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Defective neutrophil chemotaxis in patients with alpha-1-antitrypsin deficiency
Authors:A. J. Khan  H. E. Evans  M. M. Agbayani
Affiliation:(1) Department of Pediatrics of Interfaith Medical Center, Suny/Downstate Medical Center, 11213 Brooklyn, NY, USA
Abstract:The PMNs of five infants and children with homozygous alpha-1-antitrypsin deficiency (PiZZ phenotype) revealed defective chemotactic migration, and their PiZZ sera generated a higher quantity of chemotactic factor(s).This defect(s) might have been responsible for the increased incidence of infections found in our deficient PiZZ patients. Further studies are needed to clarify the role of defective chemotaxis in the pathogenesis of chronic lung disease associated with the PiZZ state.Abbreviations AAT alpha-1-antitrypsin - HBS Hank's balanced salt solution - CI chemotactic index - RM random migration - CFI chemotactic factor inactivator
Keywords:Chemotaxis  Random migration  Chemotactic factors  Alpha-1-antitrypsin deficiency
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