Augmentation therapy of alpha-1 antitrypsin deficiency associated emphysema

摘要: Summary Introduction Alpha-1 antitrypsin, secreted by the liver, inhibits neutrophil elastase. Its deficiency favours development of emphysema. Restoring a “protective” serum level in deficient patients should make it possible to inhibit State art Human plasma-derived alpha-1 antitrypsin is blood-derived drug sold France under name Alfalastin®. The recommended posology an I.V. administration 60 mg/kg once week. restores anti-elastase protection lower lung and prevents experimental emphysema induced elastasis human neutrophils hamster. low number with one difficulties perform sufficiently powerful randomised studies. However, studies have reported efficacy perfusions on mortality, FEV1 decline frequency exacerbations. Randomised control trials demonstrated loss density assessed CT scan. Conclusion Augmentation therapy simple its conception implementation, but expensive. there are currently no other solutions.