Glucose-6-phosphate Dehydrogenase Deficient Subjects Research Articles

Prevalence Of Glucose-6-Phosphate Dehydrogenase Deficiency in People Living in Katsina Metropolis

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most prevalent human enzyme defects observed in malaria endemic areas. The study included 212 participants, with 142 (67%) male and 70 (33%) female, aged between14 to 59 with a mean age of 24.20±9.35 years. The study was granted ethical approval by the Katsina State Ministry of Health. Each subject gave his/her written informed permission after counseling. G6PD deficiency, malaria parasite, reduced glutathione (GSH), glutathione peroxidase (GPx), packed cell volume (PCV) and hemoglobin (Hb) were assayed using standard procedures. Among the 212 participants tested in the study, 40 (19%) were found to be G6PD deficient and 172 (81%) participants were normal. Among 212 participants tested, 75 (35.4%) were infected with malaria parasite out of which 13 (17.3%) were G6PD deficient and 62 (82.7%) were normal. Among the 40 G6PD deficient subjects, 33 (82.5%) were male while 7 (17.5%) were females. GSH concentration was lowered significantly in G6PD-deficient subjects compared to the normal subjects. GPx, Hb and PCV showed no significant difference (p>0.05) in the deficient subjects compared to the normal subjects. The results of this study suggest that G6PD deficiency is quite common with a prevalence of 19% in the population of Katsina metropolis. The data also shows that males are more likely to be affected by G6PD deficiency than females.

The G6PD flow-cytometric assay is a reliable tool for diagnosis of G6PD deficiency in women and anaemic subjects

Glucose-6-phosphate dehydrogenase (G6PD) activity is essential for redox equilibrium of red blood cells (RBCs) and, when compromised, the RBCs are more susceptible to haemolysis. 8-aminoquinolines (primaquine and tafenoquine) are used for the radical curative treatment of Plasmodium vivax malaria and can cause haemolysis in G6PD deficient subjects. Haemolytic risk is dependent on treatment dose and patient G6PD status but ultimately it correlates with the number of G6PD deficient RBCs. The G6PD spectrophotometric assay reliably identifies deficient subjects but is less reliable in heterozygous females, especially when other blood conditions are present. In this work we analysed samples with a range of G6PD phenotypes and haematologic conditions from 243 healthy volunteers of Asian or African-American heritage using both the spectrophotomeric assay and the G6PD flow-cytometric assay. Overall 18.5% of subjects (29.3% of Asian females) presented with anaemia, associated with decreased RBCs volume (MCV) and reticulocytosis; the flow-cytometric assay showed good correlation with the spectrophotometric assay (Pearson’s r 0.918–0.957) and was less influenced by haemoglobin concentration, number of RBCs and number of reticulocytes. This resulted in more precise quantification of the number of G6PD deficient RBCs and presumably higher predictive power of drug induced haemolytic risk.

Adverse effects of herbal or dietary supplements in G6PD deficiency: a systematic review.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common genetic disorder, affecting nearly 400 million individuals worldwide. Whilst it is known that a number of drugs, foods and chemicals can trigger haemolysis in G6PD deficient individuals, the association between herbal and dietary supplements and haemolysis is less clear. The objective of this study was to evaluate the association between herbal or dietary supplements and adverse events in G6PD deficient individuals. We searched 14 electronic databases from their inception until November 2015 for articles describing the use of herbal or dietary supplements in G6PD deficient individuals. Additional publications were identified from manually searching textbooks, conference abstracts and the grey literature. All study designs were included as long as they contained clinical information. These gathered findings were summarized narratively. Thirty-two publications met inclusion criteria. These reported on 10 herbal and dietary supplements. Overall evidence linking haemolysis to a herbal/dietary supplement was only found for henna. No evidence of harm was observed for vitamin C, vitamin E, vitamin K, Gingko biloba and α-lipoic acid. The review showed that there was insufficient evidence to contravene the use of most herbal or dietary products at therapeutic doses in G6PD deficient subjects.

Glucose 6-phosphate dehydrogenase deficient subjects may be better “storers” than donors of red blood cells

Storage of packed red blood cells (RBCs) is associated with progressive accumulation of lesions, mostly triggered by energy and oxidative stresses, which potentially compromise the effectiveness of the transfusion therapy. Concerns arise as to whether glucose 6-phosphate dehydrogenase deficient subjects (G6PD−), ~5% of the population in the Mediterranean area, should be accepted as routine donors in the light of the increased oxidative stress their RBCs suffer from. To address this question, we first performed morphology (scanning electron microscopy), physiology and omics (proteomics and metabolomics) analyses on stored RBCs from healthy or G6PD− donors. We then used an in vitro model of transfusion to simulate transfusion outcomes involving G6PD− donors or recipients, by reconstituting G6PD− stored or fresh blood with fresh or stored blood from healthy volunteers, respectively, at body temperature. We found that G6PD− cells store well in relation to energy, calcium and morphology related parameters, though at the expenses of a compromised anti-oxidant system. Additional stimuli, mimicking post-transfusion conditions (37°C, reconstitution with fresh healthy blood, incubation with oxidants) promoted hemolysis and oxidative lesions in stored G6PD− cells in comparison to controls. On the other hand, stored healthy RBC units showed better oxidative parameters and lower removal signaling when reconstituted with G6PD− fresh blood compared to control. Although the measured parameters of stored RBCs from the G6PD deficient donors appeared to be acceptable, the results from the in vitro model of transfusion suggest that G6PD− RBCs could be more susceptible to hemolysis and oxidative stresses post-transfusion. On the other hand, their chronic exposure to oxidative stress might make them good recipients, as they better tolerate exposure to oxidatively damaged long stored healthy RBCs.

Making a case for hepatitis A vaccination in glucose-6 phosphate dehydrogenase deficient subjects

Making a case for hepatitis A vaccination in glucose-6 phosphate dehydrogenase deficient subjects

Several mutations including two novel mutations of the glucose-6-phosphate dehydrogenase gene in Polish G6PD deficient subjects with chronic nonspherocytic hemolytic anemia, acute hemolytic anemia, and favism

DNA sequencing revealed seven different glucose-6-phosphate dehydrogenase (G6PD) mutations in G6PD deficient subjects from 10 Polish families. Among them we found two novel mutations: 679C-->T (G6PD Radlowo, class 2) and a 1006A-->G (G6PD Torun, class 1). Variant G6PD Radlowo was characterized biochemically. Both novel mutations were analyzed using a model of the tertiary structure of the human enzyme. The main chain of G6PD Torun is different from the wild-type G6PD. The remaining mutations identified by us in deficient Polish patients were: 542A-->T (G6PD Malaga), 1160G-->A (G6PD Beverly Hills), 1178G-->A (G6PD Nashville), 1192G-->A (G6PD Puerto Limon), and 1246G-->A (G6PD Tokyo). Variant Tokyo was found in four families. In one of them favism was the first clinical sign of G6PD deficiency and chronic nonspherocytic hemolytic anemia (CNSHA) was diagnosed later. Variants G6PD Nashville and G6PD Puerto Limon were accompanied by the silent mutation 1311C-->T of the G6PD gene.

G6PD activity and gene expression in leukemic cells from G6PD-deficient subjects

In the present study we examined gene expression and glucose-6-phosphate dehydrogenase (G6PD) activity in leukemic cells isolated from G6PD normal and deficient subjects. The results have shown that G6PD activity strongly increases in G6PD normal leukemic cells as well as in G6PD deficient leukemic cells when compared to peripheral blood mononuclear cells (PBMC). Higher levels of G6PD gene expression were observed in leukemic cells from G6PD deficient patients compared to G6PD normal. A similar pattern of gene expression was also observed for 3-hydroxy-3-methylglutaryl coenzyme A (HMGCoA) reductase. These results support the hypothesis that G6PD deficient cell, in order to sustain their growth, must respond to the low activity of their mutant enzyme with an increase in quantity through an induction of gene expression.

Glucose-6-phosphate dehydrogenase (G6PD) activity in tumoral tissues of G6PD-deficient subjects affected by larynx carcinoma

The activity of glucose-6-phosphate dehydrogenase (G6PD), the key enzyme of the hexose monophosphate (HMP) shunt pathway, was measured in both normal and tumoral larynx tissues from normal and G6PD deficient subjects. Significant increases of this enzymatic activity were found in tumoral tissues of both normal and G6PD deficient subjects, who were characterized by very low levels of G6PD activity in erythrocytes as well as in larynx tissue.

Glucose-6-Phosphate Dehydrogenase (G6PD) status in Female Type 2 Diabetes Mellitus and its Relationship with HbA<sub>1</sub>C

Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency may be one of the risk factor for type 2 diabetes mellitus. Objective: To observe erythrocyte G6PD status in type 2 female diabetic patients and also to find out its relationship with glycosylated hemoglobin. Methods: This cross sectional study was carried out in the Department of Physiology, Bangabandhu Sheikh Mujib Medical University, Shahbag, Dhaka from January to December 2009. For this, 60 female patients with type 2 diabetes mellitus, age ranged from 40 to 60 years were included in the study group (group B). On the basis of glycosylated hemoglobin level (HbA1C) they were further subdivided into group B1, consisting of 30 controlled diabetics (HbA1C 4.8-6%) and group B2, consisting of 30 uncontrolled diabetic (HbA1C>6%) patients. They were selected from Out Patient Department of Bangladesh Institute of Health Science Hospital. For comparison, age & sex matched 30 apparently healthy non diabetic females (group A) were also studied. Erythrocyte G6PD level was measured by Spectrophotometer, HbA1C level by Flex reagent cartridge and serum bilirubin, Hb%, total count of RBC and reticulocyte% were measured by standard laboratory techniques. For statistical analysis ANOVA, independent sample t test, χ2 test and Pearson's correlation coefficient test were performed as applicable. Results: In this study, erythrocyte G6PD level was significantly lower in both the diabetic groups (p <0.001) than those of control group but their difference when compared between B1 and B2 was not statistically significant. In controlled diabetics 20% and in uncontrolled diabetics 6.7% patients were found G6PD deficients. No G6PD deficient subjects were found in control group. HbA1C showed negative correlation with Erythrocyte G6PD which was only significant for uncontrolled diabetes (p < 0.05) Conclusion: This study concludes that G6PD deficiency may be one of the risk factor for type 2 diabetes mellitus irrespective of blood glucose control status.Key words: Glucose-6-PD; Diabetes; Female.DOI: 10.3329/jbsp.v5i2.6778J Bangladesh Soc Physiol. 2010 December; 5(2): 60-65

Glucose Tolerance in G6PD Deficient Children

THE ENZYME glucose-6-phosphate dehydrogenase (G6PD) initiates the oxidative decarboxylation of glucose-6-phosphate in the pentose phosphate pathway. This reaction results in the regeneration of the coenzyme TPNH, and the formation of CO 2 and pentose phosphate. Since G6PD is present in most human tissues, 1 the question whether abnormalities in glucose metabolism exist in patients with G6PD deficiency is raised. Isotopic studies measuring the rate of glucose oxidation by the whole body from both glucose-1-C 14 and glucose-6-C 14 have demonstrated markedly diminished C 14 O 2 in the expired air of G6PD deficient males. 2 An abnormal oral glucose tolerance response in G6PD deficient subjects, suggesting a diabetic tendency, was reported by Chanmugam and Frumin. 3 Pointing out that diabetic state is usually mild, occurring later in life, and may be relatively asymptomatic, these authors stated that it is still unknown whether similar results would be obtained with children possessing

Factors influencing the hexose monophosphate shunt in red cells

1. (1) The oxidation of glucose in the hexose monophosphate shunt of the red cell has been studied with the use of 1- 14C-glucose. 2. (2) Besides methylene blue other substances have been shown to stimulate this pathway of glucose metabolism; these lead to the formation of methaemoglobin or oxidised glutathione. 3. (3) Drugs which produce haemolysis in glucose 6-phosphate dehydrogenase deficient subjects and which oxidise haemoglobin or glutathione also stimulate this pathway.