Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
Division of Hepato-Biliary-Pancreatic and Transplant Surgery, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Liver Transpl. 2017 Sep;23(9):1199-1209. doi: 10.1002/lt.24796.
After decades of dramatic surgical innovations in pediatric living donor liver transplantation (LDLT), LDLT for biliary atresia (BA) still poses various challenges. This study reviewed our experience with LDLT for children with post-Kasai BA and evaluated outcomes and prognostic factors. From 2005 to 2016, 168 post-Kasai BA LDLT patients were enrolled and divided into 3 groups by age. Patient characteristics and perioperative data were compared. Predictors of morbidity and mortality following LDLT were analyzed in 93 infants. Outcome was relatively worse in infants than older children, with overall survival at 1 and 5 years of 94.5% and 93.2%, respectively, and graft survival at 1 and 5 years of 91.1% each. Incidence of vascular complications was not significantly higher in infants. High Pediatric End-Stage Liver Disease (PELD) score (odds ratio [OR], 3.72; 95% confidence interval [CI], 1.30-10.67; P = 0.02) and portal vein (PV) hypoplasia (OR, 3.23; 95% CI, 1.10-9.52; P = 0.03) were independent risk factors for morbidity. Low weight-for-age z score (hazard ratio, 5.76; 95% CI, 1.05-31.47; P = 0.03) was identified as a significant risk factor for mortality after LDLT, but not age or absolute body weight (BW). Infants with BW deficit had a significantly smaller PV diameter (P = 0.005), greater blood loss (P = 0.001), and higher incidence of postoperative bacteremia (P = 0.01). In conclusion, high PELD score and PV hypoplasia were independent risk factors for morbidity, and BW deficit was associated with poor survival in infants with post-Kasai BA after LDLT. However, LDLT in these infants at the earliest possible time after referral is a feasible option with excellent patient survival in an experienced center. Liver Transplantation 23 1199-1209 2017 AASLD.
经过几十年小儿活体肝移植（LDLT）的重大手术创新，胆道闭锁（BA）的 LDLT 仍面临各种挑战。本研究回顾了我们在接受过 Kasai 手术的 BA 患儿中进行 LDLT 的经验，并评估了结果和预后因素。2005 年至 2016 年，共纳入 168 例接受过 Kasai 手术的 BA 患儿，按年龄分为 3 组。比较了患者特征和围手术期数据。分析了 93 例婴儿 LDLT 后发病率和死亡率的预测因素。结果显示，婴儿的预后相对较差，1 年和 5 年总生存率分别为 94.5%和 93.2%，1 年和 5 年移植物存活率分别为 91.1%。婴儿血管并发症的发生率没有明显升高。较高的儿童终末期肝病评分（PELD）（比值比 [OR]，3.72；95%置信区间 [CI]，1.30-10.67；P=0.02）和门静脉（PV）发育不良（OR，3.23；95%CI，1.10-9.52；P=0.03）是发病率的独立危险因素。低体重年龄 z 评分（风险比，5.76；95%CI，1.05-31.47；P=0.03）是 LDLT 后死亡的显著危险因素，但不是年龄或绝对体重（BW）。BW 不足的婴儿 PV 直径明显较小（P=0.005），出血量较大（P=0.001），术后菌血症发生率较高（P=0.01）。总之，较高的 PELD 评分和 PV 发育不良是发病率的独立危险因素，BW 不足与接受过 Kasai 手术的 BA 患儿 LDLT 后生存率较差有关。然而，在经验丰富的中心，在转诊后尽早为这些婴儿进行 LDLT 是可行的选择，患者的存活率很高。肝脏移植 23 1199-1209 2017 AASLD。
