Most common diseases related to severe AAT-deficiency are pulmonary emphysema (found in around 3% of adults with COPD), liver cirrhosis (2.5% of children and 10-20% of PI*Z adult homozygotes), systemic vasculitis (7%) and relapsing panniculitis (0.1%) [3,39,40]. Here, SERPINA1 is linked to hyperinsulinemic hypoglycemia, familial, 4.