Abernethy畸形患者临床特征的研究现状  

Research status on clinical characteristics of patients with Abernethy malformation

作　　者：白婷婷 陈东风 罗文雯 BAI Tingting;CHEN Dongfeng;LUO Wenwen(Department of Gastroenterology,Daping Hospital,Army Medical University,Chongqing 400042,China)

机构地区：[1]陆军军医大学大坪医院消化科,重庆400042

出　　处：《胃肠病学和肝病学杂志》2023年第7期837-840,共4页Chinese Journal of Gastroenterology and Hepatology

基　　金：国家自然科学基金(82170594)。

摘　　要：Abernethy畸形是一种由于胚胎脐静脉和卵黄静脉发育异常导致门静脉缺失或发育不良,门静脉系统与腔静脉系统之间出现异常分流的疾病,其发病机制尚未明确。由于该病临床上以呕血、便血和高氨血症等门静脉高压症为突出表现,临床实践中患者常被误诊为肝硬化门静脉高压等疾病。本文总结了Abernethy畸形患者的临床特点,以增强临床医师对该疾病的诊断和治疗能力。Abernethy malformation is a disease in which the portal vein is absent or hypoplastic due to abnormal development of the embryonic umbilical and yolk veins,and there is an abnormal shunt between the portal and vena cava veins.Its pathogenesis is not fully understood.Because the clinical presentations of this disease are highlighted by portal hypertension signs such as hematemesis,hematochezia or hyperammonemia,patients are often misdiagnosed as liver cirrhotic portal hypertension or other diseases in clinical practice.This paper summarized the clinical characteristics of patients with Abernethy malformation to enhance the diagnosis and treatment of this disease by clinicians.

关 键 词：ABERNETHY畸形 先天性肝外门体分流 肝硬化 门静脉高压 

分 类 号：R575[医药卫生—消化系统]