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1例用钙调神经磷酸酶抑制剂治疗的特发性婴儿期后巨细胞性肝炎

[A case of idiopathic postinfantile giant cell hepatitis treated with a calcineurin inhibitor].

作者信息

Hof Teresa, Flohr Felix, Thimme Robert, Kurz Philipp, Thiel Jens

机构信息

Universitätsklinikum Freiburg, Klinik für Innere Medizin II, Gastroenterologie, Hepatologie, Endokrinologie und Infektiologie.

Diakonissenkrankenhaus Karlsruhe-Rüppurr, ViDia Christliche Kliniken Karlsruhe, Klinik für Allgemeine Innere Medizin, Diabetologie, Endokrinologie.

出版信息

Dtsch Med Wochenschr. 2021 Feb;146(3):193-197. doi: 10.1055/a-1323-5057. Epub 2021 Jan 29.

DOI:10.1055/a-1323-5057
PMID:33513656
Abstract

INTRODUCTION

Postinfantile giant cell hepatitis is a rare entity in adult hepatopathy caused by various etiologies that can be summarized by their characteristic giant cells in histopathologic examination. Frequently, association with autoimmune, infectious and hepatotoxic events is described. Therefore, therapy consists in treatment of underlying diseases and immunosuppression.

HISTORY

We saw an 76-year-old patient due to histologically proven Postinfantile giant cell hepatitis. Despite administering budesonid as an initial attempt of treatment, no improvement in hepatitis was achieved. Hence, the patient was forwarded to us.

FINDINGS

Neither regarding the patient's history nor in laboratory and serological tests, nor in histopathological analysis of liver biopsies an underlying cause of giant cell hepatitis was identified.

THERAPY AND COURSE

Despite immunosuppressive therapy with glucocorticoids alone, cyclophosphamide and a monoclonal anti-CD20-antibody, giant cell hepatitis was not controlled. Hence, we started treatment with the calcineurin inhibitor Tacrolimus combined with low-dose prednisolone and thus were able to lower patient's liver values and stabilize hepatitis.

CONCLUSION

The good effectiveness of tacrolimus in our patient underlines the important role of calcineurin inhibitors in treating Postinfantile giant cell hepatitis, although rarely reported to date.

摘要

引言

婴儿期后巨细胞肝炎是成人肝病中的一种罕见病症,由多种病因引起,其在组织病理学检查中的特征性巨细胞可对病因进行总结。通常会描述其与自身免疫、感染及肝毒性事件相关。因此,治疗包括对基础疾病的治疗和免疫抑制。

病史

我们接诊了一名76岁的患者,其经组织学证实患有婴儿期后巨细胞肝炎。尽管最初尝试使用布地奈德进行治疗,但肝炎病情并无改善。因此,该患者被转诊至我们这里。

检查结果

无论是患者病史、实验室及血清学检查,还是肝活检的组织病理学分析,均未发现巨细胞肝炎的潜在病因。

治疗及病程

尽管单独使用糖皮质激素、环磷酰胺和一种抗CD20单克隆抗体进行免疫抑制治疗,但巨细胞肝炎仍未得到控制。因此,我们开始使用钙调神经磷酸酶抑制剂他克莫司联合小剂量泼尼松龙进行治疗,从而能够降低患者的肝脏指标并使肝炎病情稳定。

结论

他克莫司在我们这名患者身上的良好疗效凸显了钙调神经磷酸酶抑制剂在治疗婴儿期后巨细胞肝炎中的重要作用,尽管迄今为止相关报道较少。

相似文献

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[A case of idiopathic postinfantile giant cell hepatitis treated with a calcineurin inhibitor].1例用钙调神经磷酸酶抑制剂治疗的特发性婴儿期后巨细胞性肝炎
Dtsch Med Wochenschr. 2021 Feb;146(3):193-197. doi: 10.1055/a-1323-5057. Epub 2021 Jan 29.
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Postinfantile giant cell hepatitis in the setting of autoimmune hepatitis: exclusively a histological pattern or a prognosis predictor?婴儿期后巨细胞肝炎在自身免疫性肝炎中的表现:仅仅是一种组织学模式还是预后预测指标?
BMJ Case Rep. 2021 Jul 21;14(7):e243660. doi: 10.1136/bcr-2021-243660.
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J Dig Dis. 2015 Nov;16(11):683-8. doi: 10.1111/1751-2980.12273.
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Postinfantile Giant Cell Hepatitis in Native and Allograft Livers: A Multi-Institutional Clinicopathologic Study of 70 Cases.婴儿期后巨大细胞肝炎在原代和移植肝脏中的表现:70 例多机构临床病理研究。
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Eur J Gastroenterol Hepatol. 2003 May;15(5):551-5. doi: 10.1097/01.meg.0000050026.34359.7c.
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Postinfantile giant-cell transformation in hepatitis.
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Fulminant liver failure in Wilson's disease with histologic features of postinfantile giant cell hepatitis; cytomegalovirus as the trigger for both?肝豆状核变性的暴发性肝衰竭伴有婴儿后期巨细胞肝炎的组织学特征;巨细胞病毒是否同时引发了这两种疾病?
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Postinfantile Giant Cell Hepatitis with Features of Acute Severe Autoimmune Hepatitis Probably Triggered by Diclofenac in a Patient with Primary Myelofibrosis.患有原发性骨髓纤维化的患者出现婴儿期后巨细胞性肝炎,伴有急性重症自身免疫性肝炎特征,可能由双氯芬酸引发
Case Reports Hepatol. 2018 Mar 11;2018:9793868. doi: 10.1155/2018/9793868. eCollection 2018.

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