Bahador A, Salahi H, Nikeghbalian S, Dehghani S M, Dehghani M, Kakaei F, Kazemi K, Rajaei E, Gholami S, Malek-Hosseini S A
Department of Hepatobiliary and Transplantation Surgery, Shiraz Transplant Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Transplant Proc. 2009 Sep;41(7):2864-7. doi: 10.1016/j.transproceed.2009.07.046.
Liver transplantation (OLT) is accepted as the standard therapy for end-stage liver disease. The current shortage of organ donors has led to the use of split grafts and living related donors to provide timely liver transplants for these children. Herein we have reported our experience with pediatric OLT over a 9-year period.
We retrospectively studied 138 infants and children who underwent OLT from April 1999 to August 2008 including pretransplantation status, medical and surgical complications, and survival.
There were 83 (60.1%) boys and 55 (39.9%) girls. The mean patient age was 9.1 +/- 5.6 years (range = 0.5-18) with a mean weight of 28.1 +/- 17.0 kg (range = 7-80). The main indications were Wilson's disease (20.3%); cryptogenic cirrhosis (16.7%); autoimmune cirrhosis (14.5%); biliary atresia (13.8%); tyrosinemia (9.4%); and progressive familial intrahepatic cholestasis (8.7%). We used living related donors in 54 (39.1%) and split livers in 20 (14.5%) cases with 64 (46.4%) patients receiving a whole liver from a deceased donor. The mean follow-up was 25.3 +/- 20.3 months (range = 1-100). The mortality rate was 27.5% with a 26.1% in-hospital mortality. The main causes of mortality were vascular complications (32.6%); primary nonfunction (19.6%); sepsis (17.4%); chronic rejection (17.4%); and biliary complications (6.5%). The mortality rate among patients under 10 kg (58.8%) was higher than that of patients over 10 kg (23.1%). Among those patients who were discharged from the hospital (73.9%), the most common cause of mortality was chronic rejection from noncompliance (n = 4), chronic rejection (n = 3 cases), or posttransplant lymphoproliferative disease (n = 2).
Our results demonstrated that pediatric OLT is a feasible undertaking in Iran. The organ shortage in our area led to liberal use of living related and split-liver techniques. The overall results of pediatric OLT in Iran were acceptable.
肝移植（OLT）被公认为终末期肝病的标准治疗方法。目前器官供体短缺，导致采用劈裂式移植物和活体亲属供体为这些儿童及时进行肝移植。在此，我们报告了9年来小儿肝移植的经验。
我们回顾性研究了1999年4月至2008年8月期间接受肝移植的138例婴幼儿和儿童，内容包括移植前状况、医疗和手术并发症以及生存率。
有83名（60.1%）男孩和55名（39.9%）女孩。患者平均年龄为9.1±5.6岁（范围=0.5 - 18岁），平均体重为28.1±17.0千克（范围=7 - 80千克）。主要适应证为威尔逊病（20.3%）；隐源性肝硬化（16.7%）；自身免疫性肝硬化（14.5%）；胆道闭锁（13.8%）；酪氨酸血症（9.4%）；以及进行性家族性肝内胆汁淤积症（8.7%）。我们在54例（39.1%）中使用了活体亲属供体，20例（14.5%）中使用了劈裂肝脏，64例（46.4%）患者接受了来自已故供体的全肝。平均随访时间为25.3±20.3个月（范围=1 - 100个月）。死亡率为27.5%，住院死亡率为26.1%。死亡的主要原因是血管并发症（32.6%）；原发性无功能（19.6%）；败血症（17.4%）；慢性排斥反应（17.4%）；以及胆道并发症（6.5%）。体重低于10千克的患者死亡率（58.8%）高于体重超过10千克的患者（23.1%）。在出院的患者中（73.9%），最常见的死亡原因是因不依从导致的慢性排斥反应（n = 4）、慢性排斥反应（n = 3例）或移植后淋巴细胞增生性疾病（n = 2）。
我们的结果表明小儿肝移植在伊朗是一项可行的工作。我们地区的器官短缺导致广泛使用活体亲属和劈裂肝脏技术。伊朗小儿肝移植的总体结果是可以接受的。
