Dubois R S, Silverman A
Postgrad Med J. 1974 Jun;50(584):386-91. doi: 10.1136/pgmj.50.584.386.
Thirty-eight children with chronic active hepatitis are reported. Their mode of presentation was usually indistinguishable from acute viral hepatitis (63%). Extra hepatic manifestations were more common in those patients with an insidious onset. The twenty-eight patients tested for hepatitis B antigen (HB Ag) were all negative and SGOT values were higher (mean 1000 i.u./l) than those reported in adult patients with chronic active hepatitis. Thirty-three patients had marked elevation of serum gammaglobulin (mean 3·78 g/100 ml) and 34% had positive LE cells. All patients had histologic features of ‘aggressive’ hepatitis; in addition five biopsies also showed areas of submassive hepatic necrosis. The initial response to corticosteroid was excellent in all but three patients. To date drug therapy has been suspended in fifteen patients for periods of 3 months to 4 years and 7 months. There have been eight deaths. Three with hepatic insufficiency and two with sepsis. Three others have subsequently died following orthotopic liver transplantation.
报告了38例慢性活动性肝炎患儿。他们的临床表现通常与急性病毒性肝炎难以区分(63%)。肝外表现在起病隐匿的患者中更为常见。检测乙肝抗原(HB Ag)的28例患者均为阴性,谷草转氨酶(SGOT)值(平均1000国际单位/升)高于成年慢性活动性肝炎患者报告的数值。33例患者血清γ球蛋白显著升高(平均3.78克/100毫升),34%的患者狼疮细胞阳性。所有患者均有“侵袭性”肝炎的组织学特征;此外,5例活检还显示有亚大块肝坏死区域。除3例患者外,所有患者对皮质类固醇的初始反应良好。迄今为止,15例患者已停药3个月至4年零7个月。已有8例死亡。3例死于肝功能不全,2例死于败血症。另外3例在原位肝移植后死亡。