胆管消失综合征病因学诊断及预后进展

被引：7

作者：

孙玥

赵新颜

贾继东

机构：

[1] 首都医科大学附属北京友谊医院肝病中心

来源：

肝脏 | 2014年 / 19卷 / 02期

关键词：

D O I：

10.14000/j.cnki.issn.1008-1704.2014.02.019

中图分类号：

R575.7 [胆管疾病];

学科分类号：

100201 [内科学];

摘要：

<正>胆管消失综合征(Vanishing Bile Duct Syndrome,VBDS)指由诸多因素引起的、以肝内胆汁淤积为主要临床表现的综合征,病理特征为肝穿标本(至少包括11个汇管区)中汇管区小动脉伴行的小叶间胆管消失>50%[1]。病因主要包括先天畸形、免疫、肿瘤、药物毒物、感染及缺血缺氧等。VBDS临床表现多样,以胆汁淤积相关表现为主,包括乏力、厌食、瘙痒、高胆固醇血症、黄色瘤和脂溶性维生素缺乏等。实验室检查碱性磷酸酶和谷氨酰转肽酶明显升高,也可出现高直接胆红素血症和转氨酶轻度升高。因该病主要累及小胆管,影像学检查常无特异性改变,但可协助除外肝外胆管损伤。肝

引用

页码：137 / 139

页数：3

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