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1-Antitrypsin Deficiency
1 Department of Pediatrics, Division of Pulmonary Diseases, University of Florida College of Medicine, Gainesville, FL, USA
2 Clinical Studies Section, Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, Bethesda, MD, USA
3 Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Florida College of Medicine, Gainesville, FL, USA
* To whom correspondence should be addressed. E-mail: spenclt{at}peds.ufl.edu.
Individuals with 
1-antitrypsin deficiency are at risk for early onset destructive lung disease as a result of insufficient lower respiratory tract 1-antitrypsin and an increased burden of neutrophil
products such as elastase. Human neutrophil peptides, the most abundant protein component of neutrophil azurophilic granules, represent another potential inflammatory component in lung disease characterized by increased numbers of activated or deteriorating neutrophils. The
purpose of this study was to determine the role of human neutrophil peptides in lower respiratory tract inflammation and destruction in 1-antitrypsin deficient individuals. 33 1-antitrypsin deficient patients and 21 healthy control subjects were evaluated by bronchoalveolar lavage. Human neutrophil peptide concentrations were significantly higher in 1-antitrypsin deficient individuals (1976 ± 692 nM and 29 ± 12 nM, respectively, p < 0.0001), and levels correlated with markers of neutrophil-mediated lung inflammation. In vitro, human neutrophil
peptides produced a dose-dependent cytotoxic effect on alveolar macrophages, and stimulated production of the potent neutrophil chemoattractants leukotriene B4 and interleukin-8 by alveolar macrophages, with a 6 to 10-fold increase in chemoattractant production over negative control
cultures (p < 0.05). A synergistic effect was noted between human neutrophil peptides and neutrophil elastase with regard to leukotriene B4 production. Importantly, the pro-inflammatory effects of human neutrophil peptides were blocked by 1-antitrypsin. Human neutrophil peptides likely play an important role in amplifying and maintaining neutrophil-mediated inflammation in the lungs.
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