[Alpha 1-antitrypsin deficiency and emphysema. Replacement therapy?].

We present three patients with homozygous alpha-1-antitrypsin deficiency and pulmonary emphysema. They demonstrate the typical patterns of this syndrome: panlobular emphysema, early age of onset, serum alpha-1-antitrypsin below 35% of normal and phenotype PiZZ. We discuss epidemiology, pathogenesis and clinical manifestations, and review the experience concerning replacement therapy using alpha-1-antitrypsin derived from human plasma. We strongly point out the importance of stopping cigarette smoking in such patients.