alpha1-proteinase inhibitor

Ligand id: 7487

Name: alpha1-proteinase inhibitor

No information available.
Summary of Clinical Use
Exogenous alpha-1-proteinase inhibitor is used to treat emphysemic patients with severe congenital enzyme deficiency (aka augmentation therapy). Alpha-1-proteinase inhibitor deficiency is associated with progressive and ultimately fatal emphysema, due to unfettered neutrophil elastase breakdown of elastin in lung tissue. Enzyme deficiency is also associated with several forms of liver disease, including cirrhosis, neonatal hepatitis, and hepatocellular carcinoma. If intravenous augmentation therapy is unsucessful, organ transplantation may be the only option for patients with end-stage lung or liver disease.
Mechanism Of Action and Pharmacodynamic Effects
Inhibits neutrophil elastase in the lungs. Currently available drugs are administered intravenously. Inhalation formulations are being evaluated as a method of delivering the drug directly to the lungs.