Fagerhol M K
Birth Defects Orig Artic Ser. 1974;10(4):208-11.
The characteristics of emphysema and cirrhosis in Pi ZZ (alpha-1-antitrypsin deficiency) and SZ patients are reviewed. The clinical and laboratory data have been incorporated into a simple hypothesis on the development of these diseases. The PiZ protein can not be secreted normally from the liver cells. The accumulation of alpha-1-antitrypsin in the liver may result in cirrhosis, and the deficiency in serum to emphysema.
回顾了Pi ZZ（α-1抗胰蛋白酶缺乏症）和SZ患者肺气肿和肝硬化的特征。临床和实验室数据已被纳入关于这些疾病发展的一个简单假说中。PiZ蛋白无法从肝细胞正常分泌。α-1抗胰蛋白酶在肝脏中的积累可能导致肝硬化，而血清中缺乏该蛋白则会导致肺气肿。
