European Journal of Pediatrics

, Volume 144, Issue 5, pp 464–466 | Cite as

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

  • A. J. Khan
  • H. E. Evans
  • M. M. Agbayani
Original Investigations

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 deficiency 

Abbreviations

AAT

alpha-1-antitrypsin

HBS

Hank's balanced salt solution

CI

chemotactic index

RM

random migration

CFI

chemotactic factor inactivator

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References

  1. 1.
    Erlanger BF, Kakowsky N, Cohen W (1961) The preparation and properties of two new chromogenic substrate of trypsin. Arch Biochem Biophys 95:271–278Google Scholar
  2. 2.
    Fagerhol MK, Laurell CB (1967) The polymorphism in “prealbumins” and alpha 1-antitrypsin in human sera. Clin Chem Acta 16:199–202Google Scholar
  3. 3.
    Khan AJ, Evans HE, Glass L, Shin YH, Almonte D (1979) Defective neutrophil chemotaxis in patients with Down's syndrome. J Pediatr 87:87–89Google Scholar
  4. 4.
    Khan AJ, Evans HE, Glass L, Khan P, Chang CT, Nair SR (1979) Abnormal neutrophil chemotaxis and random migration induced by aminoglycoside antibiotics. J Lab Clin Med 93:295–300Google Scholar
  5. 5.
    Maderazo EG, Ward PA, Quintiliani R (1975) Defective regulation of chemotaxis in cirrhosis. J Lab Clin Med 85:621–625Google Scholar
  6. 6.
    Morse JO (1978) Alpha 1-antitrypsin deficiency. New Engl J Med 299:1045–1048Google Scholar
  7. 7.
    Morse JO (1978) Alpha 1-antitrypin deficiency. New Engl J Med 299:1099–1105Google Scholar
  8. 8.
    Prévost C, Frommel D, Duphy J-M (1975) Complement studies in alpha-1 antitrypsin deficiency in children. J Pediatr 87:571–573Google Scholar
  9. 9.
    Sveger T (1978) Alpha 1-antitrypsin deficiency in early childhood. Pediatrics 62:22–25Google Scholar
  10. 10.
    Ward PA, Becker EL (1968) The deactivation of rabbit neutrophils by chemotactic factor and the nature of activable esterase. J Exp Med 127:693–696Google Scholar
  11. 11.
    Ward PA, Talamo RC (1973) Deficiency of the chemotactic factor inactivator in human sera with alpha 1-antitrypsin deficiency. J Clin Invest 52:516–519Google Scholar

Copyright information

© Springer-Verlag 1986

Authors and Affiliations

  • A. J. Khan
    • 1
  • H. E. Evans
    • 1
  • M. M. Agbayani
    • 1
  1. 1.Department of Pediatrics of Interfaith Medical CenterSuny/Downstate Medical CenterBrooklynUSA

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