Diagnosis and treatment of pre-hepatic (sinusoidal) non-cirrhotic portal hypertension and related difficulties
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摘要: 肝(窦)前型非肝硬化性门静脉高压症(NCPH)是一组以门静脉高压症为突出表现的血管异质性疾病,病因复杂。与肝硬化门静脉高压症相比,NCPH患者肝功能基本正常,肝静脉压力梯度正常或轻度增加,预后较好,极易误诊为不明原因肝硬化门静脉高压症,肝脏病理是诊断"金标准"。目前,非选择性β受体阻滞剂及内镜是NCPH的主要治疗方法,但是缺乏前瞻性多中心、大样本、高质量的临床证据。Abstract: Pre-hepatic (sinusoidal) non-cirrhotic portal hypertension is a group of vascular heterogeneous diseases with portal hypertension as the prominent manifestation and has a complex etiology.Compared with the patients with cirrhotic portal hypertension, the patients with prehepatic (sinusoidal) non-cirrhotic portal hypertension have normal liver functions, normal or a mild increase in hepatic venous pressure gradient, and better prognosis.Pre-hepatic (sinusoidal) non-cirrhotic portal hypertension may easily be misdiagnosed as unexplained cirrhotic portal hypertension, and liver pathology is the gold standard for diagnosis.Non-selectiveβ-receptor blockers and endoscopy are major therapies for this disease, but there is a lack of high-quality clinical evidence from large-scale prospective multicenter studies.
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Key words:
- hypertension, portal /
- diagnosis /
- therapy
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