vitamin-b-12 and Anemia--Sickle-Cell

Topics: Adrenal Insufficiency; Amino Acid Metabolism, Inborn Errors; Amino Acids, Sulfur; Ammonia; Anemia, Sickle Cell; Anencephaly; Carbohydrate Metabolism, Inborn Errors; Fabry Disease; Female; Fetal Diseases; Fetus; Gangliosides; Gaucher Disease; Humans; Karyotyping; Lipid Metabolism, Inborn Errors; Lipidoses; Lysine; Metabolism, Inborn Errors; Mucopolysaccharidoses; Polyploidy; Pregnancy; Propionates; Sphingolipidoses; Turner Syndrome; Vitamin B 12

Topics: Anemia; Anemia, Hypochromic; Anemia, Macrocytic; Anemia, Sickle Cell; Child; Child, Preschool; Feces; Female; Fever; Folic Acid; Hemoglobinopathies; Hookworm Infections; Humans; Infant; Leishmaniasis, Visceral; Malaria; Male; Pregnancy; Protozoan Infections; Schistosomiasis; Vitamin B 12

Topics: Altitude; Anemia; Anemia, Macrocytic; Anemia, Sickle Cell; Ascorbic Acid; Celiac Disease; Climate; Deficiency Diseases; Female; Folic Acid; Hematology; Humans; Infant; Metabolism; Nutrition Disorders; Parasitic Diseases; Pharmaceutical Preparations; Poisons; Polycythemia; Pregnancy; Pregnancy Complications; Pregnancy Complications, Hematologic; Radiation Effects; Seasons; Sprue, Tropical; Vitamin B 12

We investigated whether pediatric patients with sickle cell disease (SCD) (9 +/- 4 years; 27 homozygous SCD [HbSS]; 19 sickle-C disease [HbSC]) have different folate status compared with age-, sex-, and race-matched normal hemoglobin (HbAA) controls (n = 20), and whether their folate status can be improved by folate supplementation. The patients were supplemented with vitamins B6 and B12 during one week and with folate during the following week. Circulating folate, homocysteine, vitamin B6 and vitamin B12 levels were measured at baseline (patients and controls), after one week and after two weeks (patients). The patients had similar folate, vitamin B6, and vitamin B12, but higher homocysteine levels compared with HbAA controls (12.7 +/- 4.5 vs. 10.9 +/- 3.5 micromol/l; P = 0.04). Vitamin B6 and B12 supplementation did not change their homocysteine levels, but folate supplementation caused a 53% reduction (to 5.7 +/- 1.6). We conclude that patients with SCD have adequate vitamin B6 and B12 status, but suboptimal folate status, leading to elevated plasma homocysteine levels. They may therefore benefit from folate supplementation to reduce their high risk for endothelial damage.

Topics: Adolescent; Anemia, Sickle Cell; Child; Child, Preschool; Dietary Supplements; Female; Folic Acid; Hemoglobin SC Disease; Homocysteine; Humans; Infant; Male; Pyridoxine; Vitamin B 12

Topics: Anemia, Sickle Cell; Antimalarials; Child; Child, Preschool; Clinical Trials as Topic; Female; Folic Acid; Humans; Male; Steroids; Sulfonamides; Vitamin B 12; Vitamin D

The pathophysiology of sickle cell disease (SCD) includes vasculopathy as well as anaemia. Elevated plasma homocysteine is a risk factor for vascular disease and may be associated with increased risk of vascular complications in SCD patients. In the present study, microvascular characteristics were assessed in the bulbar conjunctiva of 18 paediatric and 18 adult SCD patients, using the non-invasive technique of computer-assisted intravital microscopy. A vasculopathy severity index (SI) was computed to quantify the degree of microvasculopathy in each patient. Plasma homocysteine and several of its determinants [serum folate and vitamin B12, plasma pyridoxal-5'-phosphate (vitamin B6 status) and creatinine (kidney function)] were measured. Age was strongly correlated with microvasculopathy in the SCD patients, with the SI increasing about 0·1 unit per one-year increase in age (P < 0·001). After adjusting for age, gender, B-vitamin status and creatinine, homocysteine concentration was directly correlated with severity index (P < 0·05). Age and homocysteine concentration were independent predictors of microvasculopathy in SCD patients. It remains to be determined whether lowering homocysteine concentrations using appropriate B-vitamin supplements (folate and vitamins B12 and B6) - particularly if started early in life - could ameliorate microvasculopathy and its associated complications in SCD patients.

Topics: Adolescent; Adult; Anemia, Sickle Cell; Child; Child, Preschool; Creatine; Folic Acid; Homocysteine; Humans; Intravital Microscopy; Microcirculation; Middle Aged; Pyridoxal Phosphate; Severity of Illness Index; Thrombotic Microangiopathies; Vitamin B 12

Some studies comparing serum cobalamin in individuals with and without sickle cell disease (SCD) have suggested a higher prevalence of cobalamin deficiency in SCD but others have not. Our aim was to prospectively compare cobalamin status in African-Americans with and without SCD.. We analyzed blood samples from 86 subjects in two groups: SCD (n = 29) and non-SCD (n = 57). Serum cobalamin, folate, homocysteine, methylmalonic acid (MMA), anti-intrinsic factor antibody, Helicobacter pylori antibody, and gastrin were measured and compared.. The median cobalamin was 235 pM in the SCD group vs. 292 pM in the non-SCD group (P-value = 0.014). No significant differences in MMA or homocysteine were seen. Using the criteria of a low cobalamin and an elevated MMA or an elevated MMA alone, cobalamin deficiency was suggested in 4 (13.8%) in the SCD group and 6 (10.5%) in the non-SCD group. Two of these SCD patients and four of these control subjects had chronic renal disease, which may lead to elevated MMA in the absence of cobalamin deficiency. The remaining four met criteria for cobalamin deficiency, 2 (6.9%) in the SCD group and 2 (3.5%) in the non-SCD group (P = 0.6).. A lower cobalamin was observed in SCD patients without a higher prevalence of cobalamin deficiency. The inclusion of haptocorrin and holotranscobalamin measurement in future studies may provide a better assessment of cobalamin status in this patient group.

Topics: Adult; Anemia, Sickle Cell; Black or African American; Cross-Sectional Studies; District of Columbia; Homocysteine; Hospitals, Teaching; Humans; Male; Methylmalonic Acid; Middle Aged; Nutritional Status; Prevalence; Prospective Studies; Renal Insufficiency, Chronic; Vitamin B 12; Vitamin B 12 Deficiency; Young Adult

Topics: Adult; Anemia, Pernicious; Anemia, Sickle Cell; Autoantibodies; Depression; Female; Folic Acid; Gastrins; Hemoglobin SC Disease; Humans; Hydroxyurea; Hyperhomocysteinemia; Intrinsic Factor; Iron Overload; Male; Methylmalonic Acid; Pneumonia; Transcobalamins; Transfusion Reaction; Treatment Refusal; Vitamin B 12; Vitamin B 12 Deficiency; Young Adult

About 7% of the adult population has subclinical cobalamin (B12) deficiency. Subjects with sickle cell disease (SCD) may be at higher risk of cobalamin deficiency because of increased demand, inadequate supply, coexisting folate deficiency or malabsorption. We compared the clinical and laboratory characteristics of low serum cobalamin levels in patients with SCD with those patients without this hemoglobinopathy (non-SCD). Between 1993 and 2003, 105 SCD patients and 112 non-SCD patients who had serum cobalamin measurements were identified at our institution. The mean cobalamin level in SCD patients was significantly lower (496 +/- 352 pg/ml) than that in patients without SCD (869 +/- 660 pg/ml, p<0.0001). The frequency of low cobalamin levels, defined by a serum cobalamin level of <200 pg/ml, was 18.1% (19/105) and 9.8% (11/112) in SCD and non-SCD patients, respectively (chi2=3.11, nonsignificant). The mean age of the low-cobalamin SCD and non-SCD patients was 28.1 and 62.9, respectively, and their male:female ratios were 11:8 in SCD patients and 2:9 in non-SCD patients. None of the SCD patients had neurological manifestations, but nine of the 11 non-SCD low-cobalamin level patients did. The proportion of SCD patients with unexplained low cobalamin levels (13/19) was higher than that in non-SCD patients (4/11, chi2=2.92, nonsignificant) Our data suggest that cobalamin levels are lower in SCD patients than in subjects without SCD, and low-cobalamin SCD patients are younger and more likely to be males.

Topics: Adult; Aged; Anemia, Sickle Cell; Black or African American; Case-Control Studies; Female; Humans; Male; Middle Aged; Vitamin B 12

Homocysteine has associations with both vitamin insufficiency and vascular complications, and its status is therefore of interest in sickle cell disease (SCD). However, information is limited, especially in adults. We studied plasma total homocysteine (tHcy) and three of its major modifiers, cobalamin, folate, and creatinine, in 90 adult patients with SCD and 76 control subjects. The patients had higher tHcy levels than did controls (P = 0.03) and had elevated tHcy more often (20% vs. 3%, P = 0.0005). None of the hyperhomocysteinemic patients had low cobalamin or folate levels; on the contrary, patients with SCD had high folate levels more often than control subjects (32% vs. 7%; P < 0.0001). Although serum creatinine values were lower in SCD patients than in control subjects (P = 0.03), high levels also tended to occur more often (8% vs. 1%; P = 0.054). Most importantly, creatinine levels correlated significantly with tHcy (P < 0.0001) and logistic regression analyses showed creatinine to be the only significant predictor of high tHcy levels in SCD (P = 0.01). Our results show that hyperhomocysteinemia affects 20% of adults with SCD despite routine folate supplementation and is independent of folate and cobalamin status. Creatinine was the major identifiable influence on tHcy, but renal insufficiency explained only 4 of the 18 elevated tHcy levels. Longitudinal studies will be needed to determine whether the frequent hyperhomocysteinemia of SCD influences the vascular complications in SCD. If reducing tHcy becomes advisable, then interventions other than folate therapy will be needed.

Topics: Adult; Aged; Anemia, Sickle Cell; Creatinine; Female; Folic Acid; Hemoglobin SC Disease; Humans; Hyperhomocysteinemia; Male; Middle Aged; Regression Analysis; Reproducibility of Results; Vitamin B 12

Topics: Adult; Anemia, Pernicious; Anemia, Sickle Cell; Cognition Disorders; Female; Folic Acid; Humans; Mental Disorders; Neuropsychological Tests; Vitamin B 12

Topics: Anemia, Sickle Cell; Female; Folic Acid; Humans; Vitamin B 12

Using homocysteine as a functional marker, we determined optimal folic acid, vitamin B(12), and vitamin B(6) dosages in 21 pediatric sickle cell disease (SCD) patients (11 HbSS, 10 HbSC; 7-16 years). Daily supplements of folic acid (400, 700, or 1,000 microg), vitamin B(12) (1, 3, or 5 U.S. 1989 RDA), and vitamin B(6) (1 or 3 U.S. 1989 RDA) were gradually increased in an 82-week dose-escalation study. Blood was taken at 9 occasions for measurements of erythrocyte (RBC) and serum folate, plasma vitamin B(12), whole-blood vitamin B(6), and plasma homocysteine. Augmentation of folic acid from 700 to 1,000 microg and vitamin B(12) from 3 to 5 RDA did not further decrease homocysteine. Percentages of patients exhibiting significant individual homocysteine decreases amounted to 43% (folic acid from 0 to 400 microg, vitamins B(12) and B(6) from 0 to 1 RDA), 14% (folic acid from 400 to 700 microg), 24% (vitamin B(12) from 1 to 3 RDA), and 18% (vitamin B(6) from 1 to 3 RDA ). The lowest plasma homocysteine at 82 weeks was 5.9 +/- 2.2 micromol/L. Patients with HbSS had higher RBC folate than HbSC. The entire group exhibited an inverse relation between RBC folate and hemoglobin. We conclude that RBC folate is less valuable for folate status assessment in SCD patients. Optimal dosages are as follows: 700 microg folic acid (3.5-7 U.S. 1989 RDA), 3 U.S. 1989 RDA vitamin B(12) (4.2-6.0 microg), and 3 U.S. 1989 RDA vitamin B(6) (4.2-6.0 mg). A practical daily combination is 1 mg folic acid (4.3-8.5 U.S. 1998 RDA when taken with meals), 6 microg vitamin B(12) (2.5-5 U.S. 1998 RDA), and 6 mg vitamin B(6) (4.6-10 U.S. 1998 RDA). This combination may by simple and relatively inexpensive means reduce these patients' inherently high risk of endothelial damage.

Topics: Adolescent; Anemia, Sickle Cell; Child; Dietary Supplements; Erythrocytes; Female; Folic Acid; Hemoglobin SC Disease; Homocysteine; Humans; Male; Nutrition Policy; Vitamin B 12; Vitamin B 6

The aim of this study was to estimate the levels of vitamin B12 in patients with severe sickle cell disease compared to normal controls. Complete blood count, iron studies and vitamin B12 levels were obtained in 85 consecutive patients with severe sickle cell disease (56 males, 29 females, aged 14-49 years) and 100 healthy blood donors (67 males, 33 females, aged 17-60 years) as a normal control group.. Thirty-seven of the 85 patients (43.5%) had serum vitamin B12 levels below normal values (mean 84.3 +/- 28.7, range 7-145 pmol L-1) without macrocytosis or hypersegmented neutrophils. The mean level of vitamin B12 in the remaining 48 patients (56.5%) was normal (mean 233.3 +/- 73.9, range 152-435 pmol L-1) which is below the mean of normal control levels (mean 327.7 +/- 168.7, range 178-897 pmol L-1). Patients with low B12 achieved a significant symptomatic improvement when treated with vitamin B12, 1 mg intramuscularly weekly for 12 weeks when compared with patients with normal B12 levels.. We conclude that many patients with severe sickle cell disease may suffer from unrecognized vitamin B12 deficiency.

Topics: Adolescent; Adult; Anemia, Sickle Cell; Erythrocytes, Abnormal; Female; Humans; Male; Middle Aged; Severity of Illness Index; Vitamin B 12

The unsaturated vitamin B12 binding capacity (UBBC) of serum, the three transcobalamins (TC I, TC II, TC III) and serum cobalamin have been studied in adult sickle cell patients with Hb SS. An increase in the level of the binding capacities of TC I and TC III resulting in an increase in UBBC was observed in these patients. There was no difference in the level of the binding capacity of TC II, while there was a significant reduction in the level of serum cobalamin. These changes in the pattern of these binding proteins are discussed in relation to the changes of these binding proteins in paediatric sickle cell patients with Hb SS. The results indicate that the pattern of these binding proteins changes with age among sickle cell patients resulting in an increased level of the binding capacities of TC I and TC III. These increases lend support to the view that these two similar binding proteins are involved in the defence mechanism of the body.

Topics: Adolescent; Adult; Anemia, Sickle Cell; Child; Female; Humans; Male; Transcobalamins; Vitamin B 12

Although the issue of folate supplementation in sickle cell anemia remains controversial, routine supplementation has become common. The major drawback to indiscriminate folate therapy is the potential of masking findings of vitamin B12 (cobalamin) deficiency. This has been dismissed as a problem in sickle cell anemia because of the generally young age of the patients. However, because young blacks, especially women, are at higher risk for developing pernicious anemia than whites, sickle cell anemia and pernicious anemia can be expected to coexist occasionally. In this article we describe such a patient and recommend that routine folate supplementation should not be given in sickle cell anemia before determining the vitamin B12 status.

Topics: Adult; Anemia, Pernicious; Anemia, Sickle Cell; Female; Folic Acid; Humans; Vitamin B 12

The serum cobalamin, serum folate, erythrocyte folate concentrations and total leucocyte and neutrophil counts have been studied in sickle cell children (with HbSS) during the steady state. There was a significant reduction in serum cobalamin concentration with significant increases in both the total leucocyte count and neutrophil differential. A relationship was also observed between the serum cobalamin and erythrocyte folate. The lowest erythrocyte folate levels were observed in patients with the lowest serum cobalamin concentration. These abnormalities are discussed in relation to the possible role of cobalamin in the synthesis of erythrocyte folate polyglutamate and possible immunological role of cobalamin among these children. These results lend support to the view that daily cobalamin supplementation along with folate (which is being given) may prove beneficial to the sickle cell patients as it may lower the frequency and severity of infections.

Topics: Anemia, Sickle Cell; Child; Child, Preschool; Erythrocytes; Female; Humans; Infant; Leukocyte Count; Male; Neutrophils; Pteroylpolyglutamic Acids; Vitamin B 12

The etiologic explanation of an anemia is important for the most effective therapy of that anemia. This may also define an underlying disease process. The etiology of the anemia can be achieved by appropriate use of the history, physical examination, and laboratory data. This information may be used to clarify the anemia, define a diagnosis, and direct appropriate therapy.

Topics: Anemia; Anemia, Hypochromic; Anemia, Macrocytic; Anemia, Sickle Cell; Blood Cell Count; Cytodiagnosis; Erythrocyte Indices; Erythrocyte Transfusion; Female; Ferrous Compounds; Hematologic Tests; Humans; Male; Physical Examination; Reticulocytes; Thalassemia; Vitamin B 12

The unsaturated vitamin B12 binding capacity (UBBC) of serum, the three transcobalamins (TC I, TC II, TC III), and the total leucocyte and neutrophil counts have been studied in paediatric patients with sickle cell disease (SCD). Increase in the level of the binding capacities of TC I and TC II with concomitant increase of UBBC was observed in these children who also had increased total white blood and neutrophil counts. There was a significant reduction in the level of endogenous B12. These abnormalities are discussed in relation to the deficiency of the splenic reticuloendothelial function, immunologic defect, hepatic degenerative changes and aplastic crisis observed in SCD. These results lend support to the view that transcobalamins are involved in the defence mechanism of the body. The significant reduction in serum cobalamin in SCD suggests a higher demand on this vitamin for metabolic functions, especially for the sparing of folate.

Topics: Anemia, Sickle Cell; Blood Proteins; Child; Child, Preschool; Female; Humans; Infant; Leukocyte Count; Male; Neutrophils; Nigeria; Receptors, Cell Surface; Sex Factors; Transcobalamins; Vitamin B 12

The present study has examined the relationship and significance of hypocholesterolaemia to anaemia and to the red cell. The level of plasma cholesterol is closely related to haematocrit levels both initially and throughout the course of the anaemias associated with hypocholesterolaemia. The association is maintained, regardless of the cause of the haematocrit change, i.e. transfusion or response to treatment. Hypocholesterolaemia does not affect the red cell, i.e. its survival, cholesterol concentration or osmotic fragility and occurs in a diverse group of anaemias. As such, it is not causally related to anaemia. The changes in plasma cholesterol are most likely related to its 'redistribution' or to plasma 'dilution', with contributory factors occurring. The presence of anaemia, even to a modest degree, would be important to consider when comparing cholesterol levels between groups of individuals.

Topics: Anemia; Anemia, Aplastic; Anemia, Megaloblastic; Anemia, Sickle Cell; Blood Transfusion; Cholesterol; Erythrocyte Aging; Erythrocytes; Folic Acid; Hematocrit; Humans; Liver Cirrhosis; Osmotic Fragility; Spherocytosis, Hereditary; Splenectomy; Vitamin B 12

Topics: Anemia, Macrocytic; Anemia, Sickle Cell; Child; FIGLU Test; Folic Acid; Folic Acid Deficiency; Growth Disorders; Hemoglobinometry; Histidine; Humans; Intestinal Absorption; Male; Recurrence; Vitamin B 12

Topics: Anemia, Aplastic; Anemia, Hemolytic; Anemia, Hypochromic; Anemia, Sickle Cell; Anemia, Sideroblastic; Blood Cell Count; Blood Platelets; Child; Chronic Disease; Erythrocyte Count; Erythrocytes; FIGLU Test; Folic Acid; Folic Acid Deficiency; Humans; Leukocyte Count; Vitamin B 12

Topics: Adolescent; Adult; Aged; Anemia; Anemia, Macrocytic; Anemia, Pernicious; Anemia, Sickle Cell; Celiac Disease; Cell Nucleus; Cheek; Epithelium; Fatty Liver; Female; Humans; Malabsorption Syndromes; Male; Microscopy; Middle Aged; Mouth Mucosa; Pregnancy; Pregnancy Complications, Hematologic; Thalassemia; Vitamin B 12

Topics: Adolescent; Anemia, Sickle Cell; Bone Marrow; FIGLU Test; Folic Acid; Folic Acid Deficiency; Humans; Iron; L-Lactate Dehydrogenase; Male; Reticulocytes; Vitamin B 12

Topics: Adult; Aged; Anemia, Macrocytic; Anemia, Pernicious; Anemia, Sickle Cell; Blood Proteins; Carbon Isotopes; Female; Folic Acid; Humans; In Vitro Techniques; Male; Middle Aged; Reticulocytes; Ribosomes; Schilling Test; Vitamin B 12

Topics: Adolescent; Adult; Anemia, Aplastic; Anemia, Sickle Cell; Bilirubin; Electrophoresis; Female; FIGLU Test; Folic Acid Deficiency; Hemoglobinometry; Histidine; Humans; Leukocytosis; Paper; Starch; Thrombocytopenia; Vitamin B 12

Topics: Anemia; Anemia, Sickle Cell; Avitaminosis; Erythropoiesis; Folic Acid; Folic Acid Deficiency; Humans; Vitamin B 12; Vitamin B Deficiency