Abstract:

Objective To discuss the clinical and pathological features of the rare cases with idiopathicnoncirrhotic portal hypertension（INCPH）who required orthotopic liver transplantation（OLT），along with thepostoperative complications. Methods Combined with the clinical and pathological data，the diagnosis and treatment of 2 cases with INCPH who underwent OLT at Zhongshan Hospital from December 2016 to June 2019 were analyzed retrospectively. To evaluate the curative effect of OLT，close laboratory examination and radiological follow-up were performed post-transplantation，liver biopsy was performed when necessary. Results The diagnosis of INCPH were histologically confirmed，and the surgeries went smoothly. After OLT，chronic rejection was found in onecase，and the disease recurred 1 year after the liver retransplantation. Cytomegalovirus infection，inferior vena cavalthrombosis and biliary tract complications were found in the other case. Conclusion INCPH can be a rare cause of end-stage liver disease requiring liver transplantation which is misdiagnosed frequently. Considering the limited relevant experiences to be referred to and the concerns about complications and recurrence. The efficacy of OLT forINCPH remains to be evaluated.

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摘要：

目的 探讨接受肝移植手术的特发性非肝硬化性门脉高压症（idiopathic noncirrhotic portalhypertension，INCPH）病例的临床、病理特征及术后并发症。方法 结合患者临床及病理资料，回顾性分析 2016 年 12 月 - 2019 年 6 月在复旦大学附属中山医院接受肝移植术的 2 例 INCPH 病例的诊断及治疗过程。术后密切进行实验室及影像学随访，必要时行肝穿刺活检，以评估肝移植的疗效。结果 2 例患者均经组织学确诊为 INCPH，肝移植手术完成顺利，术后 1 例患者出现慢性排斥并行再次肝移植，1 年后门脉高压复发；另一例患者术后出现巨细胞病毒感染、下腔静脉血栓形成及胆道并发症。 结论 INCPH 是种常被误诊的疾病，其中极少病例可导致终末期肝病并需要肝移植治疗。鉴于相关治疗经验较少及对并发症、疾病复发的担忧，肝移植治疗 INCPH 的疗效仍有待进一步评估。

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