Alpha 1 antitrypsin deficiency is associated with?
Correct Answer: Panacinar-emphysema
Description: Ans. is 'a' i.e., Panacinar-emphysema Emphysema Emphysema is a condition of the lung characterized by abnormal permanant enlargement of the airspaces distal to the terminal bronchioles, accompanied by destruction of their walls and without obvious fibrosis. "Destruction of walls" is necessory to define emphysema. Enlargement of airspaces without destruction of their walls is termed overinflation, for example, the distention of airspaces that occurs in the remaining lung after unilateral pneumonectomy. Types of emphysema Emphysema is classified according to its anatomic distribution within the lobule. There are four major types of emphysema ? 1. Centriacinar (centrilobular) emphysema Centriacinar emphysema is the most common type of emphysema seen clinically. It is chracterized by involvement of respiratory bronchioles, i.e. central (proximal) pa of the acinus. So, both emphysematous and normal airspaces exist within the same acinus and lobule. The lesions are more common and more severe in the upper lobe, paicularly in the apical segments. This is the type of emphysema that occurs predominantly in heavy smokers and usually coexists with chronic bronchitis. 2. Panacinar (Panlobular) emphysema The acini are uniformly enlarged from the level of the respiratory bronchiole to the terminal blind alveoli. This type of emphysema 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 base. This type of emphysema is associated with al-antitrypsin deficiency. 3. Distal acinar (Paraseptal) emphysema This type of emphysema involves distal pa of the acinus, while proximal pa is normal. It is localized adjacent to the pleura, along perilobular septa. The involvement is seen adjacent to areas of fibrosis, scarring, or atelectasis and is usually more severe in the upper half of lungs. This type of emphysema is a common cause of spontaneous pneumothorax in young adults. 4. Irregular emphysema (Para-cicatricial emphysema) The acinus is involved irregularly and is almost invariably associated with scarring. It is the most common type of emphysema histologically. Most common type of emphysema is irregular emphysema, but it is not clinically significant as most patients are asymptomatic and it is only an autopsy finding. Most common type of emphysema seen clinically is centracinar emphysema.
Category:
Pathology
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