患有代谢性肝病的儿童肝移植受者的肝动脉并发症发生率高于患有胆道闭锁的儿童。

The rate of hepatic artery complications is higher in pediatric liver transplant recipients with metabolic liver diseases than with biliary atresia.

作者信息

Hong Suk Kyun, Yi Nam-Joon, Chang Hak, Ahn Sung-Woo, Kim Hyo-Sin, Yoon Kyung Chul, Kim Hyeyoung, Park Seong Oh, Jin Ung Sik, Minn Kyung Won, Lee Kwang-Woong, Suh Kyung-Suk

机构信息

Department of Surgery, Seoul National University College of Medicine, Seoul, Republic of Korea.

出版信息

J Pediatr Surg. 2018 Aug;53(8):1516-1522. doi: 10.1016/j.jpedsurg.2018.04.029. Epub 2018 Apr 27.

DOI:10.1016/j.jpedsurg.2018.04.029

PMID:29861326

Abstract

BACKGROUND

Liver transplantation (LT) is an excellent treatment option for patients with biliary atresia (BA) who fail portoenterostomy surgery. LT is also increasingly performed in patients with metabolic liver diseases. This study compared the outcomes in pediatric patients who underwent LT for metabolic liver diseases and BA.

BASIC PROCEDURES

Data from 237 pediatric patients who underwent primary LT at Seoul National University Hospital from 1988 to 2015, including 33 with metabolic liver diseases and 135 with BA, were retrospectively analyzed.

MAIN FINDINGS

Compared with children with BA, children with metabolic liver diseases were significantly older at the time of LT (121.3 vs. 37.3 months; P < 0.001), and had lower Child-Pugh (7.1 vs. 8.4; P = 0.010) and Pediatric End-stage Liver Disease (6.5 vs. 12.8; P = 0.042) scores. Overall survival rates were similar (87.8% vs. 90.8%; P = 0.402), but hepatic artery (HA) complications were significantly more frequent in children with metabolic liver diseases (12.1% vs. 1.5%; P = 0.014).

PRINCIPAL CONCLUSION

Despite similar overall survival, children with metabolic liver diseases had a higher rate of HA complications.

TYPE OF SUBMISSION

Original article, Case control study, Retrospective.

EVIDENCE LEVEL

III.

摘要

背景

肝移植（LT）是胆道闭锁（BA）患儿行门静脉肠吻合术失败后的一种理想治疗选择。肝移植在代谢性肝病患者中的应用也越来越多。本研究比较了因代谢性肝病和胆道闭锁接受肝移植的儿科患者的治疗结果。

基本程序

回顾性分析了1988年至2015年在首尔国立大学医院接受初次肝移植的237例儿科患者的数据，其中33例患有代谢性肝病，135例患有胆道闭锁。

主要发现

与胆道闭锁患儿相比，代谢性肝病患儿肝移植时年龄显著更大（121.3个月对37.3个月；P<0.001），Child-Pugh评分更低（7.1对8.4；P=0.010），小儿终末期肝病评分更低（6.5对12.8；P=0.042）。总体生存率相似（87.8%对90.8%；P=0.402），但代谢性肝病患儿肝动脉（HA）并发症明显更常见（12.1%对1.5%；P=0.014）。