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. 1968 Aug;47(8):1753–1762. doi: 10.1172/JCI105865

Leukocyte degranulation and vacuole formation in patients with chronic granulomatous disease of childhood

Emanuel Kauder 1, Louis L Kahle 1, Hernan Moreno 1, John C Partin 1
PMCID: PMC297335  PMID: 5666110

Abstract

A cellular defect associated with decreased bactericidal activity of the polymorphonuclear leukocyte has been found in a 2½ yr old Negro boy with the typical clinical and pathological findings of chronic granulomatous disease. Unlike previously described patients his polymorphonuclear leukocytes were shown to undergo apparently normal degranulation and vacuole formation after phagocytosis. Metabolic studies of the leukocytes indicated a failure to increase oxygen consumption with phagocytosis or to reduce Nitroblue tetrazolium dye. These metabolic abnormalities are identical with those previously reported in patients with chronic granulomatous disease. Two additional patients with chronic granulomatous disease have also been found to have apparently adequate degranulation of polymorphonuclear leukocytes after phagocytosis. Our studies suggest that failure of degranulation may not be a necessary part of this functional leukocyte abnormality.

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Selected References

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  1. ACKERMAN G. A. Substituted naphthol AS phosphate derivatives for the localization of leukocyte alkaline phosphatase activity. Lab Invest. 1962 Jul;11:563–567. [PubMed] [Google Scholar]
  2. Baehner R. L., Nathan D. G. Leukocyte oxidase: defective activity in chronic granulomatous disease. Science. 1967 Feb 17;155(3764):835–836. doi: 10.1126/science.155.3764.835. [DOI] [PubMed] [Google Scholar]
  3. COHN Z. A., MORSE S. I. Interactions between rabbit polymorphonuclear leucocytes and staphylococci. J Exp Med. 1959 Sep 1;110:419–443. doi: 10.1084/jem.110.3.419. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Holmes B., Page A. R., Good R. A. Studies of the metabolic activity of leukocytes from patients with a genetic abnormality of phagocytic function. J Clin Invest. 1967 Sep;46(9):1422–1432. doi: 10.1172/JCI105634. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Holmes B., Quie P. G., Windhorst D. B., Good R. A. Fatal granulomatous disease of childhood. An inborn abnormality of phagocytic function. Lancet. 1966 Jun 4;1(7449):1225–1228. doi: 10.1016/s0140-6736(66)90238-8. [DOI] [PubMed] [Google Scholar]
  6. Quie P. G., White J. G., Holmes B., Good R. A. In vitro bactericidal capacity of human polymorphonuclear leukocytes: diminished activity in chronic granulomatous disease of childhood. J Clin Invest. 1967 Apr;46(4):668–679. doi: 10.1172/JCI105568. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. REBUCK J. W., CROWLEY J. H. A method of studying leukocytic functions in vivo. Ann N Y Acad Sci. 1955 Mar 24;59(5):757–805. doi: 10.1111/j.1749-6632.1955.tb45983.x. [DOI] [PubMed] [Google Scholar]
  8. SEVERINGHAUS J. W. Oxyhemoglobin dissociation curve correction for temperature and pH variation in human blood. J Appl Physiol. 1958 May;12(3):485–486. doi: 10.1152/jappl.1958.12.3.485. [DOI] [PubMed] [Google Scholar]
  9. SHINEFIELD H. R., RIBBLE J. C., BORIS M., EICHENWALD H. F. Bacterial interference: its effect on nursery-acquired infection with Staphylococcus aureus. I. Preliminary observations on artificial colonzation of newborns. Am J Dis Child. 1963 Jun;105:646–654. [PubMed] [Google Scholar]
  10. SKARNES R. C., WATSON D. W. Characterization of leukin: an antibacterial factor from leucocytes active against gram-positive pathogens. J Exp Med. 1956 Dec 1;104(6):829–845. doi: 10.1084/jem.104.6.829. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Windhorst D. B., Holmes B., Good R. A. A newly defined X-linked trait in man with demonstration of the Lyon effect in carrier females. Lancet. 1967 Apr 8;1(7493):737–739. doi: 10.1016/s0140-6736(67)91360-8. [DOI] [PubMed] [Google Scholar]
  12. ZATTI M., ROSSI F., MENEGHELLI V. METABOLIC AND MORPHOLOGICAL CHANGES OF POLYMORPHONUCLEAR LEUCOCYTES DURING PHAGOCYTOSIS. Br J Exp Pathol. 1965 Apr;46:227–233. [PMC free article] [PubMed] [Google Scholar]
  13. Zeya H. I., Spitznagel J. K. Cationic proteins of polymorphonuclear leukocyte lysosomes. II. Composition, properties, and mechanism of antibacterial action. J Bacteriol. 1966 Feb;91(2):755–762. doi: 10.1128/jb.91.2.755-762.1966. [DOI] [PMC free article] [PubMed] [Google Scholar]

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