Theaker J M, Fleming K A
J Clin Pathol. 1986 Jan;39(1):58-62. doi: 10.1136/jcp.39.1.58.
One hundred and eighty five consecutive liver biopsies were immunostained using anti-alpha-1-antitrypsin to assess the use of routine immunohistochemistry in the diagnosis of alpha-1-antitrypsin (AAT) deficiency. About half the livers showed staining of hepatocytes for alpha-1-antitrypsin, but most of these livers showed a panlobular pattern, possibly indicating increased synthesis of AAT. Only ten contained periportal granules, said to be typical of AAT deficiency. In cases in which serum was also available for quantitation and phenotyping there was no absolute relation between staining pattern, phenotype, and serum concentrations: the immunohistological screening technique, therefore, has limitations in the diagnosis of AAT deficiency in liver biopsy specimens.
对185例连续肝活检组织进行抗α-1-抗胰蛋白酶免疫染色,以评估常规免疫组化在α-1-抗胰蛋白酶(AAT)缺乏症诊断中的应用。约一半的肝脏显示肝细胞有α-1-抗胰蛋白酶染色,但这些肝脏大多呈现全小叶模式,可能表明AAT合成增加。只有10例含有门周颗粒,据称这是AAT缺乏症的典型表现。在血清也可用于定量和表型分析的病例中,染色模式、表型和血清浓度之间没有绝对关联:因此,免疫组织学筛查技术在肝活检标本中AAT缺乏症的诊断方面存在局限性。