Vegnente A, Larcher V F, Mowat A P, Portmann B, Williams R
Arch Dis Child. 1984 Apr;59(4):330-5. doi: 10.1136/adc.59.4.330.
Chronic hepatitis implies that clinical and biochemical features of hepatitis have been present for at least 6 months; but irreversible liver damage may occur with subclinical disease suggesting that pathological features should also define chronicity. We examined 28 children with hepatitis B negative chronic active hepatitis to determine whether the severity of abnormal biochemical tests of liver function, raised serum immunoglobulin concentrations, and positive serum autoantibodies, which are characteristic features in immunosuppressant responsive disease, varied with the duration of symptoms. The pattern of abnormality in these patients was similar whether the disease had been present for less than three months, from three to 6 months, or for more than 6 months, and apart from lack of hyperbilirubinaemia was similar in patients presenting with complications of cirrhosis without previous symptoms of liver disease. Two children died of liver disease. The remainder showed a clinical, biochemical, and immunological response to prednisolone or azathioprine, or both. These drugs have now been withdrawn in 8 patients without relapse, and disease activity is completely suppressed in 13. Unfortunately, 15 survivors have definite cirrhosis and a further five have possible cirrhosis. Eight of 10 survivors with symptoms of more than 6 months duration before treatment have cirrhosis compared with two of 12 with symptoms of less than 6 months. It is suggested that consideration of this diagnosis at onset of symptoms and immediate immunosuppressant treatment after appropriate confirmatory investigations may reduce the incidence of cirrhosis in hepatitis B negative chronic active hepatitis in children.
慢性肝炎意味着肝炎的临床和生化特征已持续至少6个月;但亚临床疾病也可能发生不可逆的肝损伤,这表明病理特征也应作为慢性肝炎的定义依据。我们对28例乙型肝炎阴性慢性活动性肝炎患儿进行了检查,以确定肝功能生化检查异常的严重程度、血清免疫球蛋白浓度升高以及血清自身抗体阳性(这些是免疫抑制剂反应性疾病的特征性表现)是否随症状持续时间而变化。无论疾病存在时间少于3个月、3至6个月还是超过6个月,这些患者的异常模式相似,并且除了无高胆红素血症外,在出现肝硬化并发症但既往无肝病症状的患者中也相似。两名儿童死于肝病。其余患者对泼尼松龙或硫唑嘌呤或两者均有临床、生化和免疫学反应。目前,这些药物已在8例患者中停用且未复发,13例患者的疾病活动得到完全抑制。不幸的是,15名幸存者有明确的肝硬化,另有5名可能有肝硬化。治疗前症状持续超过6个月的10名幸存者中有8名患有肝硬化,而症状持续少于6个月的12名幸存者中有2名患有肝硬化。有人认为,在症状出现时考虑这一诊断,并在适当的确诊检查后立即进行免疫抑制治疗,可能会降低儿童乙型肝炎阴性慢性活动性肝炎中肝硬化的发生率。