Acute Deterioration of Idiopathic Portal Hypertension Requiring Living Donor Liver Transplantation: A Case Report
Acute Deterioration of Idiopathic Portal Hypertension Requiring Living Donor Liver Transplantation: A Case Report
复制标题
DOI：
10.1007/s10620-008-0504-7
复制
发表时间：
2009-07-01
影响因子：
3.1
通讯作者：
Kanematsu, Takashi
中科院分区：
文献类型：
--
作者：
Inokuma, Takamitsu;Eguchi, Susumu;Kanematsu, Takashi
严重的特发性门脉高压症（IPH）需要肝移植的病例报告非常罕见。我们报告的情况下，一个65岁的妇女谁被诊断为IPH。在60岁时，她最初的症状是呕血，由于食管静脉曲张破裂。腹部计算机断层扫描显示脾肿大和少量腹水，无肝硬化。她被诊断为患有IPH，并作为门诊患者进行随访。五年后，她出现了普通感冒的症状和迅速进行性腹胀。她被发现有严重的肝萎缩、肝功能障碍和大量腹水。然后进行了活体肝移植，她的术后病程顺利。肝脏组织学检查显示周围门静脉塌陷和狭窄。肝实质区域狭窄，而门脉束和中央静脉彼此接近，导致IPH的诊断。无肝硬化。在这种情况下，可以观察到难治性IPH的自然病程。由于严重IPH导致的终末期肝衰竭患者可以通过肝移植治疗。
Case reports of severe idiopathic portal hypertension (IPH) requiring liver transplantation are very rare. We report the case of a 65-year-old woman who was diagnosed as having IPH. At the age of 60 years, her initial symptom was hematemesis, due to ruptured esophageal varices. Computed tomography of the abdomen showed splenomegaly and a small amount of ascites, without liver cirrhosis. She was diagnosed as having IPH and followed-up as an outpatient. Five years later, she developed symptoms of a common cold and rapidly progressive abdominal distension. She was found to have severe liver atrophy, liver dysfunction, and massive ascites. Living donor liver transplantation was then performed, and her postoperative course was uneventful. Histopathological findings of the explanted liver showed collapse and stenosis of the peripheral portal vein. The areas of liver parenchyma were narrow, while the portal tracts and central veins were approximate one another, leading to a diagnosis of IPH. There was no liver cirrhosis. The natural history of refractory IPH could be observed in this case. Patients with end-stage liver failure due to severe IPH can be treated by liver transplantation.