Tanaka K, Uemoto S, Tokunaga Y, Fujita S, Sano K, Yamamoto E, Sugano M, Awane M, Yamaoka Y, Kumada K
Second Department of Surgery, Faculty of Medicine, Kyoto University, Japan.
Am J Surg. 1994 Jul;168(1):41-8. doi: 10.1016/s0002-9610(05)80069-8.
We reviewed 37 living related liver transplantations (LRLT) performed by our department during the last 27 months on children with end-stage liver disease. The patients were 15 boys and 22 girls aged 7 months to 15 years with biliary atresia (27), cryptogenic cirrhosis (3), Budd-Chiari syndrome (2), progressive intrahepatic cholestasis (2), protoporphyria (1), Wilson's disease (1), and fulminant hepatitis (1). The donors were 14 fathers and 23 mothers. Grafts were made from the left lateral segment (19), left lateral segment with partial S4 (11), left lobe (6), and right lobe (1). After graft harvesting all donors resumed normal liver function and normal life. The recipient underwent total hepatectomy with preservation of the inferior vena cava. FK506 and low-dose steroids were used for immunosuppression. The survival rate was 90% (27/30) in elective cases and 57% (4/7) in emergency cases. Six recipients had functioning grafts but died of extrahepatic complications. Hepatic vein stenosis occurred in 3 cases at 3 months after LRLT and was successfully treated by balloon dilatation. Portal vein stenosis occurred in 1 case at 8 months after LRLT and was also safely dilated. We incurred no hepatic artery thrombosis after introducing microsurgery techniques. Among 12 viral, 5 bacterial, and 3 fungal postoperative infections, 1 Candida pneumonia and 1 EBV-associated lymphoma were lethal. Three patients with ABO-blood group compatible grafts and one with an incompatible graft developed acute rejection, which was controlled in evey case by steroid bolus and/or increasing the dose of FK506. There were no definite episodes of rejection in ABO-identical cases. Children with moderate growth retardation (> or = -1.5 SD of normal growth) caught up in growth soon after LRLT, but those with severe retardation (<-1.5 SD) were slow to attain age-normal height. Appropriate timing, meticulous surgical procedures, and comprehensive management of complications are crucial for successful outcome with LRLT. LRLT is a promising option for alleviating the shortage of livers for pediatric transplantation and may be regarded as an independent modality to supplement cadaver donation.
我们回顾了本部门在过去27个月里为患有终末期肝病的儿童进行的37例活体亲属肝移植（LRLT）。患者年龄在7个月至15岁之间，其中男孩15名，女孩22名，病因包括胆道闭锁（27例）、隐源性肝硬化（3例）、布加综合征（2例）、进行性肝内胆汁淤积（2例）、原卟啉病（1例）、威尔逊病（1例）和暴发性肝炎（1例）。供体包括14名父亲和23名母亲。移植肝脏取材于左外叶（19例）、含部分S4的左外叶（11例）、左叶（6例）和右叶（1例）。供体肝脏切取后均恢复正常肝功能及正常生活。受体接受保留肝下腔静脉的全肝切除术。采用FK506和小剂量类固醇进行免疫抑制。择期手术患者的生存率为90%（27/30），急诊手术患者的生存率为57%（4/7）。6例受体移植肝脏功能良好，但死于肝外并发症。肝移植术后3个月，3例发生肝静脉狭窄，经球囊扩张成功治疗。肝移植术后8个月，1例发生门静脉狭窄，也成功进行了扩张。引入显微外科技术后未发生肝动脉血栓形成。术后12例病毒感染、5例细菌感染和3例真菌感染中，1例念珠菌肺炎和1例EBV相关淋巴瘤致死。3例ABO血型相容移植和1例不相容移植的患者发生急性排斥反应，均通过静脉推注类固醇和/或增加FK506剂量得到控制。ABO血型相同的病例未发生明确的排斥反应。中度生长发育迟缓（≥正常生长标准差-1.5）的儿童在肝移植后生长迅速追赶，但重度生长发育迟缓（<-1.5标准差）的儿童达到正常身高的速度较慢。合适的时机、细致的手术操作和并发症的综合管理对于活体亲属肝移植的成功结局至关重要。活体亲属肝移植是缓解小儿肝移植供肝短缺的一个有前景的选择，可被视为补充尸体供肝的一种独立方式。
