This medicine contains the active substance human alpha1-proteinase inhibitor, which is a normal component of the blood and is found in the lung.

There, its main function is to protect the lung tissue by limiting the action of a certain enzyme, called neutrophil elastase. Neutrophil elastase can cause damage if its action is not controlled (for example, in case you have an alpha1-proteinase inhibitor deficiency).

Human alpha1-proteinase inhibitor is a normal constituent of human blood. 

Human alpha1-proteinase inhibitor is understood to be the primary anti-protease in the lower respiratory tract, where it inhibits neutrophil elastase (NE).

Normal healthy individuals produce sufficient alpha1-proteinase inhibitor to control the NE produced by activated neutrophils and are thus able to prevent inappropriate proteolysis of lung tissue by NE. However, individuals deficient in endogenous alpha1-proteinase inhibitor are unable to maintain appropriate antiprotease defence and experience more rapid proteolysis of the alveolar walls starting prior to the development of clinically evident chronic obstructive lung disease in the third or fourth decade.

The active substance is human alpha1-proteinase inhibitor. One vial contains approximately 1,000 mg, 4,000 mg or 5,000 mg of human alpha1-proteinase inhibitor.

The other ingredients are sodium chloride, sodium dihydrogen phosphate monohydrate and mannitol (see section 2).

Solvent: Water for injections.

One pack contains:
- 1 single-use powder vial
- 1 solvent vial of 20 ml water for injections
- 1 transfer set 20/20 (Mix2Vial set) for reconstitution