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Phase 4 N=13 Treatment

Efficacy and Safety Study of Augmentation Therapy With ARALAST Fraction IV-1 (Human Alpha 1 - Proteinase Inhibitor)

Alpha 1-Antitrypsin Deficiency

Enrolled (actual)
13
Serious AEs
7.7%
Results posted
Jan 2011
Primary outcome: Primary: Change in Bronchoalveolar Lavage (BAL) Epithelial Lining Fluid (ELF) Alpha1-Proteinase Inhibitor (α1-PI) Level — 0.28 μM — p=0.0195

Study Design & Population

Study type
Interventional
Phase
Phase 4
Interventions
Alpha1-Proteinase Inhibitor (Biological)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Baxalta now part of Shire
Primary completion
Dec 2007

Outcome Measures

OutcomeResultp-value
PRIMARY
Change in Bronchoalveolar Lavage (BAL) Epithelial Lining Fluid (ELF) Alpha1-Proteinase Inhibitor (α1-PI) Level
0.28 0.0195 sig
PRIMARY
The Number of Adverse Events (AEs) Related to the Infusion of ARALAST Fr. IV 1 Administered at a Rate of 0.2 mL/kg/Min
PRIMARY
Number of Changes in the Rate of Infusion
SECONDARY
Ratio of Post- to Pre-treatment BAL ELF Antineutrophil Elastase Capacity (ANEC) Levels
1.22
SECONDARY
Change in in the Ratio of BAL ELF α1-PI to Human Neutrophil Elastase (HNE) Complex Concentration
SECONDARY
Change in the α1-PI Plasma Level
10.34 <0.0001 sig
SECONDARY
Change in the Plasma Antineutrophil Elastase Capacity (ANEC) Level
9.46 <0.0001 sig
SECONDARY
Clinically Significant Changes in Vital Signs From Pre- to Post-Infusion
2

Summary

The purpose of this study is to evaluate the effects of weekly augmentation therapy with ARALAST Fraction IV-1 (Fr IV-1) on epithelial lining fluid (ELF) alpha 1-proteinase inhibitor levels and other ELF analytes and to assess the safety of the treatment. Eligible subjects with a diagnosis of severe congenital alpha 1-antitrypsin deficiency will receive 8 consecutive weekly treatments with 60 mg/kg/week of functional ARALAST Fr IV-1 administered intravenously. The efficacy and safety assessments will include two bronchoscopies with bronchoalveolar lavage on study initiation and on study termination and multiple imaging and laboratory safety assessments. Each subject will participate for a minimum of 12 weeks.

Eligibility Criteria

Inclusion Criteria

  • Signed and dated informed consent.
  • Male or female 18 years of age or older.
  • Documented, endogenous serum α1-PI level = 50% of predicted value; or
  • FEV1 > 35% of predicted value and diffusing capacity for carbon monoxide > 45% of predicated value, with no supplemental oxygen therapy and = 1500 cells/mm3
  • Hemoglobin (Hgb) >= 10.0 g/dL
  • Platelet count >= 105/mm3
  • If the subject is treated with respiratory medications, such as inhaled bronchodilators or inhaled corticosteroids, or other chronic medications for the treatment of the subjects´s other medical condition(s), the subject's medication doses were unchanged for at least 14 days prior to the baseline BAL visit.

Exclusion Criteria

  • Clinically significant pulmonary impairment, other than chronic pulmonary disease (COPD).
  • The subject has received any alpha 1 proteinase inhibitor (α1-PI) augmentation therapy (e.g., Prolastin, Zemaira, Aralast, or an investigational α1-PI, by any route including intravenous and inhaled) within 28 days prior to screening.
  • The subject has received an investigational drug or device within 1 month prior to screening, or the subject is currently receiving an investigational drug or device. If the subject receives another investigational drug or device after enrollment, the subjects is to be withdrawn from the trial.
  • Presence of clinical symptoms of any lower respiratory tract infection or acute pulmonary exacerbation within 14 days prior to screening.
  • The subject has a known selective Immunoglobulin A (IgA) deficiency (IgA level less than 15 mg/dL) and/or antibody against IgA.
  • The subject is pregnant or lactating, or intends to become pregnant during the course of the study.
  • The subject is not a suitable candidate for a BAL procedure.
  • Moderate or severe bronchiectasis (total daily sputum production > 10 mL).
  • 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.
  • Prior history of adverse reaction to local anaesthetics, sedatives, pain control drugs, and other medication employed at the study center for perioperative care associated with the BAL procedure.
  • Long-term use of oral or parenteral glucocorticosteroid within 28 days prior to screening.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00396006). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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