Defective neutrophil chemotaxis in patients with alpha-1-antitrypsin deficiency
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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.
Key words
Chemotaxis Random migration Chemotactic factors Alpha-1-antitrypsin deficiencyAbbreviations
- AAT
alpha-1-antitrypsin
- HBS
Hank's balanced salt solution
- CI
chemotactic index
- RM
random migration
- CFI
chemotactic factor inactivator
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© Springer-Verlag 1986