Skip to main content
Clinical and Experimental Immunology logoLink to Clinical and Experimental Immunology
. 1982 Nov;50(2):454–460.

Defective immune interferon production and natural killer activity associated with poor neutrophil mobility and delayed umbilical cord separation.

E G Davies, D Isaacs, R J Levinsky
PMCID: PMC1536700  PMID: 6817956

Abstract

Two infants with recurrent infections and a history of delay in separation of the umbilical cord (1 month and 17 days) had severely impaired neutrophil mobility. In addition very poor natural killer cell (NK) activity of blood lymphocytes against a leukaemia cell line (Molt 4F) was found. Incubation of lymphocytes with lymphoblastoid interferon increased NK activity in the one case tested. No immune (gamma) interferon production was detected in Raji cell and phytohaemagglutinin (PHA) stimulated cultures from the other case. Apart from an abnormal dose-response curve in thymidine uptake after PHA stimulation of blood lymphocytes, no other abnormalities were found in a range of immunological tests. Ascorbic acid improved neutrophil mobility but had no effect, on NK activity. Both children have subsequently died from septicaemic illnesses.

Full text

PDF

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aggett P. J., Harries J. T., Harvey B. A., Soothill J. F. An inherited defect of neutrophil mobility in Shwachman syndrome. J Pediatr. 1979 Mar;94(3):391–394. doi: 10.1016/s0022-3476(79)80578-8. [DOI] [PubMed] [Google Scholar]
  2. Allen P. T., Giron D. J. Rapid sensitive assay for interferons based on the inhibition of MM virus nucleic acid synthesis. Appl Microbiol. 1970 Sep;20(3):317–322. doi: 10.1128/am.20.3.317-322.1970. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Bowen T., Ochs H. D., Wedgwood R. J. Chemotaxis and umbilical separation. Lancet. 1979 Aug 11;2(8137):302–302. doi: 10.1016/s0140-6736(79)90311-8. [DOI] [PubMed] [Google Scholar]
  4. Earhoudi A., Harvey B. A., Soothill J. F. Clinicopathological findings in patients with primary and secondary defects of neutrophil mobility. Arch Dis Child. 1978 Aug;53(8):625–630. doi: 10.1136/adc.53.8.625. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Epstein L. B., Gupta S. Human T-lymphocyte subset production of immune (gamma) interferon. J Clin Immunol. 1981 Jul;1(3):186–194. doi: 10.1007/BF00922762. [DOI] [PubMed] [Google Scholar]
  6. Haliotis T., Roder J., Klein M., Ortaldo J., Fauci A. S., Herberman R. B. Chédiak-Higashi gene in humans I. Impairment of natural-killer function. J Exp Med. 1980 May 1;151(5):1039–1048. doi: 10.1084/jem.151.5.1039. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Hayward A. R., Harvey B. A., Leonard J., Greenwood M. C., Wood C. B., Soothill J. F. Delayed separation of the umbilical cord, widespread infections, and defective neutrophil mobility. Lancet. 1979 May 26;1(8126):1099–1101. doi: 10.1016/s0140-6736(79)91786-0. [DOI] [PubMed] [Google Scholar]
  8. Herberman R. B., Ortaldo J. R. Natural killer cells: their roles in defenses against disease. Science. 1981 Oct 2;214(4516):24–30. doi: 10.1126/science.7025208. [DOI] [PubMed] [Google Scholar]
  9. Isaacs D., Clarke J. R., Tyrrell D. A., Webster A. D., Valman H. B. Deficient production of leucocyte interferon (interferon-alpha) in vitro and in vivo in children with recurrent respiratory tract infections. Lancet. 1981 Oct 31;2(8253):950–952. doi: 10.1016/s0140-6736(81)91153-3. [DOI] [PubMed] [Google Scholar]
  10. Koren H. S., Amos D. B., Buckley R. H. Natural killing in immunodeficient patients. J Immunol. 1978 Mar;120(3):796–799. [PubMed] [Google Scholar]
  11. Lipinski M., Virelizier J. L., Tursz T., Griscelli C. Natural killer and killer cell activities in patients with primary immunodeficiencies or defects in immune interferon production. Eur J Immunol. 1980 Apr;10(4):246–249. doi: 10.1002/eji.1830100405. [DOI] [PubMed] [Google Scholar]
  12. Perussia B., Mangoni L., Engers H. D., Trinchieri G. Interferon production by human and murine lymphocytes in response to alloantigens. J Immunol. 1980 Oct;125(4):1589–1595. [PubMed] [Google Scholar]
  13. Roder J. C., Haliotis T., Klein M., Korec S., Jett J. R., Ortaldo J., Heberman R. B., Katz P., Fauci A. S. A new immunodeficiency disorder in humans involving NK cells. Nature. 1980 Apr 10;284(5756):553–555. doi: 10.1038/284553a0. [DOI] [PubMed] [Google Scholar]
  14. Sirianni M. C., Fiorilli M., Pandolfi F., Quinti I., Aiuti F. Natural killer activity and lymphocyte subpopulations in patients with primary humoral and cellular immunodeficiencies. Clin Immunol Immunopathol. 1981 Oct;21(1):12–19. doi: 10.1016/0090-1229(81)90190-2. [DOI] [PubMed] [Google Scholar]
  15. Trompeter R. S., Layward L., Hayward A. R. Primary and secondary abnormalities of T cell subpopulations. Clin Exp Immunol. 1978 Dec;34(3):388–392. [PMC free article] [PubMed] [Google Scholar]

Articles from Clinical and Experimental Immunology are provided here courtesy of British Society for Immunology

RESOURCES