This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.The main function of the alpha-1 antitrypsin protein is to neutralize another protein (or enzyme) called neutrophil elastase, which is normally contained within one type of white blood corpuscle called the polymorphonuclear leukocyte, or PMN for short. Neutrophil elastase is a powerful enzyme which, upon release from the PMN, can degrade the walls of bacteria, rendering them harmless to the body. Alpha-1 antitrypsin deficiency is a hereditary disorder in which individuals have insufficienct amounts of alpha-1 antitrypsin to neutralize the neutophil elastace. This may lead to destruction of the walls of the lungs, leading to a loss of air sacs in the lung.The only therapy specifically available for these individuals to date is called augmentation therapy and involves the administration, currently by vein, of a purified form of the alpha-1 antitrypsin protein on a regular basis (e.g., usually weekly or monthly) in order to raise the levels in the bloodstream. One of these drugs, Prolastin, has been approved by the FDA for use for augmentation in these individuals since 1987. The manufacterer of Prolastin, Talecris, has developed a modified manufacturing process to increase the yield and purity of Prolastin. This new investigational drug is known as Alpha-1 MP.The objective of this clinical trial is to study the safety and tolerability of Alpha-1 MP in adult alpha-1 antitrypsin subjects as reported over 20 weeks of therapy. The primary objective and specific aim of this study is to describe the nature and frequency of treatment-emergent adverse events with 'teatment-emergent' defined as any adverse event occurring after the start of the first study drug infusion.
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