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<datestamp>2017-12-21</datestamp>
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<publisher>HAL CCSD</publisher>
<title lang=en>Traitement substitutif de l’emphysème au cours du déficit en alpha-1 antritrypsine [Augmentation therapy of alpha-1 antitrypsin deficiency associated emphysema]</title>
<creator>Traclet, J.</creator>
<creator>Delaval, P.</creator>
<creator>Terrioux, P.</creator>
<creator>Mornex, J.-F.</creator>
<contributor>Hospices Civils de Lyon</contributor>
<contributor>Institut de recherche, santé, environnement et travail [Rennes] (Irset) ; Université d'Angers (UA) - Université des Antilles et de la Guyane (UAG) - Université de Rennes 1 (UR1) - 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 )</contributor>
<description>International audience</description>
<source>Revue Des Maladies Respiratoires</source>
<identifier>hal-01146841</identifier>
<identifier>https://hal-univ-rennes1.archives-ouvertes.fr/hal-01146841</identifier>
<source>https://hal-univ-rennes1.archives-ouvertes.fr/hal-01146841</source>
<source>Revue Des Maladies Respiratoires, 2015, 32 (4), pp.435-446. 〈10.1016/j.rmr.2014.10.001〉</source>
<identifier>DOI : 10.1016/j.rmr.2014.10.001</identifier>
<relation>info:eu-repo/semantics/altIdentifier/doi/10.1016/j.rmr.2014.10.001</relation>
<identifier>PUBMED : 25908241</identifier>
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<language>en</language>
<subject>[SDV] Life Sciences [q-bio]</subject>
<type>info:eu-repo/semantics/article</type>
<type>Journal articles</type>
<description lang=en>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.</description>
<date>2015</date>
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