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Alpha1-proteinase inhibitor (human) is a sterile, stable, lyophilized preparation of purified human alpha1-proteinase inhibitor (A1-PI), also known as alpha1-antitrypsin (AAT). It is used as chronic augmentation therapy in individuals with congenital deficiency of A1-PI and clinically evident emphysema. AAT is a serine protease inhibitor (serpin) that primarily neutralizes neutrophil elastase in the lungs. In patients with AAT deficiency, the lack of this inhibitor leads to uninhibited elastase activity, which destroys alveolar walls and results in progressive pulmonary emphysema. Augmentation therapy with the human-derived protein increases the levels of A1-PI in the blood and lung epithelial lining fluid, thereby protecting lung tissue from proteolytic degradation. Major developers and manufacturers of these plasma-derived products include Grifols, CSL Behring, Takeda, and Kamada.
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