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Update on α1-antitrypsin deficiency

Ilaria Ferrarotti, Stefania Ottaviani, Annalisa De Silvestri, Angelo G. Corsico
Breathe 2018 14: e17-e24; DOI: 10.1183/20734735.015018
Ilaria Ferrarotti
1Center for Diagnosis of Inherited Alpha1-antitrypsin Deficiency, Dept of Internal Medicine and Therapeutics, Pneumology Unit, Fondazione IRCCS Policlinico San Matteo, Università di Pavia, Pavia, Italy
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Stefania Ottaviani
1Center for Diagnosis of Inherited Alpha1-antitrypsin Deficiency, Dept of Internal Medicine and Therapeutics, Pneumology Unit, Fondazione IRCCS Policlinico San Matteo, Università di Pavia, Pavia, Italy
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Annalisa De Silvestri
2Statistics Dept, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
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Angelo G. Corsico
1Center for Diagnosis of Inherited Alpha1-antitrypsin Deficiency, Dept of Internal Medicine and Therapeutics, Pneumology Unit, Fondazione IRCCS Policlinico San Matteo, Università di Pavia, Pavia, Italy
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Article Information

vol. 14 no. 2 e17-e24
DOI 
https://doi.org/10.1183/20734735.015018

Published By 
European Respiratory Society
Print ISSN 
1810-6838
Online ISSN 
2073-4735
History 
  • Published online July 11, 2018.

Copyright & Usage 
Copyright ©ERS 2018 Breathe articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

Author Information

  1. Ilaria Ferrarotti1,
  2. Stefania Ottaviani1,
  3. Annalisa De Silvestri2 and
  4. Angelo G. Corsico1
  1. 1Center for Diagnosis of Inherited Alpha1-antitrypsin Deficiency, Dept of Internal Medicine and Therapeutics, Pneumology Unit, Fondazione IRCCS Policlinico San Matteo, Università di Pavia, Pavia, Italy
  2. 2Statistics Dept, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
  1. E-mail: i.ferrarotti{at}smatteo.pv.it
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Vol 14 Issue 2 Table of Contents
Breathe: 14 (2)
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Update on α1-antitrypsin deficiency
Ilaria Ferrarotti, Stefania Ottaviani, Annalisa De Silvestri, Angelo G. Corsico
Breathe Jun 2018, 14 (2) e17-e24; DOI: 10.1183/20734735.015018

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Update on α1-antitrypsin deficiency
Ilaria Ferrarotti, Stefania Ottaviani, Annalisa De Silvestri, Angelo G. Corsico
Breathe Jun 2018, 14 (2) e17-e24; DOI: 10.1183/20734735.015018
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  • Article
    • Abstract
    • Abstract
    • Pathogenesis of AATD in the lungs
    • Different α1-AT variants have a different prognosis
    • The progression of lung damage in severe AATD
    • The lung damage progression associated with intermediate AATD
    • The paradigm of null mutations
    • Augmentation therapy and lung damage progression: a meta-analysis
    • Conclusions
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
  • PDF

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  • COPD and smoking
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