14例Dubin-Johnson综合征临床病理特征分析及文献复习  

Clinical and pathological features of 14 patients with Dubin-Johnson syndrome and the literature review

作　　者：桑伟[1] 陈兰[1] 刘铭[1] 冯志寅 张巍[1] SANG Wei;CHEN Lan;LIU Ming;FENG Zhiyin;ZHANG Wei(The First Affiliated Hospital of Xinjiang Medical University,Urumqi,Xinjiang 830054,China)

机构地区：[1]新疆医科大学第一附属医院,乌鲁木齐830054

出　　处：《新疆医学》2021年第1期27-29,55,共4页Xinjiang Medical Journal

基　　金：新疆维吾尔自治区自然科学基金-医学联合基金项目(项目编号:2017D01C312)。

摘　　要：目的通过对14例肝脏Dubin-Johnson综合征临床病理特征分析,并文献复习,加强对该疾病的认识,为临床病理诊断提供一定经验。方法收集2010年-2017年新疆医科大学第一附属医院病理科明确诊断为Dubin-Johnson综合征14例,回顾分析14例Dubin-Johnson综合征临床特征、实验室检查和组织学形态。结果 (1)临床特征:14例患者,男性9例,女性5例,年龄17-59岁;12例有幼年开始出现间歇性黄疸,其中3例伴有轻度乏力;2例体检发现结合胆红素增高。(2)实验室检查:14例均有总胆红素(TBil)和结合胆红素(DBil)增高,TBil(34.1-75.6),平均(56.2±10.1)μmoL/L,DBil(23.5-56.7)μmoL/L,平均(39.6±9.2)μmoL/L;2例伴有非结合胆红素(IBil)增高;14例其余生化指标均正常;14例病毒血清学及自身免疫抗体均为阴性。(3)B超和CT显示2例肝脾轻度增大。(4)组织学特征:肝小叶基本结构正常,肝细胞轻度水肿,在中央静脉周围或全小叶肝细胞内出现棕褐色粗大或细小颗粒;铁特殊染色显示颗粒均为阴性。结论 Dubin-Johnson综合征是一类慢性特发性黄疸,以幼年出现间歇性黄疸为临床特征,结合胆红素异常增高,其余生化指标正常,组织学以肝细胞内出现棕褐色颗粒为特征。电镜检查或MRP2基因突变点检测可确诊。Objective To analyze the clinicopathological features of 14 cases of liver Dubin-Johnson syndrome and reviewing the literature, we can strengthen the understanding of the disease and provide some experience for clinicopathological diagnosis. Methods 14 cases of Dubin-Johnson syndrome were collected from the Department of Pathology of the First Affiliated Hospital of Xinjiang Medical University from 2010 to 2017. The clinical features, laboratory examination and histological morphology of 14 cases of Dubin-Johnson syndrome were retrospectively analyzed. Results(1)clinical features: 14 patients, male 9 cases, female 5 cases, age 17-59 years old;12 cases had intermittent jaundice in childhood, of which 3 cases were accompanied by mild fatigue;2 cases had increased conjugated bilirubin in physical examination.(2)Laboratory examination: TBi L(34.1-75.6), average(56.2 ± 10.1)μmoL/L, DBi L(23.5-56.7)μmoL/L,average(39.6 ± 9.2)μmoL/L were increased in all the 14 cases;IBil was increased in 2 cases;other biochemical indexes of 14 cases were normal;serology and autoimmune antibody of 14 cases were negative.(3)B ultrasound and CT showed mild enlargement of liver and spleen in 2 cases.(4)Histological features: the basic structure of hepatic lobule was normal, the hepatocytes were slightly edematous, and there were brown coarse or small particles around the central vein or in the whole lobule hepatocytes;iron special staining showed that the particles were negative. Conclusions Dubin-Johnson syndrome is a kind of chronic idiopathic jaundice, characterized by intermittent jaundice in childhood, abnormal increase of conjugated bilirubin, normal biochemical indexes and brown granules in liver cells. The diagnosis can be confirmed by electron microscopy or MRP2 gene mutation detection.

关 键 词：DUBIN-JOHNSON综合征 慢性特发性黄疸 结合胆红素 

分 类 号：R575[医药卫生—消化系统]