a1 antitrypsin deficiency is seen in
Correct Answer: Emphysema
Description: i.e. (Emphysema): (486-Basicpathology 8th) (1638-H17th)Panacinar (Pan lobular) emphysema is associated with a-lAT deficiency which has a predilection for the lower lobesPatients with severe a 1-antiprotease (a 1-antitrypsin) deficiency are recognized by family history and the appearance of panacinar bibasilar emphysema early in life usually in the third or fourth decade; hepatic cirrhosis and hepatocellular carcinoma may occur (258-CMDT 11th)Panacinar emphysemaCentriacinar emphysema* Acini are uniformly involved from level of respiratory bronchiole to terminal blind alveoli* Central or proximal parts of acini formed by respiratory bronchioles are affected whereas distal alveoli are spared* Lesions are more common in lower zone and bases* Lesions are more severe and common in upper lobes* Occurs in association with a1 antitrypsin deficiency* Occurs predominantly in smokers*** Is the commonest pattern
Category:
Pathology
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