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马方综合征来源供肾肾移植2例并文献复习

张盟, 王亦斌, 王於尘, 等. 马方综合征来源供肾肾移植2例并文献复习[J]. 器官移植. doi: 10.3969/j.issn.1674-7445.2023239
引用本文: 张盟, 王亦斌, 王於尘, 等. 马方综合征来源供肾肾移植2例并文献复习[J]. 器官移植. doi: 10.3969/j.issn.1674-7445.2023239
Zhang Meng, Wang Yibin, Wang Yuchen, et al. Kidney transplantation from donors with Marfan syndrome: report of 2 cases and literature review[J]. ORGAN TRANSPLANTATION. doi: 10.3969/j.issn.1674-7445.2023239
Citation: Zhang Meng, Wang Yibin, Wang Yuchen, et al. Kidney transplantation from donors with Marfan syndrome: report of 2 cases and literature review[J]. ORGAN TRANSPLANTATION. doi: 10.3969/j.issn.1674-7445.2023239

马方综合征来源供肾肾移植2例并文献复习

doi: 10.3969/j.issn.1674-7445.2023239
基金项目: 国家自然科学基金(82270784、82070770);广东省基础与应用基础研究基金(2023A1515012276);广东省学位与研究生教育创新计划项目(2022JDXM031)
详细信息
    作者简介:
    通讯作者:

    苗芸(ORCID 0000-0003-3592-4695),博士,主任医师,研究方向为肾移植,Email:miaoyunecho@126.com

  • 中图分类号: R617, R692, R596

Kidney transplantation from donors with Marfan syndrome: report of 2 cases and literature review

More Information
  • 摘要:   目的  探讨马方综合征(MFS)患者供肾肾移植的可行性及临床经验。  方法  回顾性分析接受同一MFS患者供肾的2例受者临床资料及既往文献中2例相关报道,总结MFS患者供肾肾移植的特点和临床诊疗要点。  结果  该MFS患者左、右侧供肾零点穿刺Remuzzi评分分别为1分、2分,肾内小动脉壁与其他脑死亡及心脏死亡供肾相比无明显差异。接受该MFS患者肾脏的2例受者术后均发生移植肾功能延迟恢复,短暂血液透析后,左肾受者、右肾受者的移植肾功能分别于术后10 d和20 d起开始逐渐恢复。出院后左肾受者的血清肌酐稳定于80~90 μmol/L,右肾受者的血清肌酐仍在下降,截止至投稿日,血清肌酐最低为232 μmol/L(术后43 d)。既往文献中报道了2例成功使用同一MFS患者供肾的肾移植案例,2例受者均发生了移植肾功能延迟恢复,而后肾功能均恢复正常,截止至报道日期,其中1例受者持续存活了6年,另外1例受者于术后第2年因新发脑血管疾病而死亡。  结论  MFS患者是可接受的供肾来源,但术前应审慎评估受者意愿和一般状况,术中妥善处理可能的肾动脉中膜撕裂,术后警惕各类并发症的发生。

     

  • 图  1  供者胸部CT表现

    注:供者CT示主动脉夹层,为Stanford A型,夹层累及升主动脉(红色箭头所示)。

    Figure  1.  Chest CT manifestations of the donor

    图  2  供肾零点穿刺活检的病理表现(糖原染色,×400)

    注:A图示左肾未见肾小球球性硬化,未见肾小球节段性硬化,可见肾小管萎缩;B图示右肾可见1个肾小球球性硬化,未见肾小球节段性硬化。

    Figure  2.  Pathological findings of time-zero biopsy of the donor kidney

    图  3  MFS供者与正常供者的血管病理切片(Verhoeff's Van Gieson染色,×200)

    注:A图为正常供者髂动脉;B图为MFS供者髂动脉,可见中膜弹力纤维排列紊乱,有较多断裂的弹力纤维(红色箭头);C图为MFS供者肾穿刺组织的小动脉未见明显病变(红色箭头)。

    Figure  3.  The vascular pathological sections of the MFS donor and normal donor

    图  4  左侧供肾受者术后尿量及血清肌酐变化趋势

    Figure  4.  Trends of postoperative urinary volume and serum creatinine in the recipient received the left donor kidney

    图  5  左侧供肾受者术后移植肾及移植肾血管彩超结果

    注:A图为术后1 d移植肾及移植肾血管彩超示肾内血流信号欠佳,肾动脉血流速度偏低;B图为术后12 d移植肾及移植肾血管彩超示肾内血流丰富,血管走行显示清晰。

    Figure  5.  Color Doppler ultrasound results of postoperative renal allograft and renal allograft vessels in the recipient received the left donor kidney

    图  6  右侧供肾受者术后尿量及血清肌酐变化趋势

    Figure  6.  Trends in postoperative urinary volume and serum creatinine in the recipient received the right donor kidney

    图  7  右侧供肾受者术后移植肾及移植肾血管彩超结果

    注:A图为术后1 d移植肾及移植肾血管彩超示肾内血流信号欠佳;B图为术后12 d移植肾及移植肾血管彩超示肾内血流丰富,血管走行显示清晰。

    Figure  7.  Color Doppler ultrasound results of postoperative renal allograft and renal allograft vessels in the recipient received the right donor kidney

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出版历程
  • 收稿日期:  2023-11-07
  • 录用日期:  2023-12-25
  • 网络出版日期:  2024-01-05

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