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肝紫癜病作为钍造影剂肝病的晚期致命并发症。五例报告。

Peliosis hepatis as a late and fatal complication of thorotrast liver disease. Report of five cases.

作者信息

Okuda K, Omata M, Itoh Y, Ikezaki H, Nakashima T

出版信息

Liver. 1981 Jun;1(2):110-22. doi: 10.1111/j.1600-0676.1981.tb00028.x.

Abstract

Five autopsy cases of peliosis hepatis occurring as a late complication of thorotrast (ThO2) liver disease are described. The liver contained many blood-filled cystic spaces of various sizes. Marked sinusoidal dilatation, disruption of cell cords and reticulin fiber framework, and cystic dilatation of sinusoids seem to represent the developmental stages of peliosis hepatis in sequence. Of the five cases, two had no other liver disease except for hepatic fibrosis, and the other three had associated neoplasms, such as angiosarcoma, hepatocellular carcinoma, cholangiocarcinoma, benign hemangioma, and their combinations. Peliosis hepatis seemed to have directly contributed to the patient's death in four cases. The most characteristic clinical feature was the fulminant terminal course with massive ascites, deep jaundice, and hepatic failure, often accompanied by hepatorenal syndrome and tendency to hemorrhage. Liver function study suggested progressive hepatic insufficiency with reduction in serum albumin, prothrombin and the clearance rate for test dyes, and increase in bilirubin. Clinical diagnosis was almost impossible without biopsy.

摘要

本文描述了5例钍造影剂(二氧化钍)肝病晚期并发症——肝紫癜病的尸检病例。肝脏内有许多大小各异、充满血液的囊性间隙。明显的肝血窦扩张、肝细胞索和网状纤维支架破坏以及肝血窦的囊性扩张似乎依次代表了肝紫癜病的发展阶段。5例中,2例除肝纤维化外无其他肝脏疾病,另外3例伴有血管肉瘤、肝细胞癌、胆管癌、良性血管瘤及其组合等肿瘤。4例患者的死亡似乎与肝紫癜病直接相关。最典型的临床特征是终末期病情急骤,伴有大量腹水、深度黄疸和肝功能衰竭,常伴有肝肾综合征和出血倾向。肝功能检查显示血清白蛋白、凝血酶原和试验染料清除率降低,胆红素升高,提示进行性肝功能不全。若无活检,几乎无法做出临床诊断。

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