1989•PubMedRequires access
[Alpha 1-antitrypsin deficiency and emphysema. Replacement therapy?].
Simen Grøneng Johansen, Anne Kristine Gulsvik
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Abstract
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.