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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

Postinfantile Giant Cell Hepatitis with Features of Acute Severe Autoimmune Hepatitis Probably Triggered by Diclofenac in a Patient with Primary Myelofibrosis.

作者信息

Arvaniti Pinelopi, Zachou Kalliopi, Koukoulis George K, Dalekos George N

机构信息

Department of Medicine and Research Laboratory of Internal Medicine, School of Medicine, University of Thessaly, Larissa, Greece.

Department of Pathology, Medical School, University of Thessaly, Larissa, Greece.

出版信息

Case Reports Hepatol. 2018 Mar 11;2018:9793868. doi: 10.1155/2018/9793868. eCollection 2018.

DOI:10.1155/2018/9793868
PMID:29713554
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5866856/
Abstract

Giant cell hepatitis (GCH) is commonly reported in neonatal and infantile liver diseases but rarely in adults where the term postinfantile GCH (PIGCH) is used. PIGCH is associated with many diseases, including drugs toxicity, viruses, and autoimmune liver diseases, with autoimmune hepatitis (AIH) being the most prevalent. We present a case of PIGCH in a 76-year-old female without known history of liver disease who suffered from an acute severe episode of hepatitis. After careful exclusion of other hepatitis causes by imaging, virological, immunological, and microbiological investigations, a diagnosis of acute severe AIH (AS-AIH) was established. The patient was started on corticosteroids but she did not respond and died 3 days later because of advanced acute liver failure. Postmortem liver biopsy showed typical PIGCH lesions. Physicians must keep this catastrophic entity in mind in cases of unexplained acute liver injury as, contrary to our case, prompt rescue therapy with corticosteroids may be life-saving.

摘要

巨细胞性肝炎(GCH)常见于新生儿和婴儿肝脏疾病,但在成人中很少见,成人病例使用婴儿期后巨细胞性肝炎(PIGCH)这一术语。PIGCH与许多疾病相关,包括药物毒性、病毒感染和自身免疫性肝病,其中自身免疫性肝炎(AIH)最为常见。我们报告一例76岁女性的PIGCH病例,该患者既往无肝脏疾病史,突发急性重症肝炎。通过影像学、病毒学、免疫学和微生物学检查仔细排除其他肝炎病因后,确诊为急性重症AIH(AS-AIH)。患者开始使用皮质类固醇治疗,但无反应,3天后因严重急性肝衰竭死亡。尸检肝脏活检显示典型的PIGCH病变。对于不明原因的急性肝损伤病例,医生必须牢记这一灾难性疾病,因为与我们的病例不同,及时使用皮质类固醇进行抢救治疗可能挽救生命。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a63/5866856/fb2b186f34c9/CRIHEP2018-9793868.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a63/5866856/6e0229b0a41e/CRIHEP2018-9793868.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a63/5866856/fb2b186f34c9/CRIHEP2018-9793868.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a63/5866856/6e0229b0a41e/CRIHEP2018-9793868.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a63/5866856/fb2b186f34c9/CRIHEP2018-9793868.002.jpg

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BMJ Case Rep. 2021 Jul 21;14(7):e243660. doi: 10.1136/bcr-2021-243660.
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Clin J Gastroenterol. 2019 Aug;12(4):367-371. doi: 10.1007/s12328-019-00950-6. Epub 2019 Feb 14.
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