A 26-year-old non-smoking woman affected by AATD and bullous pulmonary emphysema, who has an undetectable AAT serum level and Null/Null genotype homozygous for Q0Ourem, has been receiving AAT augmentation therapy at a dose of 60 mg/kg/week for approximately 5 years. This evidence concerns the gene SERPINA1 and alpha 1-antitrypsin deficiency.