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巨细胞性肝炎:暴发性肝衰竭的一种罕见病因。

Giant cell hepatitis: an unusual cause of fulminant liver failure.

作者信息

Hartl J, Buettner R, Rockmann F, Farkas S, Holstege A, Vogel C, Schnitzbauer A, Schlitt H J, Schoelmerich J, Kirchner G

机构信息

Klinik und Poliklinik für Innere Medizin I, Universitätsklinikum Regensburg, Franz-Josef-Strauss-Allee 11, Regensburg.

出版信息

Z Gastroenterol. 2010 Nov;48(11):1293-6. doi: 10.1055/s-0029-1245476. Epub 2010 Nov 1.

Abstract

Giant cell hepatitis is a very rare disease of unknown origin. It has been hypothesized that drugs, viral infections, or autoimmune reactions may play a pathogenetic role. Here, we describe a 33 year old patient with bacterial bronchitis who was treated with doxycycline (100 mg/d) for one week. Furthermore the patient complained of malaise and a distinct jaundice. Liver parameters increased dramatically (AST 4670 U/l, ALT 5350 U/l, bilirubin 226 µmol/l) and liver function was impaired (INR = 1,45). The ultrasound scan showed a hepatomegaly with no signs of cirrhosis, normal spleen size and normal bile ducts; liver perfusion was normal. No evidence of Wilson's disease, hemochromatosis, autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cirrhosis, hepatitis A, B, C and E, HIV, CMV, VZV, adenoviral infections, or paracetamol intoxication was found. Subsequently, the patient developed acute liver failure (AST 2134 U/l, ALT 2820 U/l, bilirubin 380 µmol/l, INR 3.0) and a beginning renal failure. Therefore, he was transferred to our transplant center. Due to increasing confusion and somnolence due to cerebral edema mechanical ventilation was needed. Because of an acute renal failure and severe hepatic encephalopathia MARS-hemodialysis was performed. Three weeks after the appearance of the jaundice he underwent liver transplantation (MELD 40). Surprisingly, in the explanted liver the diagnosis of giant cell hepatitis was made. Today--2 years after successful liver transplantation--the patient is in very good condition with normal liver function. In conclusion, giant cell hepatitis is a rare cause of acute liver failure that is often recognized only histologically.

摘要

巨细胞性肝炎是一种病因不明的极为罕见的疾病。据推测,药物、病毒感染或自身免疫反应可能在其发病机制中起作用。在此,我们描述一名33岁患有细菌性支气管炎的患者,其接受了一周的强力霉素(100毫克/天)治疗。此外,该患者主诉不适和明显黄疸。肝脏参数急剧升高(谷草转氨酶4670 U/l,谷丙转氨酶5350 U/l,胆红素226 μmol/l),肝功能受损(国际标准化比值=1.45)。超声扫描显示肝脏肿大,无肝硬化迹象,脾脏大小正常,胆管正常;肝脏灌注正常。未发现威尔逊氏病、血色素沉着症、自身免疫性肝炎、原发性硬化性胆管炎、原发性胆汁性肝硬化、甲型、乙型、丙型和戊型肝炎、艾滋病毒、巨细胞病毒、水痘带状疱疹病毒、腺病毒感染或对乙酰氨基酚中毒的证据。随后,该患者发展为急性肝衰竭(谷草转氨酶2134 U/l,谷丙转氨酶2820 U/l,胆红素380 μmol/l,国际标准化比值3.0)并开始出现肾衰竭。因此,他被转至我们的移植中心。由于脑水肿导致意识混乱和嗜睡加重,需要进行机械通气。由于急性肾衰竭和严重肝性脑病,进行了分子吸附再循环系统血液透析。黄疸出现三周后,他接受了肝脏移植(终末期肝病模型评分40)。令人惊讶的是,在切除的肝脏中确诊为巨细胞性肝炎。如今——肝移植成功两年后——患者状况良好,肝功能正常。总之,巨细胞性肝炎是急性肝衰竭的罕见病因,通常仅在组织学上才能确诊。

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