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小儿暴发性肝衰竭的肝移植

Liver transplantation for fulminant hepatic failure in the pediatric patient.

作者信息

Goss J A, Shackleton C R, Maggard M, Swenson K, Seu P, McDiarmid S V, Busuttil R W

机构信息

Dumont-UCLA Transplant Center, Department of Surgery, University of California School of Medicine, Los Angeles 90095, USA.

出版信息

Arch Surg. 1998 Aug;133(8):839-46. doi: 10.1001/archsurg.133.8.839.

Abstract

OBJECTIVE

To review the clinical characteristics, outcomes, and risk factors for survival among 57 pediatric patients undergoing orthotopic liver transplantation for fulminant hepatic failure at the University of California, Los Angeles, Center for the Health Sciences.

DESIGN

The medical records of 57 consecutive pediatric patients undergoing orthotopic liver transplantation for fulminant hepatic failure from July 1, 1984, to June 25, 1997, were reviewed and survival data were analyzed via univariate and multivariate statistical methods. The type and incidence of posttransplant complications were determined as was the quality of long-term graft function. Median follow-up period was 3.38 years (range, 0-10.02 years).

RESULTS

The 1-, 3-, and 5-year actuarial patient survival rates were 77%, 77%, and 77%, respectively, while graft survivals were 73%, 65%, and 65%. Stepwise Cox regression analysis revealed that recipient age and ventilator dependency at the time of transplantation were independently and significantly correlated with patient survival, whereas no association was found between survival and grade of encephalopathy, prior abdominal surgery, recipient weight, pretransplantation values for total bilirubin or prothrombin time, ABO match, allograft type, peak posttransplantation aspartate aminotransferase levels, or the presence of posttransplantation hepatic artery thrombosis. Non-ventilator-dependent patients demonstrated a 96% 1-, 3-, and 5-year survival as compared with only 56% at these same time points for those children requiring ventilator support at the time of transplantation (P < .001). At the time of most recent follow-up, median values for total bilirubin and aspartate aminotransferase concentrations were 10.3 micromol/L (0.6 mg/dL) and 56 U/L, respectively, in the 40 surviving patients.

CONCLUSIONS

In children undergoing liver transplantation for fulminant hepatic failure: (1) overall results are comparable to those achieved for less emergent non-neoplastic indications in this same age group; (2) ventilator dependency prior to transplantation is the strongest predictor of ultimate survival, followed by recipient age; (3) 5-year survival exceeds 90% in recipients who are ventilator independent immediately prior to liver transplantation but is significantly compromised once the need for mechanical ventilation supervenes, particularly in those younger than 4 years; and (4) prompt referral and timely liver replacement are the cornerstones of optimal outcome.

摘要

目的

回顾加利福尼亚大学洛杉矶分校健康科学中心57例因暴发性肝衰竭接受原位肝移植的儿科患者的临床特征、预后及生存危险因素。

设计

回顾1984年7月1日至1997年6月25日期间57例连续因暴发性肝衰竭接受原位肝移植的儿科患者的病历,并通过单因素和多因素统计方法分析生存数据。确定移植后并发症的类型和发生率以及长期移植物功能质量。中位随访期为3.38年(范围0至10.02年)。

结果

患者1年、3年和5年的精算生存率分别为77%、77%和77%,移植物生存率分别为73%、65%和65%。逐步Cox回归分析显示,移植时受者年龄和呼吸机依赖与患者生存独立且显著相关,而生存与肝性脑病分级、既往腹部手术、受者体重、移植前总胆红素或凝血酶原时间值、ABO血型匹配、同种异体移植物类型、移植后天冬氨酸转氨酶峰值水平或移植后肝动脉血栓形成之间未发现关联。不依赖呼吸机的患者1年、3年和5年生存率为96%,而移植时需要呼吸机支持的儿童在相同时间点的生存率仅为56%(P<0.001)。在最近一次随访时,40例存活患者的总胆红素和天冬氨酸转氨酶浓度中位数分别为10.3微摩尔/升(0.6毫克/分升)和56单位/升。

结论

对于因暴发性肝衰竭接受肝移植的儿童:(1)总体结果与同年龄组中不太紧急的非肿瘤性适应证所取得的结果相当;(2)移植前的呼吸机依赖是最终生存的最强预测因素,其次是受者年龄;(3)肝移植前立即不依赖呼吸机的受者5年生存率超过90%,但一旦需要机械通气,生存率会显著降低,尤其是4岁以下的儿童;(4)及时转诊和及时进行肝移植替代是获得最佳预后的基石。

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