Chen Xiaojie, Lu Yida, Wei Lin, Sun Li-Ying, Zeng Zhigui, Qu Wei, Liu Ying, Zhu Zhijun
Liver Transplantation Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Clinical Center for Pediatric Liver Transplantation, Capital Medical University, Beijing, China.
Ann Transplant. 2025 Apr 29;30:e947639. doi: 10.12659/AOT.947639.
BACKGROUND Adult polycystic liver disease (PLD) is a rare disorder frequently associated with polycystic kidney disease (PKD). This study aimed to evaluate the therapeutic outcomes of organ transplantation in patients with PLD. MATERIAL AND METHODS A retrospective analysis was conducted on the clinical data of 9 PLD patients who underwent organ transplantation at our center from May 2015 to Jan 2024. Intraoperative conditions and postoperative complications were closely monitored and documented. The survival rates of recipients and grafts, the use of immunosuppressants in recipients, and graft function were all monitored. RESULTS All 9 patients were female, with a mean age of 51.6±7.9 years. Among them, 8 had PLD combined with polycystic kidney disease (PKD), and 1 had PLD with left renal cysts. Among the 9 patients, 7 underwent combined liver and kidney transplantation (including 2 sequential liver and kidney transplantation), while 2 underwent liver transplantation alone. Two patients developed liver graft rejection postoperatively, and 1 patient developed kidney stones and post-transplant lymphoproliferative disease (PTLD). One patient who underwent sequential liver-kidney transplantation died 135 days after kidney transplantation due to severe infection. The median follow-up time for the surviving patients was 45.0 months (range 16.0 to 108.4 months). The survival rate was 88.9%. Among the 6 surviving patients who underwent combined liver and kidney transplantation, the preoperative estimated glomerular filtration rate (eGFR) was 19.8±16.4 mL/min, while the postoperative follow-up eGFR was 64.2±12.3 mL/min. CONCLUSIONS Organ transplantation provides a reliable solution for patients with PLD and end-stage renal failure.
背景 成人多囊肝病（PLD）是一种罕见疾病，常与多囊肾病（PKD）相关。本研究旨在评估PLD患者器官移植的治疗效果。
材料与方法 对2015年5月至2024年1月在本中心接受器官移植的9例PLD患者的临床资料进行回顾性分析。密切监测并记录术中情况和术后并发症。监测受者和移植物的生存率、受者免疫抑制剂的使用情况以及移植物功能。
结果 9例患者均为女性，平均年龄51.6±7.9岁。其中，8例为PLD合并多囊肾病（PKD），1例为PLD合并左肾囊肿。9例患者中，7例接受了肝肾联合移植（包括2例序贯肝肾移植），2例仅接受了肝移植。2例患者术后发生肝移植排斥反应，1例患者出现肾结石和移植后淋巴细胞增殖性疾病（PTLD）。1例接受序贯肝肾移植的患者在肾移植后135天因严重感染死亡。存活患者的中位随访时间为45.0个月（范围16.0至108.4个月）。生存率为88.9%。在6例存活的接受肝肾联合移植的患者中，术前估计肾小球滤过率（eGFR）为19.8±16.4 mL/min，而术后随访eGFR为64.2±12.3 mL/min。
结论 器官移植为PLD和终末期肾衰竭患者提供了可靠的治疗方案。
