Pharmacokinetic Study of ARALAST (Human Alpha1- PI)

Inclusion criteria

• The subject or subject´s legally authorized representative has provided written informed consent
• Subject is 18 years of age or older
• Subject has a documented, endogenous plasma Alpha1-PI level < 8 Micromolar
• Subject is of the genotype Pi*Z/Z, Pi*Z/Null, Pi*Null/Null, Pi*Malton/Z, or others, dependent on the approval by the Sponsor
• If the subject is female or of childbearing potential, the subject has a negative urine test for pregnancy within 7 days prior to first study product administration and agrees to employ adequate birth control measures for the duration of the study
• Laboratory results obtained at the screening visit, meeting the following criteria:

• Serum alanine aminotransferase (ALT), aspartate aminotransferase (AST) <= 2 times the upper limit of normal (ULN)
• Serum total bilirubin <= 2 times ULN
• Proteinuria < +2 on dipstick analysis
• Serum creatinine <= 1.5 times ULN
• Absolute neutrophil count (ANC) >= 1500 cells/mm3
• Hemoglobin >= 10.0 g/dL
• Platelet count >= 10^5/mm3
• If the subject is treated with any respiratory medications, including inhaled bronchodilators and inhaled or oral corticosteroids, the subjects´ medication doses were unchanged for at least 14 days prior to first study product administration
• Nonsmoker for a minimum of 3 months prior to first study product administration

Exclusion criteria

• The subject has received any Alpha1-PI augmentation therapy (including Aralast and investigational Alpha1-PIs, by any route including intravenous and inhaled) within 42 days prior to first study product administration
• The subject has received an investigational drug or device within 1 month prior to first study product administration, or the subject is currently receiving an investigational drug
• The subject has a known selective immunoglobulin A (IgA) deficiency (IgA level < 15 mg/dL) and/or antibody to IgA
• The subject has a pulmonary exacerbation or had a pulmonary exacerbation in the past 14 days prior to first study product administration
• The subject is pregnant or lactating, or intends to become pregnant during the course of the study
• The subject has a clinically significant medical, psychiatric, or cognitive illness, or recreational drug/alcohol use that, in the opinion of the investigator, would affect subject safety or compliance