A 45-year-old man with cirrhosis due to al-antitrypsin deficiency receives a liver transplant. He is at increased risk of developing which of the following types of emphysema?
Correct Answer: Panacinar
Description: Ans: C (Panacinar) Ref: Robbins ami Cotran 'Pathologic Basis of Disease' 8th Edition. Elsevier, 2011. Pg 684Explanation:Types of EmphysemaCentriacinar (Centrilobular) Emphysema.The centra! or proximal parts of the acini, formed by respiratory bronchioles, are affected, where as distal alveoli are spared.Both emphysematous and normal airspaces exist within the same acinus and lobule.The lesions are more common and usually more severe in the upper lobes, particularly in the apical segments.The walls of the emphysematous spaces often contain large amounts of black pigment.Inflammation around bronchi and bronchioles is common.In severe centriacinar emphysema, the distal acinus may also be involved, and differentiation from panacinar emphysema becomes difficult.Centriacinar emphysema occurs predominantly in heavy smokers, often in association with chronic bronchitis.Panacinar (Panlobular) Emphysema.The acini are uniformly enlarged from the level of the respiratory bronchiole to the terminal blind alveoli.The prefix "pan" refers to the entire acinus but not to the entird lung.This tends to occur more commonly in the lower zones and in the anterior margins of the lung, and it is usually most severe at the bases.This type of emphysema is associated with al-antitrypsin (al-AT) deficiency.Distal Acinar (Paraseptal) Emphysema.The proximal portion of the acinus is normal, and the distal part is predominantly involved.The emphysema is more striking adjacent to the pleura, along the lobular connective tissue septa, and at the margins of the lobules.It occurs adjacent to areas of fibrosis, scarring, or atelectasis and is usually more severe in the upper half of the lungs.The characteristic findings are of multiple, continuous, enlarged airspaces from less than 0.5 cm to more than 2.0 cm in diameter, sometimes forming cystlike structures.This type of emphysema probably underlies many of the cases of spontaneous pneumothorax in young adults.Airspace Enlargement with Eibrosis (Irregular Emphysema).The acinus is irregularly involved and almost invariably associated with scarring.It may be the most common form of emphysema, because careful search of most lungs at autopsy show's one or more scars from a healed inflammatory process.In most instances, these foci of irregular emphysema are asymptomatic and clinically" insignificant.
Category:
Pathology
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