Adrian-Casavilla F, Reyes J, Tzakis A, Wright H I, Gavaler J S, Lendoire J, Gordon R, Starzl T E, Van Thiel D H
Department of Surgery, University of Pittsburgh School of Medicine, PA.
J Hepatol. 1994 Dec;21(6):1035-9. doi: 10.1016/s0168-8278(05)80614-2.
Neonatal hepatitis is a syndrome of unknown etiology occurring in children with viral liver disease, as well as children with unidentified disorders of bile salt synthesis and other poorly understood metabolic diseases. It is characterized by jaundice, giant cell hepatitis and rare liver failure necessitating liver transplantation. In the present investigation, the outcome of liver transplantation performed in 16 children with neonatal hepatitis at the investigators' institution was determined from 1 January 1989 to 31 December 1991. The results were compared to those obtained in 288 children transplanted for biliary atresia and 66 children transplanted for recognized metabolic liver disease. The children transplanted for neonatal hepatitis (4.1 +/- 1.3 years) and metabolic liver disease (5.8 +/- 0.6 years) were older than those transplanted for biliary atresia (3.3 +/- 0.2 years) (p < 0.01), but did not differ in terms of sex, ABO type, UNOS status or year in which the transplant procedure was performed. Interestingly, first allograft survival was equal in the children with neonatal hepatitis (74%) and those with metabolic liver disease (74%), but was greater than that for children transplanted for biliary atresia (68%) (p < 0.01). Despite this significant difference in first graft survival, no differences in 5-year survival were seen for the three groups (81% for neonatal hepatitis, 68% for biliary atresia and 79% for metabolic liver disease).(ABSTRACT TRUNCATED AT 250 WORDS)
新生儿肝炎是一种病因不明的综合征,发生于患有病毒性肝病的儿童,以及胆汁盐合成不明紊乱和其他了解甚少的代谢性疾病的儿童。其特征为黄疸、巨细胞肝炎,以及罕见的需要进行肝移植的肝衰竭。在本研究中,对1989年1月1日至1991年12月31日在研究机构为16例新生儿肝炎患儿进行肝移植的结果进行了测定。将结果与288例因胆道闭锁接受移植的儿童以及66例因确诊的代谢性肝病接受移植的儿童的结果进行了比较。因新生儿肝炎接受移植的儿童(4.1±1.3岁)和因代谢性肝病接受移植的儿童(5.8±0.6岁)比因胆道闭锁接受移植的儿童(3.3±0.2岁)年龄大(p<0.01),但在性别、ABO血型、器官共享联合网络(UNOS)状态或进行移植手术的年份方面无差异。有趣的是,新生儿肝炎患儿(74%)和代谢性肝病患儿(74%)的首次同种异体移植物存活率相同,但高于因胆道闭锁接受移植的儿童(68%)(p<0.01)。尽管首次移植物存活率存在显著差异,但三组的5年生存率无差异(新生儿肝炎组为81%,胆道闭锁组为68%,代谢性肝病组为79%)。(摘要截取自250词)