ALPHA-1 PROTEINASES INHIBITOR TO DELAY THE BEGINNING OR PROGRESSION OF PULMONARY EXCESSIONS

摘要

1. A method of delaying the onset or reducing the progression of one or more symptoms associated with pulmonary exacerbations in a subject in need thereof, comprising administering to the subject an effective amount of A1PI by inhalation. 2. The method according to claim 1, wherein the A1PI is aerosolized. 3. The method according to claim 1, wherein the A1PI is administered using an inhaler. 4. The method according to claim 1, wherein the A1PI is administered at least once a day. The method according to claim 1, wherein the effective amount of A1PI is from about 25 mg to about 750 mg of A1PI per day. The method according to claim 1, wherein the effective amount of A1PI is from about 0.5 mg / kg / day to about 15 mg / kg / day. The method according to claim 1, wherein the age of the subject who needs it is at least 12 years. The method according to claim 1, wherein exacerbations of pulmonary diseases are associated with fibrocystic degeneration, COPD, AATD (A1PI deficiency), emphysema, asthma, mycobacterial infection, pneumonia, bronchiectasis, or chronic bronchitis. 9. The method in accordance with paragraph 1, where pulmonary exacerbations are associated with increased severity of exacerbations; increased frequency of exacerbations; decreased lung function; reduced levels of FEV; increased loss of lung tissue; reduced A1PI levels; elevated levels of pulmonary elastase; frequent pulmonary infections; increased bacterial load of the lungs or increased cardiac arrhythmia. 10. The method according to claim 1, wherein the A1PI is a recombinant A1PI. 11. The method according to claim 1, wherein the A1PI is purified using a method for purification of A1PI from an aqueous solution