Defective neutrophil chemotaxis in patients with alpha-1-antitrypsin deficiency

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.