Severe and moderate alpha-1-antitrypsin deficiency at the age of 30
Alpha-1-antitrypsin (AAT) is a glycoprotein synthesised in the liver. Its main role is to protect lung tissue from destruction by neutrophil elastase. In severe (PiZZ) AAT deficiency, there is an increased risk of emphysema, especially in smokers. The deficiency is caused by the retention and aggregation by polymerization of the AAT molecules in the liver, which increases the risk of neonatal chol