非肝硬化性门静脉高压症的研究现状

张博静; 韩国宏; 樊代明

doi:10.3969/j.issn.1001-5256.2016.02.009

非肝硬化性门静脉高压症的研究现状

DOI: 10.3969/j.issn.1001-5256.2016.02.009

第四军医大学西京消化病医院消化介入科

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中图分类号: R657.34
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出版历程
- 收稿日期: 2015-09-30
- 出版日期: 2016-02-20

Research advances in non-cirrhotic portal hypertension

Department of Liver Disease and Digestive Interventional Radiology,Xijing Hospital of Digestive Disease,Fourth Military Medical University

摘要

摘要: 肝硬化是门静脉高压的最常见原因,但仍有约20%的门静脉高压继发于非肝硬化因素,称为非肝硬化性门静脉高压症(NCPH),在发展中国家发病率较高。NCPH是一组异源性的肝脏血管疾病,临床上多见的是特发性门静脉高压(IPH)、肝外门静脉血管阻塞(EHPVO),以及布加综合征、先天性肝纤维化和结节再生性增生等少见病。此类患者常常具有门静脉高压的证据,如反复发生的静脉曲张出血和脾脏肿大,但肝功能保存尚好。目前尚无诊断NCPH的统一标准,对其诊断仍是一个挑战。临床上往往采用排除性诊断,必要时可行肝穿刺活组织检查来确诊。介绍了IPH和EHPVO的发病机制、病理表现、诊断方法及治疗策略的选择,若能有效控制上消化道出血,NCPH被认为是预后相对良好的一类疾病。
- 非肝硬化性门静脉高压症 /
- 特发性门静脉高压 /
- 肝外门静脉血管阻塞
Abstract: Although liver cirrhosis is the most common cause of portal hypertension( PH),about 20% of PH cases are caused by non- cirrhotic reasons,which are referred to as non- cirrhotic portal hypertension( NCPH),with a high incidence rate in developing countries.NCPH is a group of heterogeneous hepatic vascular diseases,including idiopathic portal hypertension( IPH) and extrahepatic portal vein obstruction( EHPVO),as well as the rare diseases in clinical practice such as Budd- Chiari syndrome,congenital hepatic fibrosis,and nodular regenerative hyperplasia. The patients with NCPH usually have the symptoms of portal hypertension,such as recurrent variceal bleeding and splenomegaly,but liver function is well preserved in these patients. At present,the diagnosis of NCPH lacks a universally accepted standard and remains a challenge. In clinical practice,the method of exclusion is usually applied for the diagnosis of HCPH,and liver biopsy is performed when necessary to make a confirmed diagnosis. This paper introduces the pathogenesis and pathological manifestations of IPH and EHPVO,as well as the selection of diagnostic methods and therapeutic strategies. If upper gastrointestinal bleeding can be effectively controlled,NCPH is considered to have a relatively good prognosis.
- non-cirrhotic portal hypertension /
- idiopathic portal hypertension /
- extra-hepatic portal vein obstruction

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