儿童门静脉高压症研究进展

儿童门静脉高压症是指在多种病因作用下，门静脉系统的血流受阻和（或）血流量增加、血管舒缩功能障碍，引起门静脉及其属支的压力持续增高，门静脉压力＞5 mmHg（1 mmHg=0.133 kPa），或门静脉、肝静脉压力梯度＞10 mmHg，最终导致脾大、门腔侧支循环形成和开放、腹水等临床表现，是一种血流动力学异常综合征。儿童门静脉高压症根据病因不同分为肝硬化性门静脉高压症和非肝硬化性门静脉高压症；根据压力来源的解剖部位，可将门静脉高压症分为肝前性、肝内性（窦前性、窦性、窦后性）及肝后性。治疗方法有药物治疗、曲张静脉套扎或硬化、手术治疗等。虽然儿童门静脉高压症发病率较低，但可以引起胃食管静脉曲张破裂出血、肝性脑病等严重并发症。为加强对本病的认识，减少并发症，提高治愈率，现将其病因分类、发病机制、诊断和治疗方面的研究进展作一综述。

Research progress of portal hypertension in children

Portal hypertension in children refers to the blocked and/or increased blood flow of the portal vein system and the dysfunction of vasodilation and contraction caused by various causes, resulting in continuous increase of pressure of the portal vein and its branches. The pressure of the portal vein exceeds 5 mmHg (1 mmHg=0.133 kPa), or the pressure gradient of the portal vein and hepatic vein exceeds 10 mmHg, eventually leading to the formation and opening of the splenomegaly and portacaval collateral circulation, as well as ascites and other clinical manifestations. It is an abnormal hemodynamic syndrome. Portal hypertension in children can be divided into cirrhotic portal hypertension and non-cirrhotic portal hypertension according to the etiology. According to the anatomical location of pressure source, portal hypertension can be divided into pre-hepatic, intrahepatic (pre-sinusoidal, sinusoidal, post-sinusoidal) and post-hepatic. Therapeutic methods include drug therapy, varicose vein ligation or sclerosis, surgical treatment, etc. Although the incidence of portal hypertension in children is low, it can cause severe complications such as gastroesophageal varices bleeding and hepatic encephalopathy. In order to strengthen the understanding of this disease, improve the cure rate and reduce complications, this paper reviews the progress in etiological classification, pathogenesis, diagnosis and treatment of the disease.