Vázquez J, Gámez M, Santamaría M L, Murcia J, Díaz M C, Camarena C, Jara P, Tovar J A
Department of Pediatric Surgery, Children's Hospital La Paz, Madrid, Spain.
J Pediatr Surg. 1993 Aug;28(8):1051-3. doi: 10.1016/0022-3468(93)90517-o.
Pediatric liver transplantation is an effective treatment for end-stage liver disease with 1- and 5-year survivals approaching 90% and 70%, respectively. Survival is influenced by the recipient's age, weight, primary disease, vascular malformations, and nutritional status. Younger patients weighing less than 13 kg are considered to be a high-risk group. The aim of this article is to evaluate the impact of this group of patients on the overall results of our pediatric liver transplant program. From January 1986 through January 1992 we performed 76 liver transplants in 59 pediatric patients. Sixteen received a second graft and a third was required in one. Fourteen patients weighed less than 13 kg (mean, 11 kg; range, 6 to 13 kg). Their mean age was 12 months, with a range of 8 to 36 months. Indications for transplantation were: biliary atresia (9), Byler's disease (1), tyrosinemia (3), and alpha 1-antitrypsin deficiency (1). The incidence of rejection in this group (52%) was not significantly different from that in other patients (61%). Ten episodes of acute rejection required only steroids: in one monoclonal antibodies were added. Five patients had a new graft implanted, four for hepatic artery thrombosis and one for primary liver nonfunction. Nine patients are alive (64%) with the follow-up time ranging from 2 to 56 months (mean, 31). Five patients died of multiorgan failure (3), portal vein thrombosis (1), and primary liver nonfunction (1). Four-year graft and patient survival rates were 47% and 64%, respectively. Small babies are a high-risk group in a pediatric liver transplant program.(ABSTRACT TRUNCATED AT 250 WORDS)
小儿肝移植是治疗终末期肝病的有效方法,1年和5年生存率分别接近90%和70%。生存率受受者年龄、体重、原发性疾病、血管畸形和营养状况影响。体重小于13千克的年轻患者被视为高危组。本文旨在评估这组患者对我们小儿肝移植项目总体结果的影响。1986年1月至1992年1月,我们对59例小儿患者进行了76例肝移植。16例接受了第二次移植,1例需要第三次移植。14例患者体重小于13千克(平均11千克;范围6至13千克)。他们的平均年龄为12个月,范围为8至36个月。移植指征为:胆道闭锁(9例)、比勒氏病(1例)、酪氨酸血症(3例)和α1-抗胰蛋白酶缺乏症(1例)。该组排斥反应发生率(52%)与其他患者(61%)无显著差异。10次急性排斥反应仅需使用类固醇治疗:1例加用了单克隆抗体。5例患者植入了新移植物,4例因肝动脉血栓形成,1例因原发性肝功能衰竭。9例患者存活(64%),随访时间为2至56个月(平均31个月)。5例患者死于多器官功能衰竭(3例)、门静脉血栓形成(1例)和原发性肝功能衰竭(1例)。4年移植物和患者生存率分别为47%和64%。在小儿肝移植项目中,小婴儿是高危组。(摘要截稿于250词)