Alpha1-Proteinase Inhibitor (Human), Zemaira™, is a sterile, stable, lyophilized preparation of highly purified human alpha1-proteinase inhibitor (A1-PI), also known as alpha1-antitrypsin, derived from human plasma. Zemaira™ is manufactured from large pools of human plasma by cold ethanol fractionation according to a modified Cohn process followed by additional purification steps.
Zemaira™ is indicated for chronic augmentation and maintenance therapy in individuals with alpha1-proteinase inhibitor (A1-PI) deficiency and clinical evidence of emphysema.
Zemaira™ increases antigenic and functional (ANEC) serum levels and lung epithelial lining fluid levels of A1-PI.
Clinical data demonstrating the long-term effects of chronic augmentation therapy of individuals with Zemaira™ are not available.
Safety and effectiveness in pediatric patients have not been established.
Zemaira™ is not indicated as therapy for lung disease patients in whom severe congenital A1-PI deficiency has not been established.
Published Studies Related to Zemaira (Alpha 1-Antitrypsin)
The fibrinogen cleavage product Aalpha-Val360, a specific marker of neutrophil elastase activity in vivo. [2011.08]
BACKGROUND: Alpha-1-antitrypsin (A1AT) deficiency is the only recognised genetic risk factor for chronic obstructive pulmonary disease (COPD), a leading cause of morbidity and mortality worldwide. Since A1AT is the major inhibitor of neutrophil elastase (NE), this enzyme has become widely implicated in the pathogenesis of COPD in general; however, there is currently no specific biomarker for its pre-inhibition activity. Such a biomarker should be a measure of elastase-specific COPD disease activity with the potential to assess early targeted therapeutic intervention, in contrast to traditional and non-specific disease severity markers such as forced expiratory volume in 1 s... CONCLUSIONS: Aalpha-Val(360) represents the first specific footprint of pre-inhibition NE activity and is a potential biomarker of disease activity and progression in subjects with elastase-dependent COPD. TRIAL REGISTRATION: The EXACTLE study was registered in ClinicalTrials.gov as 'Antitrypsin (AAT) to Treat Emphysema in AAT-Deficient Patients'; ClinicalTrials.gov Identifier: NCT00263887.
Retinoid treatment of Emphysema in Patients on the Alpha-1 International Registry. The REPAIR study: study design, methodology and quality control of study assessments. [2010.12]
Emphysema is characterized by the destruction of alveolar wall and enlargement of alveolar airspaces, resulting in a reduction of the total lung gas exchange area, loss of lung elastic recoil and hyperinflation. The REPAIR study (Retinoid treatment of Emphysema in Patients on the Alpha-1 International Registry) is the first proof-of-concept study of a new potential disease-modifying drug, Palovarotene(c), an orally active, gamma selective retinoid agonist in patients with emphysema secondary to alpha-1-antitrypsin deficiency (AATD) as a model population for the general smoke-induced emphysema population.
Pharmacokinetic comparability of Prolastin(R)-C to Prolastin(R) in alpha-antitrypsin deficiency: a randomized study. [2010.09.30]
BACKGROUND: Alpha1-antitrypsin (AAT) deficiency is characterized by low blood levels of alpha1-proteinase inhibitor (alpha-PI) and may lead to emphysema. Alpha-PI protects pulmonary tissue from damage caused by the action of proteolytic enzymes. Augmentation therapy with Prolastin(R) (Alpha-Proteinase Inhibitor [Human]) to increase the levels of alpha-PI has been used to treat individuals with AAT deficiency for over 20 years. Modifications to the Prolastin manufacturing process, incorporating additional purification and pathogen-reduction steps, have led to the development of an alpha-PI product, designated Prolastin(R)-C (Alpha-Proteinase inhibitor [Human]). The pharmacokinetic comparability of Prolastin-C to Prolastin was assessed in subjects with AAT deficiency... CONCLUSION: Prolastin-C demonstrated pharmacokinetic equivalence and a comparable safety profile to Prolastin. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT00295061.
Exploring the role of CT densitometry: a randomised study of augmentation therapy in alpha1-antitrypsin deficiency. [2009.06]
Assessment of emphysema-modifying therapy is difficult, but newer outcome measures offer advantages over traditional methods. The EXAcerbations and Computed Tomography scan as Lung End-points (EXACTLE) trial explored the use of computed tomography (CT) densitometry and exacerbations for the assessment of the therapeutic effect of augmentation therapy in subjects with alpha(1)-antitrypsin (alpha(1)-AT) deficiency...
Prevalence of alpha-1 antitrypsin deficiency in poorly controlled asthma--results from the ALA-ACRC low-dose theophylline trial. [2007.10]
In a study comparing low-dose theophylline to montelukast in poorly controlled asthmatics, 285 subjects consented to be screened for alpha-1 antitrypsin deficiency. Of the 284 for which complete data was available, 10.5% carried a deficiency gene and 2.4% were mildly deficient with an alpha-1 antitrypsin serum level of less than 20 mu M...
Clinical Trials Related to Zemaira (Alpha 1-Antitrypsin)
Effect of Double Dose of Alpha 1-antitrypsin Augmentation Therapy on Lung Inflammation. [Recruiting]
The current treatment of individuals with alpha-1 antitrypsin deficiency (AATD) who develop
lung disease (COPD) is the administration of intravenous purified alpha-1 antitrypsin
(augmentation therapy) at a fixed dose of 60 mg/kg per week. This dose increases serum
levels just above a predetermined "safety threshold" of 11 uM. However, normal levels of AAT
are around 36 uM. AAT has shown not only to inhibit lung proteases such as neutrophil
elastase, but also to modulate inflammation.
Given that many subjects with AATD who receive augmentation therapy still have significant
lung disease and inflammation, this study will evaluate whether doubling the dose to 120
mg/kg/week has an effect in decreasing lung inflammation.
Only the dosing of 60 mg/kg /week has received FDA approval. FDA has granted an IND number
to this study to test the higher dose of 120 mg/kg/week.
The study will evaluate systemic (serum) and pulmonary (bronchoscopy samples)markers of
inflammation in 3 phases: standard dose (4 weeks), double dose (4 weeks) and standard dose
Safety & Efficacy Study of rAAV1-CB-hAAT for Alpha-1 Antitrypsin Deficiency [Recruiting]
This study will evaluate the safety and efficacy of a recombinant adeno-associated virus
vector expressing alpha-1 antitrypsin in patients with alpha-1 antitrypsin deficiency.
Three groups of three subjects each will receive the study drug by intramuscular injection,
with progressively larger doses in the second and third groups.
A Research Trial of Aralast in New Onset Diabetes (RETAIN) - Part II [Not yet recruiting]
The drug Alpha-1 Antitrypsin (AAT, Aralast NP), which is being tested in this clinical
trial, is an anti-inflammatory drug that affects the cells that are thought to be involved
in the development of type 1 diabetes. This trial, known as RETAIN, is a clinical trial is
a two-part trial investigating the effect of intravenous Alpha-1 Antitrypsin(AAT, Aralast
NP) on preserving beta cell function and to determine if AAT will help slow the progression
of type 1 diabetes.
Part I of this trial (NCT#) is an open-label, safety and dose level study consisting of two
groups. After Part I is completed, including a satisfactory safety review, enrollment in
Part II will begin. Part II is a two-arm, double-blind, placebo-controlled clinical trial,
and participants will be randomly assigned to either the treatment or placebo group.
The Comparison of the Pharmacokinetic, Safety and Tolerability of Alpha-1 MP and Prolastin in Adult alpha1-Antitrypsin Deficient Patients. [Completed]
The purpose of this clinical study (ChAMP - Comparability pHarmacokinetics of Alpha-1
Modified Process) is to compare the pharmacokinetic, safety and tolerability of Alpha-1
Proteinase Inhibitor (Human), modified process (Alpha-1 MP)and Prolastin in adult
Alpha1-antitrypsin deficient patients. Patients will be infused intravenously with study drug
on a weekly schedule for 24 weeks.
Safety and Pharmacokinetics of Alpha-1 Proteinase Inhibitor in Subjects With Alpha1-Antitrypsin Deficiency [Not yet recruiting]
This is a study to assess the safety and pharmacokinetics of weekly infusions of 120 mg/kg
of Alpha-1 PI, compared to weekly infusions of 60 mg/kg of Alpha-1 PI in patients with alpha
1-antitrypsin deficiency (AATD).
Reports of Suspected Zemaira (Alpha 1-Antitrypsin) Side Effects
Infusion Related Reaction (12),
Respiratory Tract Infection (6),
Chronic Obstructive Pulmonary Disease (5),
Upper Respiratory Tract Infection (5),
Sinusitis (5), more >>