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摘要: 门静脉高压症常见于肝硬化，而其他非肝硬化的肝脏本身疾病或其他系统疾病统称为非肝硬化门静脉高压(NCPH)。NCPH的机制包括门静脉灌注增加和血管阻力增大两方面。根据血管阻力增大的部位可分为肝前性、肝性和肝后性，而肝性又可细分为窦前性、窦性和窦后性。不同部位的NCPH，其机制和疾病谱不同。对于门静脉高压表现突出而肝损伤轻微和肝脏外形基本正常的患者需要考虑NCPH。影像学和病理学是协助病因诊断的重要工具，不同疾病有各自的特征性表现。对于NCPH的治疗，主要是针对原发病和门静脉高压的治疗，从而减少门静脉高压相关并发症的发生。Abstract: Cirrhosis is the most common cause of portal hypertension; other, less frequent non-cirrhotic liver disease and other systemic disease causes are therefore grouped as non-cirrhotic portal hypertension(NCPH). The mechanism of NCPH includes two aspects: increased portal perfusion and increased vascular resistance. NCPH can be divided into pre-hepatic, hepatic and post-hepatic, while hepatic can be subdivided into pre-sinusoidal, sinusoidal and post-sinusoidal. Different types have different causes and mechanisms. When portal hypertension occurs in case of slight liver injury and normal liver appearance, NCPH must be considered. Combining diagnostic imaging and pathology are important in etiological diagnosis, different diseases have their own characteristic manifestations. The management of NCPH consists in the treatment of associated diseases and of portal hypertension, and reduce the incidence of complications.
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Key words:
- non-cirrhotic portal hypertension /
- mechanism /
- diagnosis /
- management
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表 1 引起NCPH的肝前性、肝性(窦前性、窦性、窦后性)、肝后性等相关疾病谱
位置 疾病谱 肝前性 肝外门静脉阻塞、门静脉血栓、门静脉瘤栓、左侧门静脉高压、内脏动静脉瘘、淋巴瘤、骨髓增生性疾病等 窦前性 特发性非硬化性门静脉高压、门静脉肝窦血管病、肝动脉-门静脉瘘、HHT、纤维囊性肝病(CHF、caroli病、成年型多囊病)，PBC、PSC、上皮样血管内皮瘤、血吸虫病、结节病、肝紫癜等 窦性 血管型DILI、NASH、戈谢病、ALD、肥大细胞增生病、妊娠期急性脂肪肝、浸润性疾病(如肝淀粉样变)等 窦后性 肝小静脉闭塞、血管内皮瘤、肉芽肿性静脉炎等 肝后性 布加综合征、下腔静脉阻塞、缩窄性心包炎、三尖瓣反流、严重右心衰、限制性心肌病等 注：HHT为遗传性出血性毛细血管扩张症；CHF为先天性肝纤维化；PBC为原发性胆汁性胆管炎；PSC为原发性硬化性胆管炎；DILI为药物性肝损伤；NASH为非酒精性脂肪性肝炎；ALD为酒精性肝病。 
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