Study of the Effect of Aerosolized, Recombinant Alpha 1-Antitrypsin on Epithelial Lining Fluid Analytes in Subjects With Alpha 1-Antitrypsin Deficiency

Inclusion Criteria: Signed Informed Consent Male or female 18 years of age or older Documented, endogenous plasma AAT levels < 11 µM (< 80 mg/dL), either obtained from the medical history or, if not documented, plasma AAT measured after 28 day washout of any prior replacement therapy Forced expiratory volume at 1 second (FEV1) that is >= 50% of predicted, measured 30 minutes after a short-acting inhaled bronchodilator Arterial oxygen percent saturation (SaO2, measured using room air) within the normal limits for the individual study site For subjects receiving an inhaled corticosteroid, inhaled or oral β-2 agonist (e.g., albuterol via metered dose inhaler [MDI]) or inhaled anticholinergic bronchodilator (e.g., ipratropium bromide), or oral PDE (phosphodiesterase) inhibitor, treatment on a stable dose for at least 14 days prior to enrollment For any female of childbearing potential, a negative urine test for pregnancy within 3 days prior to enrollment and agreement to employ adequate birth control measures for the duration of the study No clinically significant abnormalities detected on a 12-lead electrocardiogram (ECG) performed at the screening visit Laboratory results obtained at the screening visit, meeting the following criteria: Serum aspartate transaminase (AST) and alanine transaminase (ALT) <= 2 times upper limit of normal range (ULN) Serum total bilirubin <= 2 times ULN < 2+ proteinuria on urine dipstick Serum creatinine <= 1.5 times ULN Absolute neutrophil count >= 1500 cells/mm3 Hemoglobin >= 10.0 g/dL Platelet count >= 100,000/mm3 Exclusion Criteria: Clinically significant pulmonary impairment, other than emphysema and/or chronic bronchitis Moderate to severe bronchiectasis Clinically significant cardiac, hemostatic, or neurologic impairment, or other significant medical condition that, in the opinion of the investigator, would affect subject safety or compliance Psychiatric or cognitive disturbance or illness, or recreational drug/alcohol use that, in the opinion of the investigator, would affect subject safety or compliance Acute exacerbation of emphysema within 28 days prior to the screening visit Pregnancy or lactation Known history of allergy to yeast products Medical history precluding the use of epinephrine or other rescue medication for treatment of anaphylaxis Prior history of adverse reactions to the local anesthetic, sedative, BAL procedure, or pre-medication employed at the study center Use of oral or parenteral glucocorticosteroids, or alpha 1-antitrypsin replacement therapy within 28 days prior to baseline BAL, or any use planned during the study. However, the subject may enroll provided that a) consent is given to undergo a 28-day washout of the replacement or steroid therapy, and b) no study procedures are done until the washout is completed. Use of another investigational drug or investigational device within 28 days prior to baseline BAL Any upper or lower respiratory infection within 28 days prior to baseline BAL Having received a lung or liver transplant