Khatoon Nazia, Keaveny Andrew P, Carames Gian P, Krishna Murli, Lewis Jason, Nakhleh Raouf E
Department of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, Florida.
Division of Gastroenterology and Hepatology, Department of Transplant Medicine, Mayo Clinic, Jacksonville, Florida.
Gastro Hep Adv. 2023 Jan 11;2(3):303-306. doi: 10.1016/j.gastha.2023.01.003. eCollection 2023.
The aim of this study was to review a large series of cases with hepatoportal sclerosis (HPS) as a pathologically recognizable entity in liver tissue specimens and describe the associated clinical and radiographic manifestations, along with the outcomes of this entity.
Data were collected through a retrospective chart review.
Twenty-eight patients were identified that had pathologically defined HPS. All patients had a significant past medical history and signs and symptoms of portal hypertension. The most consistent laboratory finding was an elevated alkaline phosphatase. Radiographically, 9 patients were mistakenly identified as having advanced fibrosis/cirrhosis. The initial histologic diagnosis was made on biopsy in 20 patients and after transplant in 8 patients. The severity of symptoms was variable and required transplantation in 11 patients, 3 were treated with transjugular intrahepatic portosystemic shunt, and the remaining patients were treated symptomatically.
HPS is associated with past medical history that may be causal in nature. Signs and symptoms may be severe enough to require liver transplantation. A significant proportion of patients are radiographically misdiagnosed as cirrhosis. In this small series, overall outcomes for transplanted patients are acceptable.
本研究旨在回顾一系列肝门脉硬化（HPS）病例，HPS在肝组织标本中是一种可通过病理识别的实体，并描述其相关的临床和影像学表现以及该实体的转归。
通过回顾性病历审查收集数据。
确定了28例经病理确诊为HPS的患者。所有患者均有重要既往病史及门静脉高压的体征和症状。最一致的实验室检查结果是碱性磷酸酶升高。在影像学上，9例患者被误诊为晚期纤维化/肝硬化。20例患者通过活检做出初始组织学诊断，8例患者在移植后做出诊断。症状严重程度各不相同，11例患者需要进行移植，3例接受经颈静脉肝内门体分流术治疗，其余患者接受对症治疗。
HPS与既往病史相关，既往病史可能具有因果关系。体征和症状可能严重到需要进行肝移植。相当一部分患者在影像学上被误诊为肝硬化。在这个小系列研究中，移植患者的总体转归是可以接受的。
