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Long-Term Outcome of Veno-Occlusive Disease After Liver Transplant: A Retrospective Single-Center Experience

机译:肝移植后静脉闭塞性疾病的长期结果:回顾性单中心经验

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Objectives: Veno-occlusive disease after liver trans-plant has been sporadically reported, and significant uncertainty exists concerning the best treatment and the long-term outcomes. Here, we reviewed our experience to evaluate clinical presentation, treat-ment, and the long-term outcomes of these patients. Materials and Methods: Between 2000 and 2015, 2165 patients underwent liver transplant at our center. The incidence of veno-occlusive disease was 0.3% (7/2165). Results: Timing of veno-occlusive disease onset (median 4.7 mo; interquartile range, 2.5-11.1 mo) varied widely as did clinical presentation, which was characterized by a variable association of liver failure and portal hypertension and different disease pro-gression rates. In all cases, diagnosis of veno-occlusive disease was confirmed by liver biopsy. Six patients (85.7%) presented with veno-occlusive disease after a previous episode of acute cellular rejection. Three patients died due to veno-occlusive disease (n = 2) or due to hepatocellular carcinoma recurrence (n = 1). Two patients were treated by increasing immunosup-pression and with interventional pro-cedures (pleurodesis and transjugular intrahepatic portosystemic shunt, respectively), and 2 had successful retransplants. 5-year patient and graft survival rates were 57.1% and 28.6%, respectively. Conclusions: A tailored approach based on clinical features and including retransplant can achieve acceptable long-term survival in patients with veno-occlusive disease after liver transplant.
机译:目的:零星报道了肝移植后的静脉闭塞性疾病,关于最佳治疗和长期结果存在重大不确定性。在这里,我们回顾了我们的经验,以评估这些患者的临床表现,治疗和长期结果。材料与方法:2000年至2015年,我们中心有2165例患者接受了肝移植。静脉阻塞性疾病的发生率为0.3%(7/2165)。结果:静脉闭塞性疾病发作的时间(中位4.7 mo;四分位间距为2.5-11.1 mo)和临床表现差异很大,其特征是肝功能衰竭和门脉高压的可变关联以及不同的疾病进展率。在所有情况下,肝活检均证实诊断为静脉闭塞性疾病。先前发生急性细胞排斥反应后,有6名患者(85.7%)出现静脉阻塞性疾病。三例患者死于静脉闭塞性疾病(n = 2)或肝细胞癌复发(n = 1)。 2例患者通过增加免疫抑制和介入程序(分别为胸膜固定术和经颈静脉肝内门体分流术)进行了治疗,其中2例成功进行了移植。 5年患者和移植物存活率分别为57.1%和28.6%。结论:基于临床特点的量身定制方法(包括再移植)可以使肝移植后静脉阻塞性疾病的患者获得可接受的长期生存。

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