劈离式肝移植治疗家族性高胆固醇血症  

Clinical insights on split liver transplantation for familial hypercholesterolemia

作　　者：童翾 汤睿[1] 李龙[2] 卢倩 Tong Xuan;Tang Rui;Li Long;Lu Qian(Department of Liver Transplantation,School of Clinical Medicine,Tsinghua Changgeng Hospital,Tsinghua University,Beijing 102218,China;Department of Pediatric Surgery,School of Clinical Medicine,Tsinghua Changgeng Hospital,Tsinghua University,Beijing 102218,China)

机构地区：[1]清华大学附属北京清华长庚医院清华大学临床医学院肝移植科,北京102218 [2]清华大学附属北京清华长庚医院清华大学临床医学院儿外科,北京102218

出　　处：《临床小儿外科杂志》2025年第3期226-230,共5页Journal of Clinical Pediatric Surgery

摘　　要：目的探讨劈离式肝移植治疗家族性高胆固醇血症的疗效与预后。方法本研究为单中心回顾性研究,以2020年9月至2022年3月北京清华长庚医院收治的5例诊断为家族性高胆固醇血症并行劈离式肝移植治疗的患儿为研究对象,其中男性1例、女性4例,中位年龄11岁(5~15岁)。收集其临床与随访资料,包括术前、术后1个月及末次随访时的低密度脂蛋白胆固醇(low-density lipoprotein cholesterol,LDL-C)、总胆固醇(total cholesterol,TC)以及移植术后相关并发症情况和生存情况。结果5例均获得随访,中位随访时间31个月(19~35个月)。术前LDL-C(16.31±2.24)mmol/L,术后1个月LDL-C降至(2.24±0.63)mmol/L,与术前LDL-C差异有统计学意义(P<0.001);术后末次随访时LDL-C(2.07±0.84)mmol/L,与术后1个月LDL-C差异无统计学意义(P=0.893)。术前TC为(18.13±2.27)mmol/L,术后1个月降至(3.79±0.35)mmol/L,与术前TC差异有统计学意义(P<0.001);术后末次随访时TC(3.62±0.92)mmol/L,与术后1个月差异无统计学意义(P=0.749)。5例术后均无需接受降脂治疗,无一例发生心血管并发症。结论劈离式肝移植可有效治疗家族性高胆固醇血症。Objective To explore the efficacy and prognosis of split liver transplantation(SLT)for familial hypercholesterolemia(FH).Methods A single-center retrospective study was conducted for 5 FH children undergoing SLT from September 2020 to March 2022.There were 4 girls and 1 boy with a median age of 11(5-15)year.Clinical and follow-up data were collected,including low-density lipoprotein cholesterol(LDL-C)and total cholesterol(TC)at pre-operation,Month 1 post-operation and the last follow-up,as well as the occurrence and survival of transplant-related postoperative complications.Results All of them were followed up completely with a median follow-up period of 31(19-35)month.LDL-C(16.31±2.24)mmol/L at pre-operation declined to(2.24±0.63)mmol/L at Month 1 post-operation.There was statistical significance(P<0.001).LDL-C was(2.07±0.84)mmol/L at the last follow-up showed no significant difference with that at Month 1 post-operation(P=0.893).Preoperative TC(18.13±2.27)mmol/L dropped to(3.79±0.35)mmol/L at Month 1 post-operation and the difference was statistically significant(P<0.001).No significant difference existed in TC(3.62±0.92)mmol/L at the last postoperative follow-up as compared with Month 1 post-operation(P=0.749).Postoperative lipid-lowering therapy was not required and there were no cardiovascular complications.Conclusions SLT may effectively treat familial hypercholesterolemia.

关 键 词：肝移植 高胆固醇血症 外科手术 儿童 

分 类 号：R726.5[医药卫生—儿科]