Red cell glucose‐6‐phosphate dehydrogenase status and pyruvate kinase activity in a Nigerian population

May, Jürgen; Meyer, Christian G.; Grossterlinden, Lars; Ademowo, Olusegun G.; Mockenhaupt, Frank P.; Olumese, P.E.; Falusi, Adeyinka G.; Luzzatto, Lucio; Bienzle, Ulrich

doi:10.1046/j.1365-3156.2000.00529.x

Cited by 50 publications

(50 citation statements)

References 15 publications

(19 reference statements)

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“…Therefore, early destruction of RBC is the consequence of higher osmotic fragility of RBC in oxidative stress 26,27 . In addition, decreased level of MCV, MCH, MCHC in hemolytic anemia with G6PD deficiency is a consequence of excessive hemolysis, more marked under oxidative stress 27,28 .…”

Section: Discussionmentioning

confidence: 99%

Effects of Oral Supplementation of Vitamin E on Fragility of RBC in Hemolytic Anemic Patients with G6PD Deficiency

Sultana¹,

Begum

et al. 2009

Bangabandhu Sheikh Mujib Medical University Journal

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Abstract:Background: Vitamin E has role in maintaining the integrity of red cell membrane by preventing oxidation of polyunsaturated fatty acids and thereby protects cells from oxidative stress-induced lysis in G6PD deficiency, which can be reflected by changes in osmotic fragility of RBC and some absolute values like MCV, MCH & MCHC. Objective: To observe the effects of vitamin E supplementation on fragility of RBC in order to evaluate role of this antioxidant vitamin in reducing chronic hemolysis in G6PD deficient patients. Methods: For this, a total number of 102 subjects with age ranged from 5 to 40 years of both sexes were included in the study. Among them 68 were G6PD enzyme deficient patients, of whom 34 were in supplemented group (study group) and 34 were in non-supplemented group (control group). The supplemented group received vitamin E supplementation for 60 consecutive days at a dose of 800 IU/day for adult and 400 IU/day for children < 12 years (in a divided dose i,e. 4 times daily). Age and sex matched 34 apparently healthy subjects with normal blood G6PD level were taken to observe the base line data (healthy control) and also for comparison. All the G6PD deficient patients were selected from Out Patient Department (OPD) of Hematology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh during the period of July 2005 to June 2006 and all the healthy subjects were selected from personal contact. Blood G6PD level, osmotic fragility of RBC were measured by standard techniques and MCV, MCH, and MCHC were obtained by calculation. All the parameters were measured on day 1 (one) of their first visit and also were on day 60 in deficient group. Data were compared among the deficient groups, also in supplemented group just before and after supplementation. Analysis of data was done by appropriate statistical method. Results: Mean starting and completing points of osmotic fragility of RBC were significantly higher but MCV, MCH, MCHC were significantly lower in patients suffering from hemolytic anemia due to G6PD deficiency in comparison to those of the healthy control. After supplementation with vitamin E starting and completing points of osmotic fragility of RBC were significantly decreased whereas, MCV, MCH, MCHC were significantly increased towards those of healthy control in supplemented group of patients in comparison to those of their pre-supplemented (day-1) and non-supplemented groups both on day 1 and day 60. Conclusion: From this study it may be concluded that, disturbances of some of the hematological parameter like higher osmotic fragility of RBC and lower MCV, MCH, MCHC occur in G6PD deficient hemolytic anemic patients, which returned towards normal after supplementation of vitamin E, which clearly indicates the role of this anti-oxidant vitamin in maintaining red cell membrane integrity and thereby decreases the rate of hemolysis in this group of patients. So, vitamin E can be supplemented along with other drugs for better management of the patients.

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Section: Discussionmentioning

confidence: 99%

Effects of Oral Supplementation of Vitamin E on Fragility of RBC in Hemolytic Anemic Patients with G6PD Deficiency

Sultana¹,

Begum

et al. 2009

Bangabandhu Sheikh Mujib Medical University Journal

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“…The possible nine genotypes were grouped as follows: hemizygous males G6PDB and G6PDA, homozygous females G6PDBB and G6PDAA and heterozygous females G6PDBA (variants with a putative normal enzyme activity) as g6pd + ; hemizygous males G6PDA − and homozygous females G6PDA − A − (putative deficient variants) as g6pd -and heterozygous females G6PDBA − and G6PDAA − (variants with a putative intermediate enzyme activity) as g6pd ± [31].…”

Section: Detection Of Glucose-6-phosphate Dehydrogenase Polymorphismsmentioning

confidence: 99%

Analysis of malaria associated genetic traits in Cabo Verde, a melting pot of European and sub Saharan settlers

Alves¹,

Machado²,

Silva³

et al. 2010

Blood Cells, Molecules, and Diseases

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“…On the other hand, G6PD activities are narrowly distributed about the main mode, with typical standard deviations of Ͻ17% (33,34). Moreover, the distribution (34) is also skewed toward high activities.…”

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confidence: 99%

Quantitative evolutionary design of glucose 6-phosphate dehydrogenase expression in human erythrocytes

Salvador

Savageau

2003

Proc. Natl. Acad. Sci. U.S.A.

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Why do the activities of some enzymes greatly exceed the flux capacity of the embedding pathways? This is a puzzling open problem in quantitative evolutionary design. In this work we investigate reasons for high expression of a thoroughly characterized enzyme: glucose 6-phosphate dehydrogenase (G6PD) in human erythrocytes. G6PD catalyses the first step of the pathway that supplies NADPH for antioxidant defense mechanisms. Normal G6PD activity far exceeds the capacity of human erythrocytes for a steady NADPH supply, which is limited upstream of G6PD. However, the distribution of erythrocyte G6PD activity in human populations reveals a selective pressure for maintaining high activity. To clarify the nature of this selective pressure, we studied how G6PD activity and other parameters in a model of the NADPH redox cycle affect metabolic performance. Our analysis indicates that normal G6PD activity is sufficient but not superfluous to avoid NADPH depletion and ensure timely adaptation of the NADPH supply during pulses of oxidative load such as those that occur during adherence of erythrocytes to phagocytes. These results suggest that large excess capacities found in some biochemical and physiological systems, rather than representing large safety factors, may reflect a close match of system design to unscrutinized performance requirements. Understanding quantitative evolutionary design thus calls for careful consideration of the various performance specifications that biological components͞processes must meet in order for the organism to be fit. The biochemical systems framework used in this paper is generally applicable for such a detailed examination of the quantitative evolutionary design of gene expression levels in other systems. U nderstanding the quantitative relationship of biological capacities to each other and to the peak natural loads on them, the problem of quantitative evolutionary design (1), is a key issue in integrative biology (2-5). Natural selection against both poor performance and misallocation of resources to superfluous ''infrastructure'' could be thought to promote a close match between capacities of components operating in series and between these capacities and the peak natural loads. Nevertheless, research at both the molecular (6-9) and the organ (2, 10) levels has shown that ratios between the highest capacities in a series and the lowest ones, as well as between capacities and their loads, are often quite high. In particular, ratios between catalytic capacities (i.e., activities, V max ) of some enzymes, which we will call ''excess-activity enzymes,'' and the flux capacity of the embedding pathways may exceed 1,000 (11). These ratios remain unexplained.It has been argued (7) that these high activities are necessary to ensure sufficient net flux in reactions that operate near equilibrium. There are two problems with this argument. First, one must still provide an evolutionary explanation for why the step in question must be near equilibrium. Second, any enzyme whose activity greatly exce...

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Red cell glucose‐6‐phosphate dehydrogenase status and pyruvate kinase activity in a Nigerian population

Cited by 50 publications

References 15 publications

Effects of Oral Supplementation of Vitamin E on Fragility of RBC in Hemolytic Anemic Patients with G6PD Deficiency

Effects of Oral Supplementation of Vitamin E on Fragility of RBC in Hemolytic Anemic Patients with G6PD Deficiency

Analysis of malaria associated genetic traits in Cabo Verde, a melting pot of European and sub Saharan settlers

Quantitative evolutionary design of glucose 6-phosphate dehydrogenase expression in human erythrocytes

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