Department of Pediatric Surgery, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8677, Japan.
Department of Pediatric Surgery, Chiba Children's Hospital, 579-1 Heta-Cho, Midori-ku, Chiba, Japan.
Pediatr Surg Int. 2023 Sep 5;39(1):263. doi: 10.1007/s00383-023-05551-6.
This study aimed to elucidate the difficulties faced by adult native liver survivors with biliary atresia (BA) in Japan.
A single-center, retrospective, observational study of 57 adult patients with BA was conducted. The clinical course of BA was compared between native liver survivors and non-survivors who reached adulthood. Indications and outcomes of liver transplantation (LT) among non-survivors were assessed.
A significantly larger portion of non-survivors (n = 10) met the criteria for LT (p < 0.001) and received treatment for portal hypertension after reaching 20 years of age (p < 0.01) compared with the survivors. Causes of death included liver cirrhosis (n = 8), graft failure of living donor liver transplantation (LDLT) (n = 1), and hepatocarcinoma (n = 1). Two of the non-survivors who died of liver cirrhosis had no indication for LT because of alcohol dependence and uncontrolled infection. An appropriate donor candidate could not be found for the five patients who opted for LDLT. All six patients waitlisted for deceased donor liver transplantation (DDLT) died after a median waiting period of 17 months.
Adult BA patients in Japan have limited options for LT, mainly owing to low donor candidate availability for LDLT and a low prevalence of DDLT.
本研究旨在阐明日本胆道闭锁（BA）成年原生肝幸存者所面临的困难。
对 57 例成年 BA 患者进行了单中心、回顾性、观察性研究。比较了 BA 临床病程在原生肝幸存者和成年后未存活者之间的差异。评估了未存活者肝移植（LT）的适应证和结局。
与幸存者相比，更多的未存活者（n=10）符合 LT 标准（p<0.001），并且在 20 岁后接受了门静脉高压的治疗（p<0.01）。死亡原因包括肝硬化（n=8）、活体供肝移植（LDLT）移植物衰竭（n=1）和肝癌（n=1）。2 名死于肝硬化的未存活者因酒精依赖和感染失控而没有 LT 的适应证。选择 LDLT 的 5 名患者中，均未能找到合适的供者候选人。所有 6 名等待接受尸体供肝移植（DDLT）的患者在中位等待期 17 个月后死亡。
日本的成年 BA 患者 LT 选择有限，主要是由于 LDLT 的供者候选者不足和 DDLT 的发生率较低。
