多囊肝合并门静脉高压症一例并文献复习

Polycystic liver disease with portal hypertension：a case report and literature review

目的：探讨多囊肝合并门静脉高压症的临床特点、诊断及治疗方法，提高诊疗水平。方法回顾性分析1例多囊肝合并门静脉高压症患者的临床资料并复习相关文献。结果该多囊肝合并门静脉高压症患者临床表现为乏力、纳差、腹胀及下肢浮肿，实验室检查提示肝功能轻度损害（碱性磷酸酶291．2 U／L，γ-谷氨酰转肽酶168．1 U／L，总蛋白59．9 g／L，白蛋白32．2 g／L，总胆红素21．0μmol／L，直接胆红素11．5μmol／L），腹部超声及CT可见正常肝脏及肾脏实质被多发大小不等的囊肿代替，囊肿相互不交通，给予保肝、营养肝细胞、利尿对症治疗后病情明显改善。结论临床上多囊肝合并门静脉高压症患者比较少见，容易漏诊，临床医生应提高对该病的认识，以免误诊漏诊。

Objective To study the clinical features,diagnosis and treatment of polycystic liver disease （PLD）complicated with portal hypertension （PHT）.Methods The clinical data of one patient with PLD and PHT was retrospectively analyzed,and relevant literature was reviewed.Results The patient presented fatigue,dyspepsia, abdominal distension and lower limb edema.Laboratory examination showed mild liver dysfunction（Alkaline phospha-tase 291.2U/L,gamma glutamyl transpeptidase 168.1U/L,59.9g/L,total protein,albumin 32.2g/L,21.0μmol /L, total bilirubin,direct bilirubin 11.5μmol /L）.Abdominal ultrasound （US）and computerized tomography （CT） showed multiple noncommunicating cysts of varying size in both liver and kidney.Antioxidant supplements and diuretic were introduced,and the therapy was approved to be effective.Conclusion The case in this report illustrates that PLD could occasionally present with PHT.Physician should be alert to prevent misdiagnosis.