Enzymatic Deficiency in Monocytes from Patients with Chronic Granulomatous Disease
Chronic granulomatous disease (CGD) is by convention defined as a disorder involving a high susceptibility to bacterial infection related to the inability of the patient’s neutrophils to increase oxygen consumption, despite normal phagocytosis and normal degranulationl,2. CGD appears to be heterogeneous; most cases show an inherihance pattern typical of X-linkage, but the remainder are of non X-linked variety2. CGD heterogeneity has been further suggested by the lack of membrane components in some CGD patients as kx Antigen in neutrophils or red blood cells3, and b-like cytochrome in neutrophils4,5. In two different patients with CGD it has been possible to induce the oxidative burst, with stimuli other than those used in standard analysis procedure6,7.
KeywordsOxidative Burst Chronic Granulomatous Disease Enzymatic Deficiency Acetate Ester Increase Oxygen Consumption
Unable to display preview. Download preview PDF.
- 8.N.C. Davis, H. Huber, S.D. Douglas, and H.H. Fudenberg, A defect in circulating mononuclear phagocytes in chronic granulomatous disease of childhood, J. Immuno1. 101: 1093 (1968)Google Scholar
- 10.F. Schmalzl, and H. Braunsteiner, The cytochemistry of monocytes and macrophages, Ser. Haemat. III: 93 (1970).Google Scholar
- 15.A. Boyum, Isolation of mononuclear cells and granulocytes from human blood, Scand. J. Clin. Lab. Invest. 21 (Supp1.97): 77 (1968).Google Scholar
- 16.L. Ornstein, H. Awsley, and A. Saunders, Improving mammal differential white cell counts with cytochemistry, Blood cells 2: 557 (1976).Google Scholar
- 22.G. Flandrin, and M.T. Daniel, Practical value of cytochemical studies for the classification of acute leukemias, in: “Nomenclature,Methodology and Results of Clinical Trials in Acute Leukemias”, G. Mathe, P. Pouillart, and L. Schwarzenberg, eds., Heinemann, London (1973).Google Scholar