肝前性非肝硬化性门静脉高压症的诊疗进展
DOI: 10.3969/j.issn.1001-5256.2022.07.002
利益冲突声明:所有作者均声明不存在利益冲突。
作者贡献声明:杨佳琪负责收集资料并撰写文章;尚玉龙负责文稿修订;韩英负责选题,并最终审阅定稿。
Research advances in the diagnosis and treatment of prehepatic noncirrhotic portal hypertension
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摘要: 肝前性非肝硬化性门静脉高压是一组由非肝硬化因素导致的门静脉高压性疾病,目前认为这组疾病的病因主要与JAK2突变、血液高凝状态有关。临床表现主要包括静脉曲张破裂出血、脾肿大等,患者肝功能正常或仅有轻微异常。对肝前性非肝硬化性门静脉高压疾病的诊断主要依靠其影像学特点,同时还需排除肝硬化门静脉高压。治疗方面主要采取抗凝治疗和内镜下治疗,患者预后一般较好。本文系统梳理了肝前性非肝硬化性门静脉高压的主要进展。
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关键词:
- 非肝硬化性门静脉高压 /
- 静脉血栓栓塞 /
- 诊断 /
- 治疗学
Abstract: Prehepatic noncirrhotic portal hypertension is a group of diseases caused by noncirrhotic factors, and currently it is believed that the etiology of these diseases is mainly associated with JAK2 mutation and blood hypercoagulability. Main clinical manifestations include variceal bleeding and splenomegaly, with normal or slightly abnormal liver function. The diagnosis of prehepatic noncirrhotic portal hypertension mainly depends on its imaging characteristics, and meanwhile cirrhotic portal hypertension should be excluded. Anticoagulant therapy and endoscopy are the major therapies for these diseases, and patients often have a good prognosis. This article systematically reviews the recent advances in prehepatic noncirrhotic portal hypertension.-
Key words:
- Noncirrhotic Portal Hypertension /
- Venous Thromboembolism /
- Diagnosis /
- Therapeutics
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