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良性复发性肝内胆汁淤积症(BRIC):一例非洲病例报告。

Benign Recurrent Intrahepatic Cholestasis (BRIC): An African Case Report.

作者信息

Salyani Adil, Barasa Linda, Rajula Allan, Ali Sayed K

机构信息

Department of Medicine, Aga Khan University Hospital, Nairobi, Kenya.

出版信息

Case Rep Gastrointest Med. 2020 Mar 10;2020:2894293. doi: 10.1155/2020/2894293. eCollection 2020.

DOI:10.1155/2020/2894293
PMID:32231815
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7085836/
Abstract

Benign recurrent intrahepatic cholestasis (BRIC) is a rare disorder characterised by recurrent episodes of cholestatic jaundice. First described in 1959, BRIC has been reported in patients all over the world including of African descent. Here, we describe a case of a 21-year-old male with recurring episodes of cholestatic jaundice where we diagnosed BRIC and terminated an episode with rifampicin. To our knowledge, this is the first case report of BRIC diagnosed in Africa.

摘要

良性复发性肝内胆汁淤积症(BRIC)是一种罕见的疾病,其特征为胆汁淤积性黄疸反复发作。BRIC于1959年首次被描述,世界各地包括非洲裔患者中均有报道。在此,我们描述一例21岁男性,其患有胆汁淤积性黄疸反复发作,我们诊断为BRIC并用利福平终止了一次发作。据我们所知,这是在非洲诊断出BRIC的首例病例报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7946/7085836/1038a3c35fbe/CRIGM2020-2894293.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7946/7085836/1038a3c35fbe/CRIGM2020-2894293.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7946/7085836/1038a3c35fbe/CRIGM2020-2894293.001.jpg

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本文引用的文献

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A rare cause of a cholestatic jaundice in a North African teenager.一位北非青少年胆汁淤积性黄疸的罕见病因。
Liver Int. 2019 Nov;39(11):2036-2041. doi: 10.1111/liv.14122. Epub 2019 Jul 24.
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New Insights in Genetic Cholestasis: From Molecular Mechanisms to Clinical Implications.遗传胆汁淤积症的新见解:从分子机制到临床意义。
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New paradigms of USP53 disease: normal GGT cholestasis, BRIC, cholangiopathy, and responsiveness to rifampicin.USP53 病的新范式:正常 GGT 胆汁淤积、BRIC、胆管病和对利福平的反应性。
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Nasobiliary Drainage for Benign Recurrent Intrahepatic Cholestasis in Patients Refractory to Standard Therapy.经鼻胆管引流术治疗标准治疗无效的良性复发性肝内胆汁淤积症患者
Clin Invest Med. 2016 Dec 1;39(6):27522.
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Successfully treated intractable pruritus with rifampin in a case of benign recurrent intrahepatic cholestasis.在一例良性复发性肝内胆汁淤积症中,利福平成功治疗顽固性瘙痒。
Clin J Gastroenterol. 2008 Dec;1(4):160-163. doi: 10.1007/s12328-008-0027-y. Epub 2008 Sep 26.
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Clinical challenge in hepatology.肝病学中的临床挑战。
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Characterization of urinary bile acids in a pediatric BRIC-1 patient: effect of rifampicin treatment.对 BRIC-1 患儿尿液胆汁酸的特征分析:利福平治疗的效果。
Clin Chim Acta. 2012 Aug 16;413(15-16):1301-4. doi: 10.1016/j.cca.2012.04.011. Epub 2012 Apr 14.
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Scand J Gastroenterol. 2012 Apr;47(4):482-8. doi: 10.3109/00365521.2011.650191. Epub 2012 Jan 10.
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Benign recurrent intrahepatic cholestasis: late initial diagnosis in adulthood.良性复发性肝内胆汁淤积症:成年期迟发性初始诊断。
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