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 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 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 Erythrocyte cellular and membrane deformability in hereditary spherocytosis

Blood ◽  
1979 ◽  
Vol 53 (3) ◽  
pp. 481-485 ◽  
Author(s):  
K Nakashima ◽  
E Beutler
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Volume Ratio ◽  
Osmotic Fragility ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Relative Rigidity ◽  
Altered Surface ◽  
Red Cell Membranes ◽  
Expected Increase

In order to determine whether the relative rigidity of the hereditary spherocytosis (HS) red cell is due to membrane rididity or merely to an altered surface/volume ratio, we investigated the deformability of resealed red cell membranes from patients with HS. Whereas the osmotic fragility of intact red cells of HS patients showed the expected increase, the osmotic fragility of resealed HS membranes was normal, thus indicating that their surface/volume ratio was normal. Measurements with an ektacytometer showed that deformability of intact HS cells was markedly diminished, whereas deformability of resealed HS membranes was normal. These findings indicate that the HS red cell membrane is not intrinsically abnormally rigid, as has been suggested, but that the lack of deformability of the erythrocyte is primarily a function of the altered surface/volume ratio.

scholarly journals Erythrocyte cellular and membrane deformability in hereditary spherocytosis

Blood ◽  
1979 ◽  
Vol 53 (3) ◽  
pp. 481-485 ◽  
Author(s):  
K Nakashima ◽  
E Beutler
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Volume Ratio ◽  
Osmotic Fragility ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Relative Rigidity ◽  
Altered Surface ◽  
Red Cell Membranes ◽  
Expected Increase

Abstract In order to determine whether the relative rigidity of the hereditary spherocytosis (HS) red cell is due to membrane rididity or merely to an altered surface/volume ratio, we investigated the deformability of resealed red cell membranes from patients with HS. Whereas the osmotic fragility of intact red cells of HS patients showed the expected increase, the osmotic fragility of resealed HS membranes was normal, thus indicating that their surface/volume ratio was normal. Measurements with an ektacytometer showed that deformability of intact HS cells was markedly diminished, whereas deformability of resealed HS membranes was normal. These findings indicate that the HS red cell membrane is not intrinsically abnormally rigid, as has been suggested, but that the lack of deformability of the erythrocyte is primarily a function of the altered surface/volume ratio.

The role of the red cell membrane in thrombin generation

2013 ◽  
Vol 131 (5) ◽  
pp. 377-382 ◽  
Author(s):  
Matthew F. Whelihan ◽  
Kenneth G. Mann
Keyword(s):  
Cell Membrane ◽  
Thrombin Generation ◽  
Red Cell ◽  
Red Cell Membrane

scholarly journals Diminished osmotic fragility of human erythrocytes following the membrane insertion of oxygenated sterol compounds

Blood ◽  
1981 ◽  
Vol 58 (2) ◽  
pp. 317-325
Author(s):  
RA Streuli ◽  
JR Kanofsky ◽  
RB Gunn ◽  
S Yachnin
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Osmotic Fragility ◽  
Human Erythrocytes ◽  
Fragility Curve ◽  
Membrane Insertion ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Mean Cell Volume ◽  
Osmotic Lysis

Oxygenated sterol compounds (OSC), when incubated for 1 hr with human erythrocytes in lipoprotein-depleted medium at concentrations of 0.625- 5 X 10(-5) M, are inserted into the cell membrane and remain there despite subsequent washing of the cells. The insertion results in expansion of the surface area of the red cell ghost membrane, an increase in critical hemolytic volume, and as a consequence, in diminished osmotic fragility of the erythrocytes. This effect is seen with echinocyte-forming as well as with non-echinocyte-forming OSC. Erythrocytes treated with OSC do not differ from control cells with respect to their mean cell volume (MCV) in isotonic solution, water content, ion fluxes, and filterability through polycarbonate filters. The shift of the osmotic fragility curve toward lower NaCl concentrations is proportional to the amount of OSC inserted into the red cell membrane. 7 beta-Hydroxycholesterol, 22-ketocholesterol, and 20 alpha-hydroxycholesterol are the most potent inhibitors of osmotic lysis. The effect of OSC on osmotic fragility is diminished if the erythrocytes are incubated in a lipoprotein-containing medium; free cholesterol, however, does not change this effect. Various progesterones also protect red cell from osmotic lysis, but only if the erythrocytes are directly exposed to the compounds present in the hypotonic NaCl solutions used for measurement of their osmotic fragility. Progesterones do not remain in the membrane after the cells have been washed. The OSC are also capable of correcting the osmotic fragility curve of red cells from patients with hereditary spherocytosis. These experiments may suggest an approach to the pharmacologic treatment of hereditary spherocytosis.

scholarly journals Diminished osmotic fragility of human erythrocytes following the membrane insertion of oxygenated sterol compounds

Blood ◽  
1981 ◽  
Vol 58 (2) ◽  
pp. 317-325 ◽  
Author(s):  
RA Streuli ◽  
JR Kanofsky ◽  
RB Gunn ◽  
S Yachnin
Keyword(s):  
Cell Membrane ◽  
Hereditary Spherocytosis ◽  
Osmotic Fragility ◽  
Human Erythrocytes ◽  
Fragility Curve ◽  
Membrane Insertion ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Mean Cell Volume ◽  
Osmotic Lysis

Abstract Oxygenated sterol compounds (OSC), when incubated for 1 hr with human erythrocytes in lipoprotein-depleted medium at concentrations of 0.625- 5 X 10(-5) M, are inserted into the cell membrane and remain there despite subsequent washing of the cells. The insertion results in expansion of the surface area of the red cell ghost membrane, an increase in critical hemolytic volume, and as a consequence, in diminished osmotic fragility of the erythrocytes. This effect is seen with echinocyte-forming as well as with non-echinocyte-forming OSC. Erythrocytes treated with OSC do not differ from control cells with respect to their mean cell volume (MCV) in isotonic solution, water content, ion fluxes, and filterability through polycarbonate filters. The shift of the osmotic fragility curve toward lower NaCl concentrations is proportional to the amount of OSC inserted into the red cell membrane. 7 beta-Hydroxycholesterol, 22-ketocholesterol, and 20 alpha-hydroxycholesterol are the most potent inhibitors of osmotic lysis. The effect of OSC on osmotic fragility is diminished if the erythrocytes are incubated in a lipoprotein-containing medium; free cholesterol, however, does not change this effect. Various progesterones also protect red cell from osmotic lysis, but only if the erythrocytes are directly exposed to the compounds present in the hypotonic NaCl solutions used for measurement of their osmotic fragility. Progesterones do not remain in the membrane after the cells have been washed. The OSC are also capable of correcting the osmotic fragility curve of red cells from patients with hereditary spherocytosis. These experiments may suggest an approach to the pharmacologic treatment of hereditary spherocytosis.

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