【摘要】 目的探讨肝小静脉闭塞病(HVOD)的诊断、鉴别诊断,特别是与布-加综合征相鉴别,以提高对该疾病的认识。方法收集2005年1月—2011年12月15例HVOD患者,所有患者均有服用中药土三七(350～1 000 g)史。临床表现为不同程度的肝区疼痛、肝肿大、黄疸、腹水,病程1～6个月。分析土三七所致患者的临床、影像学和病理学资料。结果经内科药物治疗,治愈4例,好转6例,死亡5例。15例经多种影像学检查提示肝小静脉闭塞而3支肝静脉主干和下腔静脉肝段无闭塞,CT,MRI动态增强扫描显示全肝呈典型的"地图状"、斑片状增强区和低灌注区。6例病理表现为肝小静脉血管内膜急性水肿,而非血栓性阻塞、小叶扩张淤血、肝细胞坏死。结论结合服用土三七病史和具备典型影像学表现者,应考虑为HVOD,确诊仍依靠病理活检。早期明确诊断,合理内科治疗,大多数患者预后良好。更多还原

【Abstract】 Objective To discuss the diagnosis and differential diagnosis of hepatic veno-occlusive disease(HVOD),focusing on the differentiation between HVOD and Budd-Chiari syndrome,in order to make a further understanding of HVOD.Methods A total of 15 patients with HVOD,who were encoun-tered at the hospital during the period from Jan.2005 to Dec.2011,were enrolled in this study.All the patients had a history of orally taking radix notogineseng,a kind of Chinese herb,with a dosage of 350-1 000 g.Clinically,all patients showed signs of hepatalgia of different degrees,hepatomegaly,jaundice,ascites,etc.The course of disease varied from one to 6 months.The radix notogineseng-induced clinical symptoms,the imaging manifestations and the pathological findings were analyzed.Results After medical treatment,complete cure was obtained in four patients,a favorable change was seen in six patients and death occurred in five patients.Multiple imaging examinations revealed that hepatic small veins were obstructed in all patients,while and three main branches of hepatic vein as well as the hepatic segment of inferior vena cava were unobstructed.Dynamic enhanced CT and MRI scanning showed that typical patch or map-like enhancement and hypo-perfusion areas within the hepatic parenchyma could be observed.In 6 patients,the pathological changes were characterized by acute intimal edema of small hepatic veins,and no thrombus,hepatic lobular congestion or hepatic cell necrosis could be seen.Conclusion Based on a history of orally taking radix notogineseng combined with typical imaging manifestations,the diagnosis of HVOD should be considered,although the confirmation of the diagnosis relies on the pathologic examination.Excellent prognosis can be expected in most cases if the diagnosis can be made as early as possible and the proper medical management can be carried out without delay.更多还原