Alpha-1-antitrypsin deficiency in pulmonary and liver degeneration.
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.
D006801:Humans; D008103:Liver Cirrhosis / Q000235:genetics*; D010641:Phenotype; D011656:Pulmonary Emphysema / Q000235:genetics*; D019896:alpha 1-Antitrypsin Deficiency