Short term effects of alpha1-antitrypsin substitution therapy on the degranulation of neutrophil granulocytes

Introduction: Alpha-1-antitrypsin-deficiency (AATD) is a hereditary, co-dominant condition which results in low levels of circulating alpha-1-antitrypsin (A1AT), unbalanced protease activity and frequent development of emphysema. The concept behind substitution therapy with human plasma A1AT is to normalize the concentration in blood and tissue and thereby protecting the lungs from progressive destruction by proteases. Aims: To better understand the influence of weekly augmentation therapy on polymorphnuclear neutrophils (PMNs) function we examined direct effects on chemokine release and degranulation of matrix…