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药物性胆管消失综合征的成功肝移植

Successful liver transplantation for drug-induced vanishing bile duct syndrome.

作者信息

Hashim Ahmed, Barnabas Ashley, Miquel Rosa, Agarwal Kosh

机构信息

Institute of Liver Studies, King's College Hospital NHS Foundation Trust, London, UK

Institute of Liver Studies, King's College Hospital NHS Foundation Trust, London, UK.

出版信息

BMJ Case Rep. 2020 Jan 15;13(1):e233052. doi: 10.1136/bcr-2019-233052.

Abstract

Drug-induced cholestasis has a wide range of clinical presentations, and in a small number of patients, it can progress to severe ductopenia. A 63-year-old woman was referred to our department with progressive cholestasis and hyperbilirubinaemia following a course of flucloxacillin. Despite the comprehensive laboratory, imaging and genetic investigations, no other cause for the cholestasis was demonstrated. Sequential liver biopsies confirmed the development of vanishing bile duct syndrome. She developed significant cachexia and pruritus that did not respond to medical therapy, and hence she was listed for liver transplantation. She underwent liver transplantation 6 months after the initial drug-induced injury. She has remained well with good graft function at 1-year follow-up. The case highlights an extreme form of drug-induced ductopenia and underscores the need for meticulous hepatology input and consideration of liver transplantation in some patients.

摘要

药物性胆汁淤积有广泛的临床表现,在少数患者中,它可进展为严重的小胆管减少症。一名63岁女性在接受氟氯西林治疗后出现进行性胆汁淤积和高胆红素血症,被转诊至我科。尽管进行了全面的实验室、影像学和基因检查,但未发现胆汁淤积的其他原因。连续肝活检证实了消失胆管综合征的发生。她出现了严重的恶病质和瘙痒,药物治疗无效,因此被列入肝移植名单。在最初的药物性损伤6个月后,她接受了肝移植。在1年的随访中,她移植肝功能良好,情况一直不错。该病例突出了药物性小胆管减少症的一种极端形式,并强调了在某些患者中需要细致的肝病学评估以及考虑肝移植。

相似文献

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Flucloxacillin induced delayed cholestatic hepatitis.氟氯西林诱发迟发性胆汁淤积性肝炎。
Aust N Z J Med. 1990 Jun;20(3):251-3. doi: 10.1111/j.1445-5994.1990.tb01032.x.
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Meropenem-induced vanishing bile duct syndrome.美罗培南致胆汁消失综合征。
Pharmacotherapy. 2010 Sep;30(9):953. doi: 10.1592/phco.30.9.953.

本文引用的文献

3
Management of pruritus in chronic liver disease.慢性肝病瘙痒的管理
Dermatol Res Pract. 2015;2015:295891. doi: 10.1155/2015/295891. Epub 2015 Mar 10.
10
Vanishing bile duct syndrome.消失胆管综合征
Clin Liver Dis. 2008 Feb;12(1):203-17, x. doi: 10.1016/j.cld.2007.11.007.

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