scholarly journals The biochemical basis of nitroblue tetrazolium reduction in normal human and chronic granulomatous disease polymorphonuclear leukocytes

Blood ◽  
1976 ◽  
Vol 48 (2) ◽  
pp. 309-313 ◽  
Author(s):  
RL Baehner ◽  
LA Boxer ◽  
J Davis
Keyword(s):  
Chronic Granulomatous Disease ◽  
Polymorphonuclear Leukocytes ◽  
Nitroblue Tetrazolium ◽  
Granulomatous Disease ◽  
Biochemical Basis ◽  
Nitroblue Tetrazolium Reduction ◽  
Normal Human ◽  
Nbt Reduction ◽  
Human Polymorphonuclear Leukocytes ◽  
Tetrazolium Reduction

Normal human polymorphonuclear leukocytes (PMN) placed in anaerobic chambers reaching pO2's of less than 5 mm Hg fail to generate O2-, iodinate ingested particles, and stimulate glucose-1–14C oxidation through the hexose monophosphate shunt. The observation that anaerobic cells are incapable of generating O2- or reducing nitroblue tetrazolium (NBT) to formazan supports the idea that NBT reduction in phagocytizing PMN is due exclusively to oxygen-dependent O2- generating oxidase which is deficient in chronic granulomatous disease leukocytes, despite their hyperphagocytic capacity.

scholarly journals The biochemical basis of nitroblue tetrazolium reduction in normal human and chronic granulomatous disease polymorphonuclear leukocytes

Blood ◽  
1976 ◽  
Vol 48 (2) ◽  
pp. 309-313 ◽  
Author(s):  
RL Baehner ◽  
LA Boxer ◽  
J Davis
Keyword(s):  
Chronic Granulomatous Disease ◽  
Polymorphonuclear Leukocytes ◽  
Nitroblue Tetrazolium ◽  
Granulomatous Disease ◽  
Biochemical Basis ◽  
Nitroblue Tetrazolium Reduction ◽  
Normal Human ◽  
Nbt Reduction ◽  
Human Polymorphonuclear Leukocytes ◽  
Tetrazolium Reduction

Abstract Normal human polymorphonuclear leukocytes (PMN) placed in anaerobic chambers reaching pO2's of less than 5 mm Hg fail to generate O2-, iodinate ingested particles, and stimulate glucose-1–14C oxidation through the hexose monophosphate shunt. The observation that anaerobic cells are incapable of generating O2- or reducing nitroblue tetrazolium (NBT) to formazan supports the idea that NBT reduction in phagocytizing PMN is due exclusively to oxygen-dependent O2- generating oxidase which is deficient in chronic granulomatous disease leukocytes, despite their hyperphagocytic capacity.

BACTERICIDAL FUNCTION OF HUMAN POLYMORPHONUCLEAR LEUKOCYTES

PEDIATRICS ◽  
1972 ◽  
Vol 50 (2) ◽  
pp. 264-270
Author(s):  
Paul G. Quie
Keyword(s):  
Hydrogen Peroxide ◽  
Chronic Granulomatous Disease ◽  
Polymorphonuclear Leukocytes ◽  
Bacterial Species ◽  
Bacterial Flora ◽  
Granulomatous Disease ◽  
Serum Factors ◽  
Gram Negative ◽  
Increased Risk ◽  
Human Polymorphonuclear Leukocytes

Serum from most normal persons contains specific antibodies which react with common bacterial species preparing their surfaces so that phagocytosis by leukocytes can take place. The Fab part of these antibodies reacts with immunologic specificity with antigens on the surface of bacteria. Another part of the immunoglobulin molecule termed the Fc portion is activated during the attachment of the Fab portion to bacteria and becomes a site for attachment of bacteria to receptors on the surface of phagocytic cells. This activity is greatly amplified by heat-labile serum factors. Normally bacteria are rapidly killed by human polymorphonuclear leukocytes after engulfment occurs. However staphylococci and gram-negative species of bacteria survive in the leukocytes of patients with the syndrome "Chronic Granulomatous Disease of Childhood." These patients have suffered recurrent severe infections with bacterial species that are part of the body's resident bacterial flora. By contrast these patients are not at increased risk to infection from such pyogenic bacterial species as group A streptococci or pneumococci. The leukocytes from patients with chronic granulomatous disease produce little hydrogen peroxide during phagocytosis. Catalase-producing staphylococci and gram-negative bacteria are not killed, but hydrogen peroxide-producing streptococci and pneumococci are killed. A normal metabolic response to phagocytosis as well as release of lysosonial factors are essential for the bactericidal activity of human polymorphonuclear leukocytes.

scholarly journals Kx: its relationship to chronic granulomatous disease and genetic linkage with Xg

Blood ◽  
1981 ◽  
Vol 58 (1) ◽  
pp. 34-37
Author(s):  
P Densen ◽  
S Wilkinson-Kroovand ◽  
GL Mandell ◽  
G Sullivan ◽  
R Oyen ◽  
...  
Keyword(s):  
Chronic Granulomatous Disease ◽  
Clinical History ◽  
Good Health ◽  
Neutrophil Function ◽  
Granulomatous Disease ◽  
Gene Coding ◽  
Nitroblue Tetrazolium Reduction ◽  
Erythrocyte Surface ◽  
The Relationship ◽  
Tetrazolium Reduction

The relationship between neutrophil function and the neutrophil antigen, Kx, as well as the linkage of the gene, Xk, with Xg was examined in a kindred with X-linked chronic granulomatous disease. Four of the eight male siblings had chronic granulomatous disease by clinical history and tests of neutrophil function, and all four had Kx- negative neutrophils. The remaining four were in good health and had normal nitroblue tetrazolium reduction tests. However, one of these latter four had Kx-negative neutrophils that functioned normally. These data suggest that closely linked but distinct genes on the X chromosome code for chronic granulomatous disease and Kx. In addition, close linkage was demonstrated between Xk and Xg, a gene coding for an erythrocyte surface antigen.

Defective neutrophil function in workers occupationally exposed to hexachlorobenzene

1997 ◽  
Vol 16 (6) ◽  
pp. 322-326 ◽  
Author(s):  
Mls Queiroz ◽  
C. Bincoletto ◽  
Rcr Perlingeiro ◽  
CA Souza ◽  
H. Toledo
Keyword(s):  
Respiratory Burst ◽  
Polymorphonuclear Leukocytes ◽  
Neutrophil Function ◽  
Nitroblue Tetrazolium ◽  
Chlorinated Compounds ◽  
Blood Concentrations ◽  
Nitroblue Tetrazolium Reduction ◽  
Occupationally Exposed ◽  
Tetrazolium Reduction ◽  
Age And Sex

In this work we have studied the respiratory burst and chemotaxis of polymorphonuclear leukocytes from 51 workers exposed to chlorinated compounds, which were compared with those of non-exposed, age- and sex- matched invididuals. These two neutrophil functions were significantly reduced as compared to controls. No correlation was observed between the length of exposure, hexachlorobenzene (HCB) blood concentrations and neu trophil chemotaxis or the extent of nitroblue tetrazolium reduction.

scholarly journals Variant of X-Linked Chronic Granulomatous Disease Revealed by a SevereBurkholderia cepaciaInvasive Infection in an Infant

2013 ◽  
Vol 2013 ◽  
pp. 1-5
Author(s):  
Saul Oswaldo Lugo Reyes ◽  
Nizar Mahlaoui ◽  
Carolina Prando ◽  
Lizbeth Blancas Galicia ◽  
Marjorie Hubeau ◽  
...  
Keyword(s):  
Chronic Granulomatous Disease ◽  
Burkholderia Cepacia ◽  
Recurrent Infection ◽  
Granulomatous Disease ◽  
Protein Subunits ◽  
Nitroblue Tetrazolium Reduction ◽  
Bacteria And Fungi ◽  
High Index Of Suspicion ◽  
Tetrazolium Reduction ◽  
Increased Susceptibility

Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by increased susceptibility to bacteria and fungi since early in life, caused by mutations in any of the five genes coding for protein subunits in NADPH oxidase. X-linked variant CGD can be missed during routine evaluation or present later in life due to hypomorphic mutations and a residual superoxide production. The case of a 10-month-old boy who died of pneumonia is reported. The isolation ofBurkholderia cepaciafrom his lung, together with a marginally low nitroblue tetrazolium reduction assay (NBT), made us suspect and pursue the molecular diagnosis of CGD. A postmortem genetic analysis finally demonstrated CGD caused by a hypomorphic missense mutation with normal gp91phoxexpression. In a patient being investigated for unusually severe or recurrent infection, a high index of suspicion of immunodeficiency must be maintained.

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