PEIR Digital Library

Welcome to the Pathology Education Informational Resource (PEIR) Digital Library, a multidisciplinary public access image database for use in medical education.

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RADIOLOGY: HEPATOBILIARY: Case# 3: EMPHYSEMATOUS CHANGE SECONDARY TO ALPHA I ANTITRYPSIN DEFICIENCY, CIRRHOSIS. 61 year old man referred with hepatocellular carcinoma to rule out metastasis. Bilateral lower-zone predominant emphysema is present. No pulmonary or pleural metastases. A low attenuation lesion is identified in the right posterior segement of the liver. The liver is small with a nodular contour. Alfa 1-Antitrypsin (A1-AT) is a secretory glycoprotien produced by the hepatocytes. Patients with homozygous ZZ or SS phenotype develop A1-AT deficiency which leads to early onset panacinar emphysema predominantly affecting the lower lobes as shown above. Associated bronchiectasis may also be seen. These patients also develop cirrhosis and leucocytoclastic vasculitis. Concomitant smoking and or environmental exposure hastens development of emphysema. Treatment includes intravenous substitution of A1-AT.