Augmentation therapy of alpha-1 antitrypsin deficiency associated emphysema.
| Autor: | Traclet J; Hospices civils de Lyon, 69000 Lyon, France; Centre de référence des maladies rares pulmonaires, 69000 Lyon, France., Delaval P; IRSET UMR Inserm U1085, université de Rennes 1, 35000 Rennes, France; Centre hospitalier universitaire de Rennes, 35000 Rennes, France., Terrioux P; Cabinet de pneumologie, 77100 Meaux, France., Mornex JF; Hospices civils de Lyon, 69000 Lyon, France; Centre de référence des maladies rares pulmonaires, 69000 Lyon, France; Université Lyon 1, 69000 Lyon, France; INRA, UMR754, 69000 Lyon, France. Electronic address: mornex@univ-lyon1.fr. |
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| Jazyk: | angličtina |
| Zdroj: | Revue des maladies respiratoires [Rev Mal Respir] 2015 Apr; Vol. 32 (4), pp. 435-46. Date of Electronic Publication: 2015 Apr 20. |
| DOI: | 10.1016/j.rmr.2014.10.001 |
| Abstrakt: | Introduction: Alpha-1 antitrypsin, secreted by the liver, inhibits neutrophil elastase. Its deficiency favours the development of emphysema. Restoring a "protective" serum level in deficient patients should make it possible to inhibit the development of emphysema. State of the Art: Human plasma-derived alpha-1 antitrypsin is a blood-derived drug sold in France under the name Alfalastin(®). The recommended posology is an I.V. administration of 60 mg/kg once a week. Human plasma-derived alpha-1 antitrypsin restores anti-elastase protection in the lower lung and prevents experimental emphysema induced by the elastasis of human neutrophils in hamster. The low number of patients with alpha-1 antitrypsin deficiency is one of the difficulties to perform sufficiently powerful randomised studies. However, randomised studies have reported the efficacy of human plasma-derived alpha-1 antitrypsin perfusions on mortality, FEV1 decline and the frequency of exacerbations. Randomised control trials have demonstrated the efficacy of human plasma-derived alpha-1 antitrypsin perfusions on the loss of lung density assessed by CT scan. Conclusion: Augmentation therapy is simple in its conception and implementation, but it is expensive. However, there are currently no other solutions. (Copyright © 2014 SPLF. Published by Elsevier Masson SAS. All rights reserved.) |
| Databáze: | MEDLINE |
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