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[严重继发性硬化性胆管炎作为一种极为罕见潜在疾病的表现]

[Severe secondary sclerosing cholangitis as manifestation of a very rare underlying disease].

作者信息

Zecher Britta Franziska, Zenouzi Roman, Lang Melanie, Karagiannis Panagiotis, Clauditz Till, Fischer Lutz, Sterneck Martina, Schramm Christoph, Lohse Ansgar W, Sebode Marcial

机构信息

I. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland.

Europäisches Referenznetzwerk für seltene Lebererkrankungen (ERN RARE-LIVER), Hamburg, Deutschland.

出版信息

Internist (Berl). 2021 Dec;62(12):1349-1353. doi: 10.1007/s00108-021-01148-0. Epub 2021 Sep 21.

DOI:10.1007/s00108-021-01148-0
PMID:34546401
Abstract

Langerhans cell histiocytosis (LCH) is a very rare cause of secondary sclerosing cholangitis. We report the case of a 42-year-old male patient with sclerosing cholangitis and histological evidence of LCH from a bile duct biopsy. Due to rapid disease progression and exhaustion of conservative therapeutic approaches the patient received a liver transplantation. Nearly 2 years after transplantation the patient has a good graft function and no signs of recurrence of the underlying LCH.

摘要

朗格汉斯细胞组织细胞增多症（LCH）是继发性硬化性胆管炎非常罕见的病因。我们报告一例42岁男性患者，患有硬化性胆管炎，胆管活检有LCH的组织学证据。由于疾病进展迅速且保守治疗方法用尽，该患者接受了肝移植。移植后近2年，患者移植物功能良好，无潜在LCH复发迹象。

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本文引用的文献

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Langerhans-Cell Histiocytosis.朗格汉斯细胞组织细胞增多症
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Antiprogrammed Cell Death-1 Immunotherapy-Related Secondary Sclerosing Cholangitis.抗程序性细胞死亡蛋白1免疫治疗相关的继发性硬化性胆管炎
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Drug-induced cholestasis.药物性胆汁淤积
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Primary sclerosing cholangitis - a comprehensive review.原发性硬化性胆管炎——全面综述。
J Hepatol. 2017 Dec;67(6):1298-1323. doi: 10.1016/j.jhep.2017.07.022. Epub 2017 Aug 10.
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Secondary Sclerosing Cholangitis: a Review of Recent Literature.继发性硬化性胆管炎：近期文献综述
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