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肝门静脉性硬变的临床不同演变阶段:3 例病例介绍及文献复习。

Hepatoportal sclerosis clinical different evolutionary stages: presentation of 3 cases and literature review.

机构信息

Department of Digestive Diseases, Hospital Universitario Virgen de la Arrixaca, Murcia, Spain.

出版信息

Rev Esp Enferm Dig. 2012 Feb;104(2):94-7. doi: 10.4321/s1130-01082012000200010.

Abstract

Hepatoportal sclerosis (HPS) is characterized by presinusoidal intrahepatic portal hypertension associated with splenomegaly and anemia in patients with non-cirrhotic liver. Liver biopsy is essential, especially to rule out other processes. Being a disease of unknown etiology, the majority of cases have been described in eastern countries. However, it may be an underdiagnosed disease in the West. Symptoms are related to portal hypertension and the clinical spectrum is wide, ranging from anemia with normal liver function tests to bleeding due to esophageal varices. Treatment is directed to the complications and the prognosis is better than in patients with cirrhosis.We report three cases of HPS presenting at different clinical stages and the findings of liver biopsies, the clinical outcomes and a review of scientific literature.

摘要

肝门静脉硬化症(HPS)的特征是肝内窦性门静脉高压,伴有脾肿大和非肝硬化患者的贫血。肝活检至关重要,尤其是为了排除其他病变。作为一种病因不明的疾病,大多数病例都在东方国家报道过。然而,在西方国家,HPS 可能是一种被低估的疾病。症状与门静脉高压有关,临床谱广泛,从肝功能正常的贫血到食管静脉曲张出血。治疗针对并发症,预后优于肝硬化患者。我们报告了三例在不同临床阶段出现的 HPS 病例,并对肝活检、临床转归和文献复习进行了总结。

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