Abstract

A patient who manifested both pulmonary and hepatic disease associated with alpha1-antitrypsin deficiency is described. The biological function of alpha1-antitrypsin is discussed, as well as the inheritance of deficiency states and the spectrum of disorders which may ensue. Severe deficiency of alpha1-antitrypsin has been linked with pulmonary emphysema in adult life, progressive liver disease in childhood, adult cirrhosis but rarely with both pulmonary and liver disease.

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