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Long-term experience in the treatment of α1-antitrypsin deficiency: 25 years of augmentation therapy

Helmut Teschler
European Respiratory Review 2015 24: 46-51; DOI: 10.1183/09059180.10010714
Helmut Teschler
Dept of Respiratory Medicine, West German Lung Clinic, Essen University Hospital, Essen, Germany
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  • For correspondence: Helmut.Teschler@Ruhrlandklinik.UK-Essen.de
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  • FIGURE 1
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    FIGURE 1

    Suggested areas corresponding to severe α1-antitrypsin (AAT) deficiency (below the protective threshold) and intermediate AAT deficiency (above the protective threshold and below the 10th percentile of the AAT range for subjects carrying the PI*MS genotype). Bars represent the 5th and 95th percentiles of AAT serum levels. Even MZ can have normal serum levels. Reproduced from [3] with permission from the publisher.

  • FIGURE 2
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    FIGURE 2

    Cumulative mortality over 60 months in patients with α1-antitrypsin deficiency and an initial forced expiratory volume in 1 s <50% predicted who never (n=382), partially (n=357) or always (n=390) received augmentation therapy. Reproduced with modification from [8] with permission from the publisher.

  • FIGURE 3
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    FIGURE 3

    Change from baseline in total lung capacity (TLC)-adjusted 15th percentile lung density (PD15) over the course of the study. The error bars indicate sem. Estimated treatment difference in mean slopes (annual change in lung density) 0.857 (p=0.068). Reproduced with modification from [13] with permission.

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  • TABLE 1

    Commercially available preparations of purified α1-antitrypsin

    DrugManufacturerSourceDedicated pathogen safety stepsRegulatory approval
    ProlastinGrifols (Barcelona, Spain)Pooled human plasmaPasteurisationAustria, Belgium, Denmark, Finland, Germany, Greece, Ireland, Italy, Netherlands, Norway, Poland, Portugal, Spain, Sweden and Switzerland
    Prolastin CGrifols (Barcelona, Spain)Pooled human plasmaS/D purification + nanofiltrationUSA, Canada, Argentina and Colombia
    TrypsoneGrifols (Barcelona, Spain)Pooled human plasmaS/D purification + nanofiltrationSpain, Argentina, Brazil, Chile and Mexico
    Aralast NPBaxter (Deerfield, IL, USA)Pooled human plasmaS/D purification + nanofiltrationUSA
    ZemairaCSL Behring (King of Prussia, PA, USA)Pooled human plasmaPasteurisation + nanofiltrationUSA and Brazil
    GlassiaKamada (Ness Ziona, Israel)Pooled human plasmaS/D purification + nanofiltrationUSA and Brazil
    AlfalastinLFB (Courtabœuf, France)Pooled human plasmaPasteurisationFrance
    • S/D: solvent/detergent. Data from [6].

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    • H. Teschler
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Long-term experience in the treatment of α1-antitrypsin deficiency: 25 years of augmentation therapy
Helmut Teschler
European Respiratory Review Mar 2015, 24 (135) 46-51; DOI: 10.1183/09059180.10010714

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Long-term experience in the treatment of α1-antitrypsin deficiency: 25 years of augmentation therapy
Helmut Teschler
European Respiratory Review Mar 2015, 24 (135) 46-51; DOI: 10.1183/09059180.10010714
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  • Article
    • Abstract
    • Abstract
    • Introduction
    • Goals of augmentation therapy with AAT
    • Other clinical manifestations of AATD and the role of AAT augmentation therapy in lung transplant recipients
    • Conclusion
    • Acknowledgements
    • Footnotes
    • References
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  • Medications for the treatment of pulmonary arterial hypertension
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