scholarly journals Effects of vitamin E supplementation on some aspects of hematological variables in patients of hemolytic anemia with glucose 6 phosphate dehydrogenase (G6PD) deficiency

1970 ◽  
pp. 12-17 ◽  
Author(s):  
Nayma Sultana ◽  
Noorzahan Begum ◽  
Shelina Begum ◽  
Sultana Ferdousi ◽  
Taskina Ali
Keyword(s):  
Vitamin E ◽  
Hemolytic Anemia ◽  
G6pd Deficiency ◽  
Serum Bilirubin ◽  
Reticulocyte Count ◽  
Healthy Control ◽  
Hb Concentration ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Vitamin E Supplementation

Vitamin E works within the cell membrane as a biological antioxidant and may prevent premature destruction of RBC in Glucose 6-phosphate dehydrogenase (G6PD) deficient hemolytic anemia. Changes in some of the hematological variables like hemoglobin (Hb) concentration, total count (TC) of RBC, packed cell volume (PCV) and reticulocyte counts may occur due to hemolysis of RBC in G6PD deficiency In the present study the role of vitamin E supplementation on these changes were observed in reducing chronic hemolysis in anemic patients with glucose 6-phosphate dehydrogenase (G6PD) deficiency 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 (experimental group) and 34 were nonsupplemented 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 5. 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 baseline 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, Hb%, TC of RBC, PCV, reticulocyte count and serum bilirubin level of all subjects were measured by standard laboratory techniques. All the parameters were measured on day 1(one) of their 1st visit forall the groups and also were on day 60 in deficient group. Data were compared among the different groups, also in supplemented group just before and after supplementation. Analysis of data was done by appropriate statistical method. Mean blood Hb%, TC of RBC and PCV were significantly lower but reticulocyte count and serum bilirubin levels were significantly higher in patients suffering from hemolytic anemia due to G6PD deficiency in comparison to those of the healthy control. After supplementation with vitamin E (i.e. on day-60) Hb concentration, total count of RBC, PCV were significantly increased whereas, reticulocyte count and serum bilirubin levels were significantly decreased 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. Therefore, from this study it may be concluded that, deterioration of some of the hematological parameters occur in G6PD deficient hemolytic anemic patients, improvement of which occur following vitamin E supplementation, which clearly indicates the role of this antioxidant vitamin in reducing the rate of hemolysis in this group of patients. So, vitamin E supplementation can be considered along with other drugs to treat this group of patients. DOI: 10.3329/bjpp.v22i1.3563 Bangladesh J Physiol Pharmacol 2006; 22(1/2) : 12-17

scholarly journals Effects of Vitamin E Supplementation on Reducing Chronic Hemolysis in Glucose 6 Phosphate Dehydrogenase (G6PD) Deficiency

1970 ◽  
Vol 6 (1) ◽  
pp. 70-76
Author(s):  
Nayma Sultana ◽  
Noorzahan Begum ◽  
Shelima Begum ◽  
Sultana Ferdousi ◽  
Taskina Ali
Keyword(s):  
Vitamin E ◽  
Peripheral Blood ◽  
G6pd Deficiency ◽  
Blood Film ◽  
Antioxidant Vitamin ◽  
Peripheral Blood Film ◽  
Healthy Control ◽  
Group B ◽  
Vitamin E Supplementation

Background: Vitamin E works within the cell membrane as an antioxidant and may prevent destruction of RBC in G6PD deficient hemolytic anemia, which can be reflected by changes in peripheral blood film. Objective: To observe the role of vitamin E supplementation on restoring normal cell types in peripheral blood film in order to evaluate the role of this antioxidant vitamin in reducing chronic hemolysis in G6PD deficient patients. Method: Total 102 subjects, age range from 5-40 years of both sexes were included in the study. Among them 68 were G6PD enzyme deficient patients, of whom 34 were in non-supplemented group (Group B) and 34 were in supplemented group (Group C). Both group B and C were divided into Group B1 and C1 (on day 1 ) and also into B2 and C2 (on day 60) respectively. Supplemented group received vitamin E supplementation for 60 consecutive days (800 IU/day for adult and 400 IU/day for children in a divided dose i,e. 4 times daily). Age and sex matched 34 apparently healthy subjects with normal G6PD level (Group A) were also taken to observe baseline data. Determination of Erythrocyte G6PD level and preparation of peripheral blood film were done on day 1 for all groups and also on day 60 in deficient groups. Results: Percentage of subjects with presence of some abnormal red cells in peripheral blood film was significantly higher in patients of hemolytic anemia with G6PD deficiency in comparison to that of healthy control. After supplementation with vitamin E (i,e. on day-60) this percentage was significantly decreased towards those of healthy control in their supplemented group in comparison to that of pre-supplemented (day-1) and nonsupplemented groups. Conclusion: Some abnormal red cells may be found in peripheral blood film of G6PD deficient patients, improvement of which occur following vitamin E supplementation, and thereby indicates role of this antioxidant vitamin in reducing the rate of hemolysis. Key words: G6PD deficiency; Peripheral blood film; Vitamin E DOI: http://dx.doi.org/10.3329/jbsp.v6i1.8088 J Bangladesh Soc Physiol. 2011 June; 6(1): 70-76

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

2009 ◽  
Vol 1 (1) ◽  
pp. 6
Author(s):  
Nayma Sultana ◽  
Noorzahan Begum ◽  
Shelina Begum ◽  
Sultana Ferdousi ◽  
Taskina Ali
Keyword(s):  
Vitamin E ◽  
Cell Membrane ◽  
G6pd Deficiency ◽  
Healthy Subjects ◽  
Osmotic Fragility ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Healthy Control ◽  
Vitamin E Supplementation

<p><strong>Background: </strong>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 &amp; MCHC.</p> <p><strong>Objective: </strong>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.</p> <p><strong>Methods: </strong>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 &lt; 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.</p> <p><strong>Results: </strong>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.</p> <p><strong>Conclusion: </strong>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.</p> <p><strong>Key words: </strong>Osmotic fragility, G6PD, hemolytic anemia, vitamin E.</p><p>DOI: 10.3329/bsmmuj.v1i1.3688</p> <p><em>BSMMU J </em>2008; 1(1): 6-10</p>

scholarly journals Clinical spectrum and severity of hemolytic anemia in glucose 6-phosphate dehydrogenase–deficient children receiving dapsone

Blood ◽  
2012 ◽  
Vol 120 (20) ◽  
pp. 4123-4133 ◽  
Author(s):  
Allan Pamba ◽  
Naomi D. Richardson ◽  
Nick Carter ◽  
Stephan Duparc ◽  
Zul Premji ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Hemolytic Anemia ◽  
G6pd Deficiency ◽  
Case Reports ◽  
Drug Induced ◽  
Once Daily ◽  
Maximum Decrease ◽  
Life Threatening ◽  
The Mean ◽  
Glucose 6 Phosphate Dehydrogenase

AbstractDrug-induced acute hemolytic anemia led to the discovery of G6PD deficiency. However, most clinical data are from isolated case reports. In 2 clinical trials of antimalarial preparations containing dapsone (4,4′-diaminodiphenylsulfone; 2.5 mg/kg once daily for 3 days), 95 G6PD-deficient hemizygous boys, 24 G6PD-deficient homozygous girls, and 200 girls heterozygous for G6PD deficiency received this agent. In the first 2 groups, there was a maximum decrease in hemoglobin averaging −2.64 g/dL (range −6.70 to +0.30 g/dL), which was significantly greater than for the comparator group receiving artemether-lumefantrine (adjusted difference −1.46 g/dL; 95% confidence interval −1.76, −1.15). Hemoglobin concentrations were decreased by ≥ 40% versus pretreatment in 24/119 (20.2%) of the G6PD-deficient children; 13/119 (10.9%) required blood transfusion. In the heterozygous girls, the mean maximum decrease in hemoglobin was −1.83 g/dL (range +0.90 to −5.20 g/dL); 1 in 200 (0.5%) required blood transfusion. All children eventually recovered. All the G6PD-deficient children had the G6PD A− variant, ie, mutations V68M and N126D. Drug-induced acute hemolytic anemia in G6PD A− subjects can be life-threatening, depending on the nature and dosage of the drug trigger. Therefore, contrary to current perception, in clinical terms the A− type of G6PD deficiency cannot be regarded as mild. This study is registered at http://www.clinicaltrials.gov as NCT00344006 and NCT00371735.

Severe-glucose-6-phosphate dehydrogenase (G6PD) deficiency associated with chronic hemolytic anemia, granulocyte dysfunction, and increased susceptibility to infections: description of a new molecular variant (G6PD Barcelona)

Blood ◽  
1982 ◽  
Vol 59 (2) ◽  
pp. 428-434 ◽  
Author(s):  
JL Vives Corrons ◽  
E Feliu ◽  
MA Pujades ◽  
F Cardellach ◽  
C Rozman ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
G6pd Deficiency ◽  
Specific Activity ◽  
Heat Stability ◽  
Molecular Characteristics ◽  
Functional Studies ◽  
Leukocyte Alkaline Phosphatase ◽  
Severe Deficiency ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Increased Susceptibility

Abstract Molecular, kinetic, and functional studies were carried out on erythrocytes and leukocytes in a Spanish male with G6PD deficiency, congenital nonspherocytic hemolytic anemia (CNSHA), and increased susceptibility to infections. G6PD activity was absent in patient's red cells and was about 2% of normal in leukocytes. Molecular studies using standard methods (WHO, 1967) showed G6PD in the patient to have a slightly fast electrophoretic mobility at pH 8.0 with otherwise normal properties (heat stability at 46 degrees C, apparent affinity for substrates, optimum pH, and utilization of substrate analogues). Other tests showed the patient's granulocytes to engulf latex particles normally, but to have impaired reduction of nitroblue tetrazolium and ferricytochrome-c as well as reduced iodination. Chemotaxis and random migration of the patient's granulocytes were normal as were myeloperoxidase, leukocyte alkaline phosphatase (LAP), and ultrastructural features. The molecular characteristics of G6PD in the patient differed from those of all previously reported variants associated with CNSHA, so the present variant was provisionally called G6PD Barcelona to distinguish it from other G6PD variants previously described. Possible mechanisms for the severe deficiency of G6PD in erythrocytes and granulocytes was investigated by studies on the immunologic specific activity of the mutant enzyme.

An examination of the role of vitamin E in glucose-6-phosphate dehydrogenase

1979 ◽  
Vol 12 (5) ◽  
pp. 149-151 ◽  
Author(s):  
Johannah G. Newman ◽  
Thomas B. Newman ◽  
Lemuel J. Bowie ◽  
John Mendelsohn
Keyword(s):  
Vitamin E ◽  
Glucose 6 Phosphate Dehydrogenase

scholarly journals tert-Butyl Hydroperoxide (tBHP)-Induced Lipid Peroxidation and Embryonic Defects Resemble Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency in C. elegans

2020 ◽  
Vol 21 (22) ◽  
pp. 8688
Author(s):  
Hung-Chi Yang ◽  
Hsiang Yu ◽  
Tian-Hsiang Ma ◽  
Wen-Ye Tjong ◽  
Arnold Stern ◽  
...  
Keyword(s):  
Lipid Peroxidation ◽  
G6pd Deficiency ◽  
Short Term ◽  
Butyl Hydroperoxide ◽  
Tert Butyl ◽  
C Elegans ◽  
Tert Butyl Hydroperoxide ◽  
Calcium Independent Phospholipase A2 ◽  
Glucose 6 Phosphate Dehydrogenase

G6PD is required for embryonic development in animals, as severe G6PD deficiency is lethal to mice, zebrafish and nematode. Lipid peroxidation is linked to membrane-associated embryonic defects in Caenorhabditis elegans (C. elegans). However, the direct link between lipid peroxidation and embryonic lethality has not been established. The aim of this study was to delineate the role of lipid peroxidation in gspd-1-knockdown (ortholog of g6pd) C. elegans during reproduction. tert-butyl hydroperoxide (tBHP) was used as an exogenous inducer. Short-term tBHP administration reduced brood size and enhanced germ cell death in C. elegans. The altered phenotypes caused by tBHP resembled GSPD-1 deficiency in C. elegans. Mechanistically, tBHP-induced malondialdehyde (MDA) production and stimulated calcium-independent phospholipase A2 (iPLA) activity, leading to disturbed oogenesis and embryogenesis. The current study provides strong evidence to support the notion that enhanced lipid peroxidation in G6PD deficiency promotes death of germ cells and impairs embryogenesis in C. elegans.

Chronic nonspherocytic hemolytic anemia (CNSHA) and glucose 6 phosphate dehydrogenase (G6PD) deficiency in a patient with familial amyloidotic polyneuropathy (FAP)

1989 ◽  
Vol 81 (2) ◽  
pp. 161-164 ◽  
Author(s):  
Joan Lluis Vives-Corrons ◽  
M. Assumpci� Pujades ◽  
Josep Petit ◽  
Dolors Colomer ◽  
Montserrat Corbella ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
G6pd Deficiency ◽  
Familial Amyloidotic Polyneuropathy ◽  
Glucose 6 Phosphate Dehydrogenase

Vitamin E in the neuroprotection of cisplatin induced peripheral neurotoxicity and ototoxicity

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 9114-9114 ◽  
Author(s):  
A. Pace ◽  
S. Carpano ◽  
E. Galiè ◽  
A. Savarese ◽  
M. Della Giulia ◽  
...  
Keyword(s):  
Placebo Group ◽  
Vitamin E ◽  
Peripheral Neurotoxicity ◽  
Double Blind ◽  
Multicentric Study ◽  
Phase Ii Studies ◽  
Significant Difference ◽  
Before And After ◽  
Vitamin E Supplementation

9114 Background: Peripheral neurotoxicity is a well recognized effect of cisplatin chemotherapy that can result in severe disability and represents a major dose-limiting factor. Several phase II studies have recently investigated the role of vitamin E as neuroprotectant in the prevention of cisplatin induced peripheral neurotoxicity and ototoxicity. Methods: An Italian randomized, placebo controlled, double blind multicentric study is ongoing to confirm the role of vitamin E supplementation in the prevention of neurotoxicity and ototoxicity induced by cisplatin Patients candidates to cisplatin chemotherapy were randomised to either vitamin E supplementation (a-tocopherol 400 mg/day) or to placebo. Patients were evaluated with neurological and neurophysiological examination before and after treatment. Neurotoxicity was measured using the comprehensive clinical and neurophysiological Total Neuropathy Score (TNS). Ototoxicity was evaluated with audiometric test and acoustic evoked potential before and after treatment. Results: 81 patients have been enrolled in 3 italian oncologic centers. An interim analysis on the first 50 patients was carried out. 25 patients (11 in the vit E group and 14 in the placebo group) received a cumulative dose higher than 300 mg/mq and were evaluable for neurotoxicity. Statistical analysis showed a significant difference (p < 0.05) in median neurotoxicity score observed in vit E group (TNS=1) respect to the placebo group (TNS=5). Conclusions: This is the first randomised, placebo controlled, double blind trial exploring the efficacy of vitamin E in the neuroprotection of cisplatin neurotoxicity. The results of this study confirm the neuroprotective effect of vitamin E supplementation against cisplatin-induced neurotoxicity. No significant financial relationships to disclose.

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