Blenkinsopp W K, Haffenden G P
J Clin Pathol. 1977 Feb;30(2):132-7. doi: 10.1136/jcp.30.2.132.
The cytoplasmic bodies in hepatocytes thought to indicate possession of the Z allele for alpha 1-antitrypsin deficiency were found in necropsy in 10 of 64 adults with cirrhosis, four of nine with hepatic fibrosis, and four of 15 with hepatocellular carcinoma. They were also found in six of 76 adults with severe panacinar emphysema, and in four of a control series of 110 adults with neither emphysema nor liver disease. The association of the bodies with each of the three liver diseases was statistically significant, but the association of the bodies with emphysema was not. It is considered probable that heterozygous (PiMZ) alpha 1-antitrypsin deficiency is associated with an increased incidence of cirrhosis, hepatic fibrosis, and hepatocellular carcinoma.
在尸检中发现,64例肝硬化成年患者中有10例、9例肝纤维化患者中有4例、15例肝细胞癌患者中有4例的肝细胞中存在被认为表明携带α1-抗胰蛋白酶缺乏症Z等位基因的胞质小体。在76例严重全腺泡型肺气肿成年患者中有6例也发现了这些胞质小体,在110例既无肺气肿也无肝病的对照成年患者中有4例发现了它们。这些胞质小体与三种肝脏疾病中的每一种的关联具有统计学意义,但与肺气肿的关联不具有统计学意义。据认为,杂合子(PiMZ)α1-抗胰蛋白酶缺乏症可能与肝硬化、肝纤维化和肝细胞癌的发病率增加有关。