PDF(Pubmed)
Abstract：
Acute cellular rejection (ACR) after pediatric living donor liver transplantation (LDLT) is often curable with steroid pulse therapy, but a few pediatric patients show steroid-resistant ACR, which is difficult to control. We report the effect of everolimus as a rescue therapy for ACR in a case of pediatric LDLT. The patient was a 11-year-old girl who was admitted due to subacute liver failure of unknown cause. LDLT operation using a modified right liver graft from her mother was performed. The graft-recipient weight ratio was 1.30. The explant liver showed massive hepatic necrosis. The patient recovered uneventfully with immunosuppression using tacrolimus and low-dose steroid. However, at postoperative day (POD) 20, the liver enzyme levels began to increase. The first liver biopsy taken at POD 25 showed moderate ACR with rejection activity index (RAI) score of 7. At that time, steroid pulse therapy was performed, but the patient did not respond and the liver enzyme levels increased further. The second liver biopsy taken at POD 40 showed moderate ACR with RAI score of 7. At this time, everolimus was administered, and soon after that, liver enzyme levels had gradually improved. Currently, the patient is doing well for 44 months to date without any abnormal findings. The maintenance target trough concentrations were tacrolimus 5 ng/ml and everolimus 3 ng/ml. Our case demonstrated the effect of rescue therapy using everolimus for ACR following pediatric LDLT. Further studies are needed to assess the role of everolimus in pediatric liver transplant recipients suffering from ACR.
摘要：
小儿活体肝移植（LDLT）后的急性细胞排斥反应（ACR）通常可以通过类固醇脉冲疗法治愈。但是一些儿科患者表现出类固醇耐药的ACR，这是难以控制的。我们报告了依维莫司在小儿LDLT病例中作为ACR的抢救疗法的效果。该患者是一名11岁的女孩，由于原因不明的亚急性肝功能衰竭而入院。使用母亲的改良右肝移植进行了LDLT手术。移植物-受体重量比为1.30。外植体肝脏显示大量肝坏死。患者使用他克莫司和低剂量类固醇进行免疫抑制后恢复顺利。然而,在术后第20天（POD），肝酶水平开始增加。在POD25进行的第一次肝活检显示中度ACR，排斥活动指数（RAI）评分为7。当时,进行了类固醇脉冲治疗，但患者没有反应,肝酶水平进一步升高.在POD40处进行的第二次肝活检显示中度ACR，RAI评分为7。此时,服用依维莫司，在那之后不久,肝酶水平逐渐改善。目前,到目前为止，患者在44个月内表现良好，没有任何异常发现。维持目标谷浓度为他克莫司5ng/ml和依维莫司3ng/ml。我们的病例证明了在小儿LDLT后使用依维莫司进行ACR的抢救治疗的效果。需要进一步的研究来评估依维莫司在患有ACR的小儿肝移植受者中的作用。
