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Emphysema, Cirrhosis, and Hepatoma with Alpha-1 Antitrypsin Deficiency

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Department of Respiratory Diseases, City of Hope National Medical Center, Duarte, California

Ann Intern Med. 1974;81(6):850-852. doi:10.7326/0003-4819-81-6-850
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The role of alpha-1 antitrypsin deficiency in predisposing to pulmonary emphysema has been universally accepted, and even the likelihood that an intermediate deficiency may play such a role is receiving growing support. Twenty-four molecular variants of antitrypsin have been discovered in man, but antitrypsin deficiency is usually associated with the Z variant. This variant has the lowest blood levels of antitrypsin and is the only variant whose serum concentration does not rise after an appropriate stimulus. The Z variant is also characterized by the presence of unusual intrahepatic globules immunologically related to antitrypsin, and this finding has focused much of


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