Diurnal Fluctuation of Leukocyte G6PD Activity. A Possible Explanation for the Normal Neutrophil Bactericidal Activity and the Low Incidence of Pyogenic Infections in Patients with Severe G6PD Deficiency in Israel
Abstract:Acute hemolytic anemia associated with red blood cell (RBC) glucose-6-phosphate dehydrogenase (G6PD) deficiency is commonly encountered in the Mediterranean basin. Nevertheless, concomitant clinical evidence of white blood cell G6PD deficiency is extremely rare in Israel. This study sought to assess simultaneously levels of G6PD activity in polymorphonuclear leukocytes (PMN) and in red blood cells (RBC) of patients with G6PD deficiency, including full-term newborn infants. In PMN, the correlation between G6PD … Show more
“…32 In 24 Israeli patients average G6PD enzyme activity of 13.6% in neutrophils with residual ROS production of 42.1% was sufficient for E coli killing and absence of clinical bacterial or fungal infections. 33 NET formation was not analyzed in these patients, but it is likely to be normal. This highlights the importance of determining G6PD enzyme activity, as well as levels of ROS production for optimal counseling of class I/II G6PD-deficient patients with very low or absent levels of enzyme function.…”
Because severe G6PD deficiency can be a phenocopy of chronic granulomatous disease with regard to the cellular and clinical phenotype, careful evaluation of neutrophil function seems mandatory in these patients to decide on appropriate anti-infective preventive measures. Determining the level of G6PD enzyme activity should be followed by analysis of reactive oxygen species production and NET formation to decide on required antibiotic and antimycotic prophylaxis.
“…32 In 24 Israeli patients average G6PD enzyme activity of 13.6% in neutrophils with residual ROS production of 42.1% was sufficient for E coli killing and absence of clinical bacterial or fungal infections. 33 NET formation was not analyzed in these patients, but it is likely to be normal. This highlights the importance of determining G6PD enzyme activity, as well as levels of ROS production for optimal counseling of class I/II G6PD-deficient patients with very low or absent levels of enzyme function.…”
Because severe G6PD deficiency can be a phenocopy of chronic granulomatous disease with regard to the cellular and clinical phenotype, careful evaluation of neutrophil function seems mandatory in these patients to decide on appropriate anti-infective preventive measures. Determining the level of G6PD enzyme activity should be followed by analysis of reactive oxygen species production and NET formation to decide on required antibiotic and antimycotic prophylaxis.
“…The data on our three brothers with neutrophil G6PD deficiency were previously published [51]. A possible explanation for normal SOP and BA and lack of recurrent infections in two of them could be related to low fluctuating levels of G6PD activity of neutrophils (0-5%), which is sufficient for maintaining an adequate NADPH oxidase response [52]. Four patients were diagnosed with LAD-1; two died before mutation analysis was performed.…”
PPDs are identified in 5.7% of patients with recurrent pyogenic infections; in the remainder, phagocytic dysfunction may be related to deleterious effects of persistent infection, drug consumption, or disorders not yet established.
“…On the other hand, iron deficiency suppresses circadian changes in monocyte phagocytosis (Barkova and Nazarenko 2005). A significant circadian fluctuation of glucose-6-phosphate dehydrogenase was found in both erythrocytes and neutrophils (Wolach et al 2004).…”
Cyclic changes play an important part of the space structure of an organism. Knowledge of the rhythms in haematological characteristics is important for both laboratory medicine and comparative pathology. New findings in chronohaematology published during the last two years are discussed. It seems that the haemato-immune circadian system reflects a circadian clock which is partially independent on the circadian clock in suprachiasmatic nuclei.
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