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