Hattori H, Higuchi Y, Tsuji M, Inomata Y, Uemoto S, Asonuma K, Egawa H, Kiuchi T, Furusho K, Yamaoka Y, Tanaka K
Department of Pediatrics, Kyoto University School of Medicine, Japan.
Transplantation. 1998 Mar 15;65(5):686-92. doi: 10.1097/00007890-199803150-00015.
Fulminant hepatic failure (FHF) in children is associated with high mortality under medical management. Living-related liver transplantation (LRLT) is an accepted measure to treat the children with end-stage liver disease. Reversibility of hepatic encephalopathy is crucial for the quality of life among the survivors after transplantation.
A retrospective review was made of the records of children undergoing LRLT at this hospital between May 1992 and November 1996.
Eleven children with FHF underwent emergency LRLT. The mean age was 5 years (range, 2 months to 15 years). The indication for transplantation was persistent or worsening hepatic encephalopathy and severe coagulopathy, despite repeated plasma exchanges or exchange transfusions. The cause of FHF was non-A, non-B hepatitis in seven children, hepatitis B in two children, herpes simplex virus hepatitis in one child, and fulminant Wilson's disease with intravascular hemolysis in one child. The grade of hepatic encephalopathy was II in four children, III in two, and IV in five. The actuarial survival rate was 73% after a mean follow-up of 28 months (range, 13-67 months). Short-term neurological morbidity was present in two children with grade IV encephalopathy who also showed brain edema on cranial computed tomography. Eight survivors exhibited no long-term neurological deficit; the mean intelligence or developmental quotient was 97 (range, 86-110) at the end of the follow-up period.
LRLT is an effective option for the treatment of FHF in children. The long-term neurological status is satisfactory among survivors.
儿童暴发性肝衰竭（FHF）在药物治疗下死亡率很高。亲属活体肝移植（LRLT）是治疗儿童终末期肝病的一种公认措施。肝性脑病的可逆性对于移植后幸存者的生活质量至关重要。
对1992年5月至1996年11月在本院接受LRLT的儿童记录进行回顾性分析。
11例FHF患儿接受了急诊LRLT。平均年龄为5岁（范围2个月至15岁）。移植指征为尽管反复进行血浆置换或换血输血，但仍存在持续性或进行性肝性脑病及严重凝血障碍。FHF的病因在7例患儿中为非甲非乙型肝炎，2例为乙型肝炎，1例为单纯疱疹病毒性肝炎，1例为伴有血管内溶血的暴发性威尔逊病。肝性脑病分级为Ⅱ级的有4例患儿，Ⅲ级的有2例，Ⅳ级的有5例。平均随访28个月（范围13 - 67个月）后的精算生存率为73%。2例Ⅳ级脑病患儿出现短期神经功能障碍，其头颅计算机断层扫描显示有脑水肿。8名幸存者无长期神经功能缺损；随访期末平均智力或发育商为97（范围86 - 110）。
LRLT是治疗儿童FHF的有效选择。幸存者的长期神经状态令人满意。
