STUDIES OF ALPHA-1-ANTITRYPSIN PHENOTYPES AND METABOLISM

蛋白酶抑制剂（Pi）表型已使用 聚丙烯酰胺凝胶等电聚焦技术在正常人群中的应用 患有类风湿性关节炎、系统性狼疮 红斑、干燥综合征和肝细胞癌。 80名南非黑人患者， 肝细胞癌，异常（非MM）的发生率 表型占8.7%。 在103名年龄、性别和部落匹配的对照组中， 受试者相应发生率为12.6%。 概无 患者或对照具有piZZ表型。 5%的患者， 1.9%的对照为Z基因杂合子携带者。 没有 一名肝细胞癌患者的血清 α-L-抗胰蛋白酶的浓度。 四个病人 杂合子Z表型没有纤维板层癌。 这些发现表明，α-l-抗胰蛋白酶缺乏症 在南方肝癌的病因中起作用 非洲黑人

Protease inhibitor (Pi) phenotypes have been determined using isoelectric focusing on polyacrylamide gel in populations of normal subjects and patients with rheumatoid arthritis, systemic lupus erythematosus, Sjogren's syndrome and hepatocellular carcinoma. Of 80 unselected southern African Black patients with hepatocellular carcinoma, the incidence of aberrant (non-MM) phenotypes was 8.7%. In 103 age, sex and tribally-matched control subjects the corresponding incidence was 12.6%. None of the patients or controls had the piZZ phenotype. 5% of patients and 1.9% of controls were heterozygous carriers of the Z gene. No patient with hepatocellular carcinoma had a subnormal serum concentration of alpha-l-antitrypsin. The four patients with the heterozygous Z phenotype did not have fibrolamellar carcinomas. These findings suggest that alpha-l-antitrypsin deficiency does not play an etiologic role in hepatocellular carcinoma in southern African Blacks.