Petrowsky H, Allers C, Herrmann G, Jacobi V, Wenisch H J, Markus B H
Klinik für Allgemeinchirurgie, Johann Wolfgang Goethe-Universität, Frankfurt am Main.
Z Gastroenterol. 1997 May;35(5):331-6.
Idiopathic portal hypertension (IPH) was diagnosed in a 30-year-old man. Clinical signs were splenomegaly, leucothrombocytopenia, and esophageal varices of fourth degree. The histology of the liver biopsy showed portal fibrosis with no evidence of cirrhosis. No causing agent or known disease could be found for the histopathological and clinical features. Due to a severe deterioration of general condition and a decline of synthetic liver function, liver transplantation and splenectomy were performed. The histological examination of the explanted liver revealed features of IPH, demonstrating portal fibrosis and dilated vessels adjacent to portal tracts; no cirrhosis was found. The postoperative recovery was without any severe complications. The duration of hospitalization was 28 days. Following liver transplantation, the esophageal varices disappeared and leucocytes, platelets as well as parameters of hepatic synthesis reached normal values. Initially, the immunosuppression was composed of prednisolon, tacrolimus, and antibodies against IL-2 receptors (BT 563) and was later continued with prednisolon and tacrolimus. Within the follow-up observation of 26 months, there was no evidence for graft rejection, severe infection, or occurrence of portal hypertension. Up till now the patient is in good condition with normal graft function. Liver transplantation may be a curative therapy for patients with advanced disease of IPH but the long-term follow-up after transplantation has to show whether IPH can reoccur.
一名30岁男性被诊断为特发性门静脉高压症（IPH）。临床症状为脾肿大、白细胞血小板减少以及四度食管静脉曲张。肝脏活检组织学显示门静脉纤维化，无肝硬化证据。针对组织病理学和临床特征，未发现致病因素或已知疾病。由于全身状况严重恶化以及肝脏合成功能下降，进行了肝移植和脾切除术。切除肝脏的组织学检查显示为IPH特征，表现为门静脉纤维化以及门静脉周围血管扩张；未发现肝硬化。术后恢复过程中无任何严重并发症。住院时间为28天。肝移植后，食管静脉曲张消失，白细胞、血小板以及肝脏合成指标恢复正常。最初，免疫抑制方案由泼尼松龙、他克莫司和抗白细胞介素-2受体抗体（BT 563）组成，后来继续使用泼尼松龙和他克莫司。在26个月的随访观察中，未发现移植物排斥、严重感染或门静脉高压复发。到目前为止，患者状况良好，移植物功能正常。肝移植可能是晚期IPH患者的一种治愈性疗法，但移植后的长期随访必须观察IPH是否会复发。
