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"Alpha ANTITRYPSIN DEFICIENCY "

“ Alpha  antitrypsin is an inhibitor of a protease for elastin. The deficiency is a lso associated with cirrhosis, necrotising panniculitis ...

Alpha  antitrypsin is an inhibitor of a protease for elastin. The deficiency is also associated with cirrhosis, necrotising panniculitis and Wegener granulomatosis. It causes a pan-lobar emphysema in the lungs in young patients (aged 40-50 years).
PLAIN FILM 
•  Normal in mild disease
•  Later on, lucent, hyperinflated lungs in a young patient
HRCT 
• Pan-lobular emphysema (i.e. enlarged and destroyed secondary pulmonary lobule) tending to affect the lower lobes bilaterally
• Associated with bronchiectasis
al antitrypsin deficiency. Chest x-ray demonstrating hyperexpanded lungs and predominant lower zone emphysema.
 al antitrypsin deficiency. Computed tomography image demonstrating marked pan-lobular emphysema.