Traitement substitutif de l’emphysème au cours du déficit en alpha-1 antritrypsine [Augmentation therapy of alpha-1 antitrypsin deficiency associated emphysema]

Autor: Traclet, J., Delaval, P., Terrioux, P., Mornex, J.-F.
Přispěvatelé: Hospices Civils de Lyon (HCL), Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )
Jazyk: angličtina
Rok vydání: 2015
Předmět:
Zdroj: Revue des Maladies Respiratoires
Revue des Maladies Respiratoires, Elsevier Masson, 2015, 32 (4), pp.435-446. ⟨10.1016/j.rmr.2014.10.001⟩
Revue des Maladies Respiratoires, 2015, 32 (4), pp.435-446. ⟨10.1016/j.rmr.2014.10.001⟩
ISSN: 0761-8425
1776-2588
Popis: International audience; 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 60mg/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.
Databáze: OpenAIRE