肝移植治疗儿童急性肝衰竭：单中心8年治疗经验

刘颖; 孙丽莹; 朱志军; 魏林; 曲伟; 曾志贵

doi:10.3969/j.issn.1674-7445.2022.05.009

肝移植治疗儿童急性肝衰竭：单中心8年治疗经验

doi: 10.3969/j.issn.1674-7445.2022.05.009

100050 北京，首都医科大学附属北京友谊医院重症肝病科国家消化系统疾病临床医学研究中心（刘颖、孙丽莹），肝脏移植中心国家消化系统疾病临床医学研究中心（朱志军、魏林、曲伟、曾志贵）

基金项目:

首都卫生发展科研专项首发2020-1-2024

详细信息

作者简介:

通讯作者:
孙丽莹，主任医师，研究方向为肝移植及肝病患者的重症管理、移植感染与免疫，Email：sunxlx@outlook.com

中图分类号: R617,R575.3
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出版历程
- 收稿日期: 2022-04-22
- 网络出版日期: 2022-09-14
- 刊出日期: 2022-09-15

Liver transplantation for acute liver failure in children: 8-year experience in a single center

Department of Critical Liver Diseases, Beijing Friendship Hospital, Capital Medical University, National Clinical Research Center for Digestive Diseases, Beijing 100050, China

More Information

Corresponding author: Sun Liying, Email: sunxlx@outlook.com

摘要

摘要: 目的探讨肝移植治疗儿童急性肝衰竭（ALF）的疗效。方法收集行肝移植的15例ALF患儿的临床资料进行回顾性分析，统计同期儿童肝移植受者中ALF所占比例，分析ALF患儿肝移植手术特点、术后并发症及治疗转归情况。结果同期儿童肝移植受者中ALF占2.0%（15/743）。15例ALF患儿均急性起病，多数伴有发热、腹泻、进行性皮肤及巩膜黄染加重。13例患儿术前合并肝性脑病（其中6例为4期脑病），2例患儿术前合并骨髓抑制、粒细胞减少。10例患儿行亲属供肝的活体肝移植，4例行心脏死亡器官捐献（DCD）供肝肝移植，1例行多米诺供肝辅助性肝移植。15例患儿中，12例供、受者血型相同，1例血型相容，2例跨血型。15例患儿中，有10例术后出现并发症。术后并发脑水肿5例，其中4例死于弥漫性脑水肿，另1例为持续植物状态（睁眼昏迷）。术后发生巨细胞病毒（CMV）感染5例，2例术后出现再生障碍性贫血行骨髓移植治疗后生存，1例死于CMV性肝炎、病毒性脑炎，2例死于弥漫性脑水肿。1例患儿术后出现移植物抗宿主病（GVHD），行骨髓移植后死于感染性休克。存活的9例患儿随访期间肝功能良好。结论肝移植是治疗儿童ALF的有效手段，可以提高患儿的生存率。脑水肿是ALF患儿肝移植术后死亡的主要原因，应积极行降颅压、改善脑代谢及血液净化等治疗。在ALF患儿出现不可逆性神经系统损害前尽早行肝移植手术，有望延长患儿的生存期和改善其长期预后。
- 儿童 /
- 急性肝衰竭 /
- 肝移植 /
- 肝性脑病 /
- 脑水肿 /
- 活体肝移植（LDLT） /
- 心脏死亡器官捐献（DCD） /
- 连续性肾脏替代治疗（CRRT） /
- 移植物抗宿主病（GVHD）
Abstract: Objective To evaluate the efficacy of liver transplantation for acute liver failure (ALF) in children. Methods Clinical data of 15 children with ALF who underwent liver transplantation were collected and retrospectively analyzed. The proportion of ALF among children undergoing liver transplantation during the same period was calculated. The characteristics, postoperative complications and clinical prognosis of ALF children receiving liver transplantation were analyzed. Results In the same period, the proportion of ALF was 2.0% (15/743) among pediatric recipients undergoing liver transplantation. All 15 children had acute onset of ALF, and most of them were accompanied by fever, diarrhea and progressive yellowing of skin and sclera. Thirteen children were complicated with hepatic encephalopathy before operation (6 cases of stage Ⅳ hepatic encephalopathy), and two children were complicated with myelosuppression and granulocytopenia before liver transplantation. Ten children underwent living donor liver transplantation with relative donor liver, 4 received liver transplantation from donation after cardiac death (DCD), and 1 underwent Domino donor-auxiliary liver transplantation. Of 15 children, 12 recipients had the same blood type with their donors, 1 recipient had compatible blood type with the donor and 2 cases had different blood type with their donors. Among 15 children, 10 cases developed postoperative complications. Postoperative cerebral edema occurred in 5 cases, of whom 4 cases died of diffuse cerebral edema, and the remaining case was in a persistent vegetative state (eyes-open coma). Postoperative cytomegalovirus (CMV) infection was seen in 5 cases. Two children presented with aplastic anemia and survived after bone marrow transplantation, 1 case died of CMV hepatitis and viral encephalitis, and 2 cases died of diffuse brain edema. One child developed graft-versus-host disease (GVHD) after liver transplantation, and died of septic shock after bone marrow transplantation. Nine children survived and obtained favorable liver function during postoperative follow-up. Conclusions Liver transplantation is an efficacious treatment for ALF in children, which may enhance the survival rate. Brain edema is the main cause of death in ALF children following liver transplantation, and treatment such as lowering intracranial pressure, improving brain metabolism and blood purification should be actively performed. Liver transplantation should be promptly performed prior to the incidence of irreversible neurological damage in ALF children, which might prolong the survival and enhance long-term prognosis.
- Children /
- Acute liver failure /
- Liver transplantation /
- Hepatic encephalopathy /
- Brain edema /
- Living donor liver transplantation (LDLT) /
- Donation after cardiac death (DCD) /
- Continuous renal replacement therapy (CRRT) /
- Graft-versus-host disease (GVHD)

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表 1 15例ALF患儿的临床资料

Table 1. Clinical data of 15 children with ALF

例序	性别	年龄	原发病	肝性脑病分期	手术方式	供、受者血型	并发症	治疗	住院时间（d）	预后
1	男	1岁	CMV性肝炎	3期	活体肝移植	相容	肺部感染、腹腔感染，肠漏，CMV性肝炎	抗感染，肠切除+肠吻合术，抗病毒	74	术后74 d死于CMV性肝炎、病毒性脑炎
2	女	3岁	药物性肝损伤	3期	劈离式右半肝辅助肝移植（DCD^①供肝）	相同	无	-^②	28	生存
3	男	3岁	原因不明	3期	活体肝移植	相同	无	-	24	生存
4	男	4岁	药物性肝损伤	3期	活体辅助式肝移植	相同	GVHD^③	骨髓移植	27	术后373 d死于感染性休克
5	男	5岁	药物性肝损伤	4期	原位肝移植（DCD供肝）	相同	脑水肿、癫痫发作，肺部感染	脱水、抗癫痫、呼吸机辅助呼吸，抗感染	85	生存，睁眼昏迷
6	男	6岁	药物性肝损伤	1期	活体肝移植	相同	无	-	20	生存
7	男	6岁	原因不明	4期	多米诺辅助性肝移植（活体供肝）	跨血型	脑水肿、癫痫，肺部感染、腹腔感染	抗癫痫、脱水、镇静治疗，抗感染	65	术后65 d死于弥漫性脑水肿
8	女	6岁	药物性肝损伤	4期	活体肝移植	相同	弥漫性脑水肿，AKI^④，肺部感染，CMV性肝炎	脱水、呼吸机辅助呼吸，CRRT^⑤，抗感染，抗病毒	106	术后106 d死于重度颅内神经系统损伤、弥漫性脑水肿
9	男	9岁	CMV性肝炎	3期	活体肝移植	跨血型	发热伴粒细胞减少、CMV感染、再生障碍性贫血、骨髓衰竭	抗病毒，骨髓移植	18	生存
10	男	9岁	原因不明	无	活体辅助式肝移植	相同	发热伴粒细胞减少、CMV感染、再生障碍性贫血、骨髓衰竭	抗病毒，骨髓移植	14	生存
11	男	10岁	肝豆状核变性	2期	活体肝移植	相同	无	-	30	生存
12	男	11岁	药物性肝损伤	4期	活体辅助式肝移植	相同	脑水肿、中枢性尿崩症	脱水，CRRT	5	术后5 d死于弥漫性脑水肿
13	女	14岁	原因不明	4期	活体辅助式肝移植	相同	无	-	24	生存
14	女	10个月	药物性肝损伤	无	原位减体积肝移植（DCD供肝）	相同	肺感染、腹腔感染，癫痫局灶发作	抗感染，抗癫痫	31	生存
15	男	9个月	药物性肝损伤	4期	原位肝移植（DCD供肝）	相同	脑水肿、癫痫发作，腺病毒及CMV感染	脱水，抗癫痫，抗病毒	40	术后103 d死于弥漫性脑水肿
注：①DCD为心脏死亡器官捐献。 ②-为无数据。 ③GVHD为移植物抗宿主病。 ④AKI为急性肾损伤。 ⑤CRRT为连续性肾脏替代治疗。

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