Zenda Takahiro, Araki Ichiro, Sasaki Motoko
Department of Internal Medicine, Asanogawa General Hospital, Kosaka-machi Naka 83, Kanazawa 920-8621, Japan.
Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan.
Clin J Gastroenterol. 2019 Aug;12(4):367-371. doi: 10.1007/s12328-019-00950-6. Epub 2019 Feb 14.
Giant cell hepatitis in adults is considered a rapidly progressive and life-threatening disease, but there are few descriptions of a prolonged disease course. A 36-year-old Japanese man was referred to our hospital for further evaluation of abnormal liver function test results. Although asymptomatic, he had undergone follow-up for 9 years with these abnormalities. Because the cause of liver injury was not identified despite extensive noninvasive examinations, the patient underwent needle biopsy. He was finally diagnosed with post-infantile giant cell hepatitis (PIGCH) based on the presence of small numbers of giant multinucleated hepatocytes scattered primarily around the portal area. Necroinflammatory changes were very mild in the portal tracts and hepatic parenchyma. According to the histological findings as well as the accepted international diagnostic scoring system for autoimmune hepatitis (AIH), which is closely related to PIGCH, AIH was unlikely, although antinuclear antibody was positive at a titer of 1:160. The present case may describe an unknown subtype of PIGCH, characterized by insidious disease onset and progression with concurrent, mildly active underlying hepatitis, which is in contrast with the well-documented aggressive nature of PIGCH.
成人巨细胞性肝炎被认为是一种进展迅速且危及生命的疾病,但关于病程迁延的描述较少。一名36岁的日本男性因肝功能检查结果异常被转诊至我院作进一步评估。尽管无症状,但他因这些异常情况已接受了9年的随访。由于尽管进行了广泛的非侵入性检查仍未明确肝损伤的原因,该患者接受了肝穿刺活检。最终,基于主要散在于门周区域的少量巨大多核肝细胞,他被诊断为婴儿期后巨细胞性肝炎(PIGCH)。门管区和肝实质的坏死性炎症改变非常轻微。根据组织学 findings 以及与PIGCH密切相关的公认的自身免疫性肝炎(AIH)国际诊断评分系统,尽管抗核抗体滴度为1:160呈阳性,但AIH的可能性不大。本病例可能描述了一种未知亚型的PIGCH,其特征为起病隐匿、进展缓慢且伴有轻度活动性潜在肝炎,这与文献记载的PIGCH的侵袭性本质形成对比。 (注:原文中“findings”未翻译,可能是原文有误,应为“发现”之类的意思,这里保留英文以便你参考原文情况)