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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors examined the ability of antioxidants to prevent in vitro oxidant damage to the sickle red blood cell (RBC). One millimolar ascorbic acid and alpha-mercaptopropionylglycine significantly (p less than 0.005) protected against RBC Heinz body formation during incubation with acetylphenylhydrazine, while cysteine, cysteamine, and methionine did not. The effect of ascorbic acid was concentration dependent with concentrations as low as 0.1 mM having significant antioxidant effects. Ascorbic acid protected the RBC against hydrogen peroxide induced hemolysis as well (p less than 0.05). Ascorbic acid had a significant stimulatory effect on the rate of glucose oxidation by the pentose phosphate shunt (PPS), especially in the sickle RBC. Ascorbic acid did not protect the RBC from a patient with chronic hemolytic anemia due to G6PDTorrance from Heinz body formation, suggesting that an intact PPS is necessary for ascorbic acid to express its antioxidant properties. These data suggest that clinical trials should be undertaken to examine the efficacy of ascorbic acid in the treatment of SCD.
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PMID:Antioxidants in sickle cell disease: the in vitro effects of ascorbic acid. 352 Dec 79

The role of oxidant damage to red cells in sickle cell disease has been of interest in recent years. Ascorbic acid is a well known antioxidant. The present study found that sickle cell patients have low levels of plasma ascorbic acid. In addition, it was observed that the pretreatment of sickle cells with ascorbic acid protects their membranes against in vitro peroxidative lipid damage induced by their exposure to hydrogen peroxide. The data suggest that low plasma ascorbic acid levels in sickle cell patients may be a factor in the increased vulnerability of sickle cells to oxidant damage in vivo.
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PMID:Reduced levels of plasma ascorbic acid (vitamin C) in sickle cell disease patients: its possible role in the oxidant damage to sickle cells in vivo. 402 44

In the circulation of sickle cell anemia patients, a certain population of erythrocytes has an elevated density. These abnormally dense cells are believed to be at the root of the painful crisis and anemia of the patients. We have developed an in vitro method for the preparation of these heavier erythrocytes by a repeated deoxy-oxy cycling of erythrocytes from sickle cell anemia patients. By using this method, we studied whether certain nutritional supplements would inhibit the formation of dense cells in vitro. It was found that aged garlic extract (AGE) as well as its components with antioxidant activity, i.e., S-allylcysteine and N alpha-(1-deoxy-D-fructos-1-yl)-L-arginine (fructosyl arginine), inhibited the formation of dense cells in vitro. Vitamin C, vitamin E and the spin-trapping agents, 5-diethoxyphophoryl-5-methyl-1-pyrroline-N-oxide and alpha-(4-pyridyl-1-oxide)-N-t-butylnitrone were all found to inhibit the formation of dense cells in vitro. These results suggest that, when extremely stretched sickle-shaped cells are formed by the repeated deoxy-oxy cycling, the erythrocyte membrane becomes susceptible to oxidative injury by reactive oxygen species. The protection of the erythrocyte membrane from such an oxidative injury would prevent the membranes from becoming leaky to the calcium ion, thus inhibiting the activation of the calcium-activated potassium efflux channel and the formation of dense cells. We also developed a new ex vivo method of studying the possible efficacy of antioxidants taken orally on the dense cell formation in sickle cell patients. It involved the use of blood plasma taken from a healthy donor (with normal hemoglobin) of AB blood type who had consumed different types of antioxidants orally. By suspending sickle erythrocytes in such plasma and exposing them to the deoxy-oxy cycling, the degree of dense cell formation was determined. The degree of inhibition in vitro by antioxidants taken orally may be related to their efficacy in inhibiting dense cell formation in the patients. On the basis of these in vivo and ex vivo studies, we propose that a cocktail of antioxidants would have beneficial effects in lessening the incidence and severity of crisis and reducing anemia in sickle cell disease.
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PMID:In vitro effects of aged garlic extract and other nutritional supplements on sickle erythrocytes. 1123 22

This study seeks to examine the effects of vitamin C supplementation or/and warmth on forearm blood flow (FBF) and forearm vascular resistance (FVR) in sickle cell anaemia (SCA) subjects in the steady state. Sixteen (16) SCA subjects of both sexes (mean age, 23.4+/-1.5 yrs.) were studied. Blood pressure (BP, mm Hg) and FBF (ml/min) measurements were made at rest, with warmth stimulation, after vitamin C supplementation for 6 weeks at 300 mg per day and with warmth stimulation after vitamin C supplementation. Warmth stimulation was induced by immersing the left foot in a bowl of water at a temperature of 40 degrees C for 2 minutes. Forearm blood flow (FBF) [corrected] was measured by means of a forearm plethysmograph. Forearm vascular resistance (FVR, arbitrary units) was calculated by dividing mean arterial pressure (MAP) with FBF. Warmth stimulation at 40 C significantly decreased systolic blood pressure (SBP) (p<0.05), diastolic blood pressure (DBP) (p<0.01), MAP (p<0.01) and FVR (p<0.01) but significantly increased FBF (p<0.01). Vitamin C supplementation also significantly reduced SBP (p<0.001), DBP (p<0.01), MAP (p<0.01) and FVR (p<0.05) but significantly increased FBF (p<0.01). After vitamin C supplementation, warmth stimulation potentiated the reduction in SBP (p<0.001), DBP (p<0.01), FVR (p <0.01) and increase in FBF (p<0.01). In conclusion, warmth stimulation at 40 [corrected] degrees C or vitamin C supplementation caused a decrease in arterial blood pressure, forearm vascular resistance and increase in forearm blood flow in sickle cell anaemia subjects. Pretreatment with vitamin C enhanced the vasodilator effect of warmth.
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PMID:The effect of vitamin C and/or warmth on forearm blood flow and vascular resistance in sickle cell anaemia subjects. 1216 81

The effect of ascorbic acid supplementation (100 mg/day for 6 weeks) on blood pressure, packed cell volume, irreversibly sickled cells, per cent fetal hemoglobin, hemoglobin concentration, and erythrocyte osmotic fragility was assessed in children suffering from sickle cell anemia. Fifteen children whose ages ranged from 4 to 11 years (7.5 +/- 0.75 years) were studied. Ascorbic acid supplementation reduced systolic blood pressure by 10.9 +/- 3.4 mmHg (p < 0.01), diastolic blood pressure by 7.3 +/- 2.0 mmHg (p < 0.01) and mean arterial pressure by 9.4 +/- 2.6 mmHg (p < 0.01). It significantly increased packed cell volume (p < 0.001), hemoglobin concentration (p < 0.001) and per cent fetal hemoglobin (p < 0.001), but reduced per cent irreversibly sickled cells (p < 0.001). Ascorbic acid supplementation also abolished the long tail of the erythrocyte osmotic fragiligram and increased the resistance of the cells to lysis.
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PMID:Blood pressure, hematologic and erythrocyte fragility changes in children suffering from sickle cell anemia following ascorbic acid supplementation. 1252 Dec 81

Autonomic function following change in posture with or without vitamin C supplementation was studied in ten (10) sickle cell anemia (SCA) and twelve (12) non-sickle cell anemia (NSCA) subjects. Arterial blood pressure and electrocardiographic measurements were taken in the supine position on a couch 80cm high and immediately on assumption of the upright position. Vitamin C was then administered orally (300mg/day for 6 weeks). At the end of the period, blood pressure and ECG measurements were again made in the supine position and in response to change in posture. Change in posture significantly decreased QRS amplitude, QRS duration, PR interval, RR interval and MABP but increased HR and rate pressure product (RPP) in both groups of subjects. The HR and RPP responses were significantly higher in NSCA than in SCA subjects (p<0.001, respectively). Vitamin C caused greater reductions in QRS duration (p<0.01), PR duration p<0.001) in the NSCA subjects than in SCA subjects. It caused, however, greater reduction in RR duration (p<0.001) and MABP in SCA subjects than in NSCA subjects. It also caused significantly greater increases in HR and RPP (p<0.001, respectively) in the SCA subjects than in NSCA subjects. After vitamin C supplementation, change in posture decreased RR interval (p<0.001), QT interval (p<0.01) and MABP (p<0.05) but increased RPP (p<0.01) in NSCA subjects. In SCA subjects, there was a fall in RR interval (p<0.001) and MABP (p<0.01), but elevated RPP (p<0.001). Changes (Delta) in MABP, HR and RPP were similar between NSCA and SCA subjects. In conclusion, these findings indicate a blunted cardiovascular autonomic response to change in posture in sickle cell anemia subjects. Chronic, oral, low-dose vitamin C supplementation equilibrates this response with those of non-sickle cell anemia subjects.
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PMID:Cardiac and autonomic responses to change in posture or vitamin C supplementation in sickle cell anemia subjects. 1823 80