马方综合征来源供肾肾移植2例并文献复习  

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

作　　者：张盟 王亦斌[1] 王於尘 刘如敏[1] 严紫嫣 夏仁飞[1] 曾文利[1] 惠佳亮 周敏捷[1] 徐健[1] 苗芸[1] Zhang Meng;Wang Yibin;Wang Yuchen;Liu Rumin;Yan Ziyan;Xia Renfei;Zeng Wenli;Hui Jialiang;Zhou Minjie;Xu Jian;Miao Yun(Department of Organ Transplantation,Nanfang Hospital of Southern Medical University,Guangzhou 510515,China)

机构地区：[1]南方医科大学南方医院器官移植科,广州510515

出　　处：《器官移植》2024年第2期257-262,共6页Organ Transplantation

基　　金：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)。

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物功能延迟恢复,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80~90μmol/L,右肾受者的血清肌酐仍在下降,截至投稿日,血清肌酐最低为232μmol/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物功能延迟恢复,而后肾功能均恢复正常,截至报道日期,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。Objective To investigate the feasibility and clinical experience of kidney transplantation from donors with Marfan syndrome(MFS).Methods Clinical data of 2 recipients undergoing kidney transplantation from the same MFS patient were retrospectively analyzed and literature review of 2 cases was conducted.Characteristics and clinical diagnosis and treatment of kidney transplantation from MFS patients were summarized.Results The Remuzzi scores of the left and right donor kidneys of the MFS patient during time-zero biopsy were 1 and 2.No significant difference was observed in the renal arteriole wall compared with other donors of brain death and cardiac death.Two recipients who received kidney transplantation from the MFS patient suffered from postoperative delayed graft function.After short-term hemodialysis,the graft function of the recipients received the left and right kidney began to gradually recover at postoperative 10 d and 20 d.After discharge,serum creatinine level of the recipient received the left kidney was ranged from 80 to 90 μmol/L,whereas that of the recipient received the right kidney kept declining,and the lowest serum creatinine level was 232 μmol/L before the submission date(at postoperative 43 d).Through literature review,two cases successfully undergoing kidney transplantation from the same MFS donor were reported.Both two recipients experienced delayed graft function,and then renal function was restored to normal.Until the publication date,1 recipient has survived for 6 years,and the other recipient died of de novo cerebrovascular disease at postoperative 2 years.Conclusions MFS patients may serve as an acceptable source of kidney donors.However,the willingness and general conditions of the recipients should be carefully evaluated before kidney transplantation.Intraoperatively,potential risk of tear of renal arterial media should be properly treated.Extensive attention should be paid to the incidence of postoperative complications.

关 键 词：肾移植 马方综合征 遗传性疾病 动脉夹层 移植物功能延迟恢复 原纤维蛋白1 活组织检查 Remuzzi评分 

分 类 号：R617[医药卫生—外科学] R692[医药卫生—临床医学] R596