Teufel M, Niessen K H, Berg P A
Eur J Pediatr. 1983 Mar;140(1):30-3. doi: 10.1007/BF00661901.
This paper describes a special form of chronic active hepatitis (CAH) with hypergammaglobulinemia in a ten-year-old girl. Because of unclear symptoms she was admitted to our hospital for exclusion of leukemia. However, further clinical and laboratory investigations suggested an autoimmune hepatitis although the typical antibodies could not be found in the immunofluorescence test. Only the detection of liver-pancreas-specific, complement-fixing autoantibodies (LP-antibodies) confirmed the diagnosis of an autoimmune hepatitis. Histology showed typical infiltrations of plasma cells and lymphocytes in the periportal area. LP-antibodies had been recently described in 20 adults with HBs-Ag-negative CAH[6]. To our knowledge this is the first report on LP-positive hepatitis in childhood. As a therapeutic response is likely, early immunosuppressive treatment is indicated.
本文描述了一名10岁女孩患有的一种伴有高球蛋白血症的特殊形式的慢性活动性肝炎(CAH)。由于症状不明确,她被收治入我院以排除白血病。然而,进一步的临床和实验室检查提示为自身免疫性肝炎,尽管在免疫荧光试验中未发现典型抗体。仅肝 - 胰腺特异性补体结合自身抗体(LP抗体)的检测确诊为自身免疫性肝炎。组织学检查显示门静脉周围区域有典型的浆细胞和淋巴细胞浸润。LP抗体最近在20例HBs - Ag阴性的成人CAH患者中被描述[6]。据我们所知,这是关于儿童LP阳性肝炎的首例报告。鉴于可能有治疗反应,建议早期进行免疫抑制治疗。