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摘要: <正>Dubin-Johnson综合征又称为先天性非溶血性黄疸直接胆红素升高I型,临床少见且易误诊,现就临床Dubin-Johnson综合征追踪12年1例病例报告如下。1病例资料患者女性,65岁,因"间断目黄、尿黄30余年,间断上腹部不适15年,加重伴乏力2个月"于2014年10月22日入院;30余年前无明显诱因出现目黄、尿黄,尿呈淡茶色,无皮肤黄染及瘙痒,未系统诊治;15年前无明显诱因出现目黄、尿黄加重,并
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Key words:
- Jaundice /
- chronic idiopathic /
- bilirubin
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[1]JI M,ZHANG JP,LI JS.Clinical features of Dubin-Johnson syndrome[J].J Clin Hepatol,2013,29(4):319-320.(in Chinese)姬蒙,张金平,李建生.Dubin-Johnson综合征的临床特点分析[J].临床肝胆病杂志,2013,29(4):319-320. [2]DUBIN IN,JOHNSON FB.Chronic idiopathic jaundice with unidentified pigment in liver cells:a new clinicopathologic entity with a report of 12 cases[J].Medicine(Baltimore),1954,33(3):155-197. [3]HASHIMOTO K,UCHIUMI T,KONNO T,et al.Trafficking and functional defects by mutations of the ATP-binding domains in MRP2 in patients with Dubin-Johnson syndrome[J].Hepatology,2002,36(5):1236-1245. [4]IKEMURA K,IWAMOTO T,OKUDA M.Altered functions and expressions of drug transporters in liver,kidney and intestine in disorders of local and remote organs:possible role of oxidative stress in the pathogenesis[J].Expert Opin Drug Metab Toxicol,2009,5(8):907-920. [5]QOSA H,MILLER DS,PASINELLI P,et al.Regulation of ABCefflux transporters at blood-brain barrier in health and neurological disorders[J].Brain Res,2015,1628(Pt B):298-316. [6]UCHIUMI T,TANAMACHI H,KUCHIWAKI K,et al.Mutation and fuctional analysis of ABCC2/multidrug resistance protein 2 in a Japanese patient with Dubin-Johnson syndrome[J].Hepatol Res,2013,43(5):569-575. [7]STRASSBURG CP.Hyperbilirubinemia syndromes(Gilbert-Meulengracht,Crigler-Najjar,Dubin-Johnson,and Rotor Syndrome)[J].Best Pract Res Clin Gastroenterol,2010,24(5):555-571. -
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