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本文引用的文献

1
Stool color card screening for early detection of biliary atresia and long-term native liver survival: a 19-year cohort study in Japan.粪便颜色卡筛查用于早期发现胆道闭锁和长期固有肝存活率:日本的一项 19 年队列研究。
J Pediatr. 2015 Apr;166(4):897-902.e1. doi: 10.1016/j.jpeds.2014.12.063. Epub 2015 Feb 11.
2
Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant.超越小儿终末期肝病系统:针对需要肝移植的胆道闭锁婴儿的解决方案。
World J Gastroenterol. 2014 Aug 28;20(32):11062-8. doi: 10.3748/wjg.v20.i32.11062.
3
Cause of death among infants in rural western China: a community-based study using verbal autopsy.中国西部农村婴儿死亡原因:基于社区的使用死因推断的研究。
J Pediatr. 2014 Sep;165(3):577-84. doi: 10.1016/j.jpeds.2014.04.047. Epub 2014 Jun 11.
4
Operative outcomes of adult living donor liver transplantation and deceased donor liver transplantation: a systematic review and meta-analysis.成人活体供肝肝移植和尸体供肝肝移植的手术结果:一项系统评价和荟萃分析。
Liver Transpl. 2014 Apr;20(4):425-36. doi: 10.1002/lt.23836.
5
De novo hepatitis B virus infection from anti-HBc-positive donors in pediatric living donor liver transplantation.儿童活体肝移植中源于抗-HBc 阳性供者的乙型肝炎病毒再感染。
J Dig Dis. 2013 Aug;14(8):439-45. doi: 10.1111/1751-2980.12066.
6
Biliary atresia in preterm infants in Taiwan: a nationwide survey.台湾地区早产儿胆道闭锁症:一项全国性调查。
J Pediatr. 2013 Jul;163(1):100-3.e1. doi: 10.1016/j.jpeds.2012.12.085. Epub 2013 Feb 12.
7
Improving outcomes of biliary atresia: French national series 1986-2009.提高胆道闭锁的治疗效果:法国全国系列研究 1986-2009。
J Hepatol. 2013 Jun;58(6):1209-17. doi: 10.1016/j.jhep.2013.01.040. Epub 2013 Feb 8.
8
The impact of hepatic portoenterostomy on liver transplantation for the treatment of biliary atresia: early failure adversely affects outcome.肝门空肠吻合术对胆道闭锁肝移植治疗的影响:早期失败对预后产生不利影响。
Pediatr Transplant. 2012 Jun;16(4):373-8. doi: 10.1111/j.1399-3046.2012.01677.x. Epub 2012 Mar 30.
9
Geographical disparities of infant mortality in rural China.中国农村婴儿死亡率的地域差异。
Arch Dis Child Fetal Neonatal Ed. 2012 Jul;97(4):F285-90. doi: 10.1136/archdischild-2011-300412. Epub 2012 Jan 12.
10
Biliary atresia in the Netherlands: outcome of patients diagnosed between 1987 and 2008.荷兰的先天性胆道闭锁:1987 年至 2008 年诊断出的患者的结果。
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中国大陆地区胆道闭锁肝移植的单中心研究

Liver transplantation for biliary atresia: A single-center study from mainland China.

作者信息

Li Qi-Gen, Wan Ping, Zhang Jian-Jun, Chen Qi-Min, Chen Xiao-Song, Han Long-Zhi, Xia Qiang

机构信息

Qi-Gen Li, Ping Wan, Jian-Jun Zhang, Xiao-Song Chen, Long-Zhi Han, Qiang Xia, Department of Liver Surgery, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China.

出版信息

World J Gastroenterol. 2015 Aug 28;21(32):9638-47. doi: 10.3748/wjg.v21.i32.9638.

DOI:10.3748/wjg.v21.i32.9638
PMID:26327772
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4548125/
Abstract

AIM

To summarize our single-center experience with liver transplantation (LT) for biliary atresia (BA).

METHODS

From October 2006 to December 2012, 188 children with BA were analyzed retrospectively. The stage I group (from October 2006 to December 2010) comprised the first 74 patients, and the stage II group (from January 2011 to December 2012) comprised the remaining 114 patients. Finally, 123 liver transplants were performed in 122 (64.9%) patients, whereas 66 patients did not undergo LT due to denial by their parents or lack of suitable liver grafts. The selection of graft types depended on the patients' clinical status and whether a suitable living donor was available. The characteristics of patients in stages I and II were described, and the surgical outcomes of LT recipients were compared between the two stages. The Kaplan-Meier method was used to estimate the cumulative patient and graft survival rates, and the equality of survival distributions was evaluated using the log-rank test.

RESULTS

The 188 children consisted of 102 boys and 86 girls. Their ages ranged from 3 to 144 mo with a median of 8 mo. One hundred and fifteen (61.2%) patients were born in rural areas. Comparing stage I and stage II patients, the proportion of patients referred by pediatricians (43.2% vs 71.1%, respectively; P < 0.001) and the proportion of patients who previously received a Kasai procedure (KP) (32.4% vs 44.7%, respectively; P = 0.092) obviously increased, and significantly more parents were willing to treat their children with LT (73% vs 86%, respectively; P = 0.027). Grafts from living donors (102/122, 83.6%) were the most commonly used graft type. Surgical complications (16/25, 64.0%) were the main reason for posttransplant mortality. Among the living donor liver transplantation recipients (n = 102), the incidence of surgical complications was significantly reduced (34.1% vs 15.5%, respectively; P = 0.029) and survival rates of patients and grafts were greatly improved (81.8% vs 89.7%, respectively, at 1 year; 75.0% vs 87.8%, respectively, at 3 years; P = 0.107) from stage I to stage II.

CONCLUSION

The status of surgical treatments for BA has been changing in mainland China. Favorable midterm outcomes after LT were achieved as centers gained greater technical experience.

摘要

目的

总结我们单中心进行的胆道闭锁(BA)肝移植(LT)经验。

方法

回顾性分析2006年10月至2012年12月期间188例BA患儿。I期组(2006年10月至2010年12月)包括前74例患者,II期组(2011年1月至2012年12月)包括其余114例患者。最终,122例(64.9%)患者接受了123次肝移植,而66例患者因家长拒绝或缺乏合适的肝移植物未接受LT。移植物类型的选择取决于患者的临床状况以及是否有合适的活体供体。描述了I期和II期患者的特征,并比较了两个阶段LT受者的手术结果。采用Kaplan-Meier法估计患者和移植物的累积生存率,并使用对数秩检验评估生存分布的平等性。

结果

188例患儿中,男102例,女86例。年龄范围为3至144个月,中位数为8个月。115例(61.2%)患者出生在农村地区。比较I期和II期患者,儿科医生转诊的患者比例(分别为43.2%和71.1%;P<0.001)以及先前接受过Kasai手术(KP)的患者比例(分别为32.4%和44.7%;P = 0.092)明显增加,并且愿意为孩子进行LT治疗的家长明显增多(分别为73%和86%;P = 0.027)。活体供肝(102/122,83.6%)是最常用的移植物类型。手术并发症(16/25,64.0%)是移植后死亡的主要原因。在活体肝移植受者(n = 102)中,手术并发症的发生率从I期到II期显著降低(分别为34.1%和15.5%;P = 0.029),患者和移植物的生存率大幅提高(1年时分别为81.8%和89.7%;3年时分别为75.0%和87.8%;P = 0.107)。

结论

中国大陆BA的外科治疗状况一直在变化。随着各中心技术经验的增加,LT术后取得了良好的中期结果。