特发性门脉高压1例并文献复习

Idiopathic Portal Hypertension: A Case Report and Literature Review

摘要: 本文报告一例53岁男性患者因“黑便3天”入院，有自服感冒药史(具体成分不详)，体检发现皮肤巩膜轻度黄染、中度贫血貌及脾大。实验室检查提示三系下降、肝功能基本正常、肝炎病毒标志物及自身免疫抗体(抗核抗体19项及自身免疫性肝病相关抗体谱9项)均阴性。影像学检查示脾大、门静脉高压，肝活检示结节性再生性增生，胃镜提示食管及胃底静脉曲张，予内镜下曲张静脉硬化治疗及对症支持后，患者黑便转黄，血红蛋白回升。结合患者临床表现、影像学及病理学结果，最终诊断为特发性门脉高压。患者出院后服用鲨肝醇、利可君、瑞巴派特，但血红蛋白仍呈进行性下降，后续因再发出血入院，建议行经颈静脉肝内门体分流术(TIPS)治疗，患者最终决定保守治疗。该病例提示，IPH临床罕见且缺乏特异性表现，需通过完整病因筛查、影像学及病理学检查以明确诊断，根据患者情况个体化选择治疗方案，同时应重视患者的长期临床管理。

Abstract: This article reports a case of a 53-year-old male patient admitted with “melena for 3 days” and a history of self-administration of cold medication (specific components unknown). Physical examination revealed mild icteric skin and sclera, moderate anemic appearance, and splenomegaly. Laboratory findings showed pancytopenia, essentially normal liver function, negative results for hepatitis virus markers, and negative autoantibodies (including 19 items of antinuclear antibody and 9 items of autoimmune liver disease-related antibody panel). Imaging studies revealed splenomegaly and portal hypertension. Liver biopsy demonstrated nodular regenerative hyperplasia. Gastroscopy showed esophageal and gastric varices. After endoscopic sclerotherapy for varices and symptomatic supportive treatment, the patient’s melena resolved and hemoglobin levels recovered. Based on the clinical manifestations, imaging, and pathological findings, the final diagnosis was idiopathic portal hypertension. After discharge, the patient took batylol, leucogen, and rebamipide, but his hemoglobin progressively decreased. He was subsequently readmitted due to recurrent bleeding. Transjugular intrahepatic portosystemic shunt (TIPS) was recommended, but the patient ultimately opted for conservative management. This case suggests that idiopathic portal hypertension is clinically rare and lacks specific manifestations. Definitive diagnosis requires comprehensive etiological screening, imaging, and pathological examination. Treatment should be individualized based on the patient’s condition, and long-term clinical management should be emphasized.