肝移植治疗儿童朗格汉斯细胞组织细胞增多症导致的肝硬化:单中心经验  

Liver transplantation for langerhans cell histiocytosis in children with liver cirrhosis:a single-center experience

作　　者：陈天然 董冲[2] 孙超[2] 王凯[2] 张威[2] 杨洋[2] 张国锋 高伟[2] Chen Tianran;Dong Chong;Sun Chao;Wang Kai;Zhang Wei;Yang Yang;Zhang Guofeng;Gao Wei(The First Central Clinical School,Tianjin Medical University,Tianjin 300192,China;The Department of Children's Organ Transplantation,Tianjin First Central Hospital,Tianjin 300192,China)

机构地区：[1]天津医科大学一中心临床学院,天津300192 [2]天津市第一中心医院儿童器官移植科,天津300192

出　　处：《实用器官移植电子杂志》2023年第4期296-300,共5页Practical Journal of Organ Transplantation(Electronic Version)

摘　　要：目的探讨肝移植治疗儿童朗格汉斯细胞组织细胞增多症(langerhans cell histiocytosis,LCH)导致的肝硬化临床疗效及预后。方法描述性分析2017年1月至2023年3月天津市第一中心医院10例肝移植治疗LCH导致的肝硬化患儿的临床资料和诊疗经验。结果10例患儿手术前均处于LCH导致的肝硬化失代偿期,其中7例患儿行器官捐献肝移植术,3例患儿行活体肝移植术。7例患儿移植物类型为左外叶,2例患儿移植物类型为全肝,1例患儿移植物类型为右半肝,移植物受者体重比率(graft-to-recipient weight ratio,GRWR)范围为1.41%~4.53%,手术治疗后1~7 d患儿的丙氨酸转氨酶、天冬氨酸转氨酶、总胆红素逐渐降低并于出院前恢复至正常水平,中位随访时间为12.11个月,术后2例患儿出现肝外复发,分别给予化疗、靶向药物治疗后,患儿目前控制良好,术后总体生存率为90%。结论肝移植是治疗儿童LCH导致的肝硬化失代偿期的有效治疗方案,可以使患儿获得较好的生存受益。Objective To evaluate the clinical efficacy and prognosis of liver transplantation for langerhans cell histiocytosis(LCH)in children with liver cirrhosis.Methods The clinical data and treatment experience of 10 cases of liver transplantation for LCH in children with liver cirrhosis in the Tianjin First Central Hospital were descriptively analyzed.Results All children were in decompensated stage of liver cirrhosis caused by LCH before liver transplantation.Among the 10 LCH patients,7 patients underwent cadaveric liver transplantation,3 patients underwent living donor liver transplantation.The type of graft was left lateral lobe in 7 cases,whole liver in 2 cases,and right hemi-liver in 1 case.The graft to recipient weight ratio ranged from 1.41%to 4.53%.The values of alanine transaminase(ALT),aspartate aminotransferase(AST),and total bilirubin(TBil)started to decrease from the first postoperative day and continued with a downward daily trend in most recipients.The median followup time was 12.11 months.Two patients presented with extrahepatic recurrence and were well-controlled after the administration of chemotherapy and targeted drugs.The survival rate of the patients after liver transplantation was 90%.Conclusion Liver transplantation is an effective treatment for the decompensated stage of liver cirrhosis caused by LCH in children,a better survival outcome may be achieved.

关 键 词：朗格汉斯细胞组织细胞增多症 肝移植 儿童 

分 类 号：R726.5[医药卫生—儿科]