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亲属活体肝移植治疗婴儿Crigler-Najjar综合征Ⅰ型1例并文献复习

顾广祥 宗志鹏 周韬 夏强

顾广祥, 宗志鹏, 周韬, 等. 亲属活体肝移植治疗婴儿Crigler-Najjar综合征Ⅰ型1例并文献复习[J]. 器官移植, 2020, 11(4): 471-476. doi: 10.3969/j.issn.1674-7445.2020.04.008
引用本文: 顾广祥, 宗志鹏, 周韬, 等. 亲属活体肝移植治疗婴儿Crigler-Najjar综合征Ⅰ型1例并文献复习[J]. 器官移植, 2020, 11(4): 471-476. doi: 10.3969/j.issn.1674-7445.2020.04.008
Gu Guangxiang, Zong Zhipeng, Zhou Tao, et al. Living related donor liver transplantation for infant with Crigler-Najjar syndrome type Ⅰ: a report of one case and literature review[J]. ORGAN TRANSPLANTATION, 2020, 11(4): 471-476. doi: 10.3969/j.issn.1674-7445.2020.04.008
Citation: Gu Guangxiang, Zong Zhipeng, Zhou Tao, et al. Living related donor liver transplantation for infant with Crigler-Najjar syndrome type Ⅰ: a report of one case and literature review[J]. ORGAN TRANSPLANTATION, 2020, 11(4): 471-476. doi: 10.3969/j.issn.1674-7445.2020.04.008

亲属活体肝移植治疗婴儿Crigler-Najjar综合征Ⅰ型1例并文献复习

doi: 10.3969/j.issn.1674-7445.2020.04.008
基金项目: 

国家自然科学基金青年项目 81700570

详细信息
    作者简介:

    顾广祥,男,1985年生,博士,住院医师,研究方向为儿童肝移植,Email:guguangxiang@163.com

    通讯作者:

    夏强,男,1966年生,博士,主任医师,研究方向为肝移植,Email:xiaqiang@shsmu.edu.cn

  • 中图分类号: R617, R596

Living related donor liver transplantation for infant with Crigler-Najjar syndrome type Ⅰ: a report of one case and literature review

More Information
  • 摘要:   目的  总结克里格勒-纳贾尔综合征(CNS)Ⅰ型的亲属活体肝移植治疗效果。  方法  1例男性3月龄患儿,出生后4 d无明显诱因下出现皮肤、巩膜进行性黄染,完善相关检查后排除其他诱因,并经基因检测鉴定为CNS Ⅰ型。其母亲作为供者,行亲属活体肝移植术。术后常规应用免疫抑制方案,并根据生化指标及受者细胞色素P450(CYP)3A5基因型调整他克莫司剂量。  结果  受者术后7 d肝酶恢复正常,胆红素逐日下降并于术后22 d降至正常范围。随访至投稿日,受者皮肤巩膜黄染褪去,胆红素正常,肝酶稳定,一般情况佳,生活质量高。  结论  活体肝移植可治疗CNS Ⅰ型所带来的高非结合胆红素血症等病症,极大提高患者的生活质量。

     

  • 图  1  肝移植术前通过EDDA IQQA-Liver系统预估血管走向及供肝体积

    注:A图为肝静脉分割走形;B图为门静脉分割走形;C图为肝动脉分割走形;D图为供肝体积评估。

    Figure  1.  Prediction of vascular direction and donor livervolume by EDDA IQQA-liver system before liver transplantation

    图  2  患儿肝脏脏面大体观

    Figure  2.  General view of the visceral surface of the child's liver

    表  1  肝移植治疗CNS Ⅰ型患者的预后

    Table  1.   Prognosis of CNS Ⅰ patients treated with liver transplantation

    研究作者 年份 手术方式 原发病(n 存活率(%)
    1年 5年 10年 其他
    Adam R, et al[20] 2012 OLT、LDLT、ALT、DLT CNS(59) 88、97 79、92 79、92 79、92(15年)
    86、100 76、95
    Arnon R, et al[21] 2010 OLT、LDLT、ALT 代谢性肝病(446)包括CNS(21) 94.6 88.9
    Sze YK, et al[22] 2009 OLT、LDLT、ALT 代谢性肝病(96)包括CNS Ⅰ(11) 83、91 77、86 62、82
    Strauss KA, et al[23] 2006 OLT CNS Ⅰ(4)CNS Ⅱ(1) 100(16年)
    Ozçay F, et al[24] 2009 LDLT CNS Ⅰ(4) 75
    Morioka D, et al[25] 2005 LDLT 代谢性肝病(46)包括CNS Ⅰ(2) 86.9 81.2 81.2
    Morioka D, et al[26] 2005 LDLT 代谢性肝病(21)包括CNS Ⅰ(2) 85.7 85.7
    Valmiki S, et al[27] 2020 LDLT CNS Ⅰ(1) 100
    Rela M, et al[28] 1999 ALT CNS Ⅰ(6) 100
    Shanmugam NP, et al[29] 2019 ALT 代谢性肝病(13)包括CNS(5) 100 100
    Celik N, et al[30] 2019 DLT 代谢性肝病(4)包括CNS(2) 100
    注:①为1988年至2009年移植物、受者存活率。
    ②为1999年至2009年移植物、受者存活率。
    ③为移植物、受者存活率。
    ④—为无数据。
    下载: 导出CSV
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出版历程
  • 收稿日期:  2020-03-26
  • 网络出版日期:  2021-01-19
  • 刊出日期:  2020-07-15

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