重视非肝硬化性门静脉高压的诊断和治疗

Diagnosis and treatment of on-cirrhotic portal hypertension

非肝硬化性门静脉高压(NCPH)是指除肝硬化外多种疾病导致的门静脉高压症。NCPH常见的原因有门静脉血栓形成、先天性肝纤维化和特发性门静脉高压等。这组疾病的主要特点是门静脉高压相关的表现突出,而肝功能储备相对较好,鉴别该类疾病需要临床,影像学和病理学的深入检查。通过适当的内外科治疗,多数患者预后较好。

Non-cirrhotic portal hypertension （NCPH） is a group of diseases that show evidences of portal hypertension but no cirrhosis is present. Common causes of NCPH include pre-sinusoidal portal lesions such as portal vein thrombosis, congenital liver fibrosis and idiopathic portal hypertension, and post-sinusoidal portal lesions. The major feature of this group of diseases is well preserved liver function in spite of prominent portal hypertensive manifestations such as esophageal varices/gastrointestinal bleeding and splenomegaly/hypersplenism. Careful differentiation from cirrhosis requires thorough clinical, radiological and pathological investigation. Preventing and control of varieeal bleeding and hyporsplenism through medical, endoscopic and interventional procedures yield good prognosis in most of the patients with NCPH.