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目的探讨特发性门脉高压征(IPH)的临床和病理特点。方法回顾性分析16例病理证实为IPH患者的临床及病理资料,分别将临床、影像表现与病理特点进行对比研究。结果 16例患者男、女各8例,发病年龄(34.2士14.7)岁,病程1个月~14年不等;脾大、脾亢10例,食管胃底静脉曲张6例,上消化道出血和黑便5例,贫血7例;肝功能指标正常;肝功能正常9例,升高7例,以胆碱酯酶(CHE)的中位数6000为分界线,CHE﹤6000U/L(8例)门脉高压程度较重,但病理损伤较轻;B超及CT呈肝硬化表现8例,与无肝硬化表现者相比,ALT、AST、TBiL明显升高,CHE明显降低,但病理表现无明显差异;侧支循环形成的6例患者门脉高压和病理损伤程度明显轻于其余病例,但CHE均低于6000U/L。结论 IPH患者的门脉高压症状突出,而肝功能储备相对较好;病理特征仍是IPH必要的诊断依据。
Objective To investigate the clinical and pathological features of idiopathic portal hypertension (IPH). Methods The clinical and pathological data of 16 pathologically confirmed patients with IPH were retrospectively analyzed. The clinical, imaging and pathological features were compared respectively. Results There were 8 males and 16 females in each of the 16 patients (34.2 ± 14.7 years old), ranging in duration from 1 month to 14 years. Splenomegaly and hypersplenism in 10 cases, esophageal varices in 6 cases, upper gastrointestinal bleeding And 5 cases of melena, anemia in 7 cases; normal liver function; normal liver function in 9 cases, increased in 7 cases, choline esterase (CHE) median of 6000 as the dividing line, CHE <6000U / L Cases) portal hypertension was severe, but pathological damage was mild; B ultrasound and CT showed cirrhosis in 8 cases, compared with those without cirrhosis, ALT, AST, TBiL significantly increased, CHE decreased significantly, but the pathology There was no significant difference between the two groups. The incidence of portal hypertension and pathological injury in the 6 patients formed by collateral circulation were significantly lower than those in the remaining cases, but CHE were lower than 6000U / L. Conclusion The symptoms of portal hypertension in patients with IPH are prominent, while the reserve of liver function is relatively good. The pathological features are still the necessary diagnostic criteria for IPH.