT 受者中并不常见。最近的供体分配变化、普遍的器官短缺和不断发展的 LT 实践对 BA LT 人群的影响尚不清楚。
我们使用 UNOS 数据库确定了 2010 年 1 月至 2021 年 12 月期间接受 LT 的患者。我们比较了 BA 患者和非 BA 胆汁淤积性肝病患者的临床结局。根据年龄、<12 岁（通过 PELD 系统分配）和≥12 岁（通过 MELD 系统分配）将两组进行分层。使用竞争风险回归分析比较等待名单结果，使用 Kaplan-Meier 时间事件分析比较移植物存活率，并使用 Cox 比例风险模型提供调整后的估计。
共有 2754 例 BA LT 等待名单添加和 2206 例 BA LT（1937 例<12 岁[年轻组]，269 例≥12 岁[年长组]）。BA 和非 BA 胆汁淤积患者在等待名单上的死亡率没有差异。在 BA LT 受者中，有 441 例（20.0%）接受了活体供肝移植（LDLT），611 例（27.7%）接受了分割尸体供肝移植。在年长组中，BA 患者的 5 年移植物存活率明显高于非 BA 胆汁淤积患者（88.3%比 79.5%，p<0.01），但在年轻组中无差异（89.3%比 89.5%）。在 BA LT 受者中，改善的移植物结局与 LDLT（与分割 LT 相比：HR：2，95%CI：1.03-3.91）和更高的移植中心容量（容量>100 与<40 例 BA LT 相比：HR：3.41，95%CI：1.87-6.2）相关。
BA 患者的 LT 结局非常出色，LDLT 和更高的移植中心容量与最佳移植物结局相关。
