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. 2010 Mar;35(3 Section 2):2-6.

Alpha(1)-proteinase inhibitor (human)

No authors listed

Alpha(1)-proteinase inhibitor (human)

No authors listed. P T. 2010 Mar.
No abstract available

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Figures

FIGURE 1
FIGURE 1
Number of Physicians Seen Before an Accurate Diagnosis of AATD is Made (By Year)
FIGURE 2
FIGURE 2
When neutrophil elastase docks on the reactive center loop of AAT (a), it induces a conformational change in the antiprotease, where the reactive center snaps back to the opposite pole (b). Mutations in AAT can alter this process and lead to biologic impairments consistent with several diseases.
FIGURE 3
FIGURE 3
Kaplan-Meier Plot of Cumulative Mortality From Time of Enrollment for Subjects With FEV1 <50% Predicted: Always, Partly, and Never Receiving Augmentation Therapy
FIGURE 4
FIGURE 4
Serum Levels of Alpha1-Antitrypsin Significantly Increased (P<0.001) During Active Treatment With Augmentation Therapy (Closed Circles) Compared With Placebo (Open Circles)
FIGURE 5
FIGURE 5
Number of Respiratory Infections per Year During Augmentation Therapy With Alpha1-PI (Prolastin®) Among 89 Patients with PI*ZZ AATD
FIGURE 6
FIGURE 6
Mean ± SD of Trough Serum Antigenic Alpha1-PI During Weeks 7–11
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References

    1. Alpha-1 Facts. Alpha-1 Association Web site. http://www.alpha1.org Accessed Dec. 11, 2009.
    1. ATS (American Thoracic Society)/European Respiratory Society Statement Standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003;168:818–900. - PubMed
    1. ATTD (Alpha 1-Antitrypsin Deficiency Registry Study Group) Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin. Am J Respir Crit Care Med. 1998;158:49–59. - PubMed
    1. Campos MA, Wanner A, Zhang G, Sandhaus RA. Trends in the diagnosis of symptomatic patients with α-1 antitrypsin deficiency between 1968 and 2003. Chest. 2005;128:1179–1186. - PubMed
    1. Carrell RW, Lomas DA. Alpha1-antitrypsin deficiency: a model for conformational diseases. N Engl J Med. 2002;346:45–53. - PubMed

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