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. 1970 Feb;45(239):24–32. doi: 10.1136/adc.45.239.24

Chronic Granulomatous Disease: Quantitative Clinicopathological Relationships

Eileen N Thompson, J F Soothill
PMCID: PMC2020392  PMID: 4191616

Abstract

10 children with chronic granulomatous disease are described. Though the clinical features were typical, a wide range of clinical severity was noted. A significant correlation between the severity of the syndrome and the qualitative nitro-blue tetrazolium results was found.

Facial rashes (4) and polyarthritis (1) were noted in female presumed heterozygotes, the incidence of which was also related to the nitro-blue tetrazolium test results. A father and a paternal great uncle died of leukaemia. The possible aetiological relation between these and the partial leucocyte abnormality is discussed.

Three children were treated with busulphan, in order to induce neutropenia, to expose the ingested organisms to humoral bactericidal mechanisms, and to antibiotics. There was evidence suggestive of benefit in one of them.

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Selected References

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

  1. BERENDES H., BRIDGES R. A., GOOD R. A. A fatal granulomatosus of childhood: the clinical study of a new syndrome. Minn Med. 1957 May;40(5):309–312. [PubMed] [Google Scholar]
  2. BRIDGES R. A., BERENDES H., GOOD R. A. A fatal granulomatous disease of childhood; the clinical, pathological, and laboratory features of a new syndrome. AMA J Dis Child. 1959 Apr;97(4):387–408. [PubMed] [Google Scholar]
  3. 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]
  4. CARSON M. J., CHADWICK D. L., BRUBAKER C. A., CLELAND R. S., LANDING B. H. THIRTEEN BOYS WITH PROGRESSIVE SEPTIC GRANULOMATOSIS. Pediatrics. 1965 Mar;35:405–412. [PubMed] [Google Scholar]
  5. Chandra R. K., Cope W. A., Soothill J. F. Chronic granulomatous disease. Lancet. 1969 Jul 12;2(7611):71–74. doi: 10.1016/s0140-6736(69)92388-5. [DOI] [PubMed] [Google Scholar]
  6. Evans P. Disseminated Granulomata. Proc R Soc Med. 1962 Nov;55(11):982–983. [PMC free article] [PubMed] [Google Scholar]
  7. 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]
  8. Johnston R. B., Jr, McMurry J. S. Chronic familial granulomatosis. Report of five cases and review of the literature. Am J Dis Child. 1967 Oct;114(4):370–378. doi: 10.1001/archpedi.1967.02090250068002. [DOI] [PubMed] [Google Scholar]
  9. LANDING B. H., SHIRKEY H. S. A syndrome of recurrent infection and infiltration of viscera by pigmented lipid histiocytes. Pediatrics. 1957 Sep;20(3):431–438. [PubMed] [Google Scholar]
  10. Macfarlane P. S., Speirs A. L., Sommerville R. G. Fatal granulomatous disease of childhood and benign lymphocytic infiltration of the skin (congenital dysphagocytosis). Lancet. 1967 Feb 25;1(7487):408–410. doi: 10.1016/s0140-6736(67)91174-9. [DOI] [PubMed] [Google Scholar]
  11. Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
  12. Quie P. G., Kaplan E. L., Page A. R., Gruskay F. L., Malawista S. E. Defective polymorphonuclear-leukocyte function and chronic granulomatous disease in two female children. N Engl J Med. 1968 May 2;278(18):976–980. doi: 10.1056/NEJM196805022781802. [DOI] [PubMed] [Google Scholar]
  13. 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]
  14. Thompson E. N., Cope W. A., Chandra R. K., Soothill J. F. Leucocyte abnormality in both parents of a patient with chronic granulomatous disease. Lancet. 1969 Apr 19;1(7599):799–800. doi: 10.1016/s0140-6736(69)92065-0. [DOI] [PubMed] [Google Scholar]
  15. 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]
  16. Windhorst D. B., Page A. R., Holmes B., Quie P. G., Good R. A. The pattern of genetic transmission of the leukocyte defect in fatal granulomatous disease of childhood. J Clin Invest. 1968 May;47(5):1026–1034. doi: 10.1172/JCI105792. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Zeya H. I., Spitznagel J. K. Arginine-rich proteins of polymorphonuclear leukocyte lysosomes. Antimicrobial specificity and biochemical heterogeneity. J Exp Med. 1968 May 1;127(5):927–941. doi: 10.1084/jem.127.5.927. [DOI] [PMC free article] [PubMed] [Google Scholar]

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