Gimson A E, O'Grady J, Ede R J, Portmann B, Williams R
Hepatology. 1986 Mar-Apr;6(2):288-94. doi: 10.1002/hep.1840060222.
The clinical, laboratory and histological features of 47 patients with what is defined as late onset hepatic failure are reviewed. Twenty-five of the patients were female and 22 male with a median age of 45 years. Hepatic dysfunction was severe as evidenced by the prolongation of prothrombin time (median = 32 sec, range = 17 to 120 sec). In only four cases was a viral etiology proven (2 hepatitis B, 2 hepatitis A) although the similarity of the clinical features to patients with fulminant viral hepatitis--apart from the longer period of illness prior to the onset of encephalopathy (median = 9 weeks, range = 8 to 24 weeks)--made non-A, non-B infection a possibility in the remainder. There were also similarities to chronic active hepatitis with low titer antibodies to smooth muscle or antinuclear factor in 17% and elevation of the serum IgG in 49%. Liver biopsy in 5 of 8 survivors more than 1 year after initial presentation showed chronic active hepatitis in three. Lobular inflammatory infiltrate, bridging necrosis and multilobular collapse were the features of the acute stage of illness in both the survivors and fatal cases. The patients given corticosteroids did not have a statistically significant improvement in survival, and overall mortality for the series was 81%. Hepatic transplantation, successfully performed in one patient, would appear to offer the best chance of survival for the majority of these patients.
对47例被定义为迟发性肝衰竭患者的临床、实验室及组织学特征进行了回顾。患者中25例为女性,22例为男性,中位年龄45岁。凝血酶原时间延长(中位值=32秒,范围=17至120秒)表明肝功能障碍严重。尽管与暴发性病毒性肝炎患者的临床特征相似——除了脑病发作前病程较长(中位值=9周,范围=8至24周)——但仅4例证实为病毒病因(2例乙型肝炎,2例甲型肝炎),其余患者有可能为非甲非乙型感染。这些患者还与慢性活动性肝炎有相似之处,17%的患者平滑肌或抗核因子抗体滴度低,49%的患者血清IgG升高。8例存活超过1年的患者中,5例在初次就诊1年后进行肝活检,其中3例显示为慢性活动性肝炎。小叶炎性浸润、桥接坏死和多小叶塌陷是存活者和死亡病例急性期的特征。接受皮质类固醇治疗的患者生存率无统计学显著改善,该系列患者的总体死亡率为81%。1例患者成功进行了肝移植,这似乎为大多数此类患者提供了最佳的生存机会。