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.

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Severe vitamin A deficiency in rats is known to cause anaemia associated with growth retardation and impaired water retention. However, study of the effect of marginal vitamin A intake is of more interest because such intake may mirror the situation in humans in many developing countries. Therefore, in two experiments, the effect of marginal vitamin A deficiency on Fe status was investigated in male rats. After 28 d of feeding either low- or high-vitamin A diets (0 or 120 v. 1200 retinol equivalents/kg feed), body weight and feed intake were not influenced by the level of vitamin A in the diet. Liver weight was lowered by vitamin A deficiency. Water intake was not influenced in rats fed on a low-vitamin A diet. Plasma retinol concentrations were decreased in rats fed on diets low in vitamin A. Marginal vitamin A deficiency produced slightly lower blood haemoglobin concentrations; it did not systematically affect packed cell volume. The concentration of Fe in liver was significantly higher when diets low in vitamin A were fed, but hepatic Fe mass was not affected. Significantly lower Fe levels were observed in femurs of rats with vitamin A deficiency. The effects on liver and femur Fe concentrations were seen with diets adequate in Fe but not with diets deficient in Fe. The efficiency of apparent Fe absorption was significantly increased by low intakes of vitamin A, provided that the dietary Fe concentration was adequate. It is speculated that depressed uptake of Fe by bone marrow is the primary feature of altered Fe status in rats with marginal vitamin A deficiency.

The effect of a high concentration (1%, w/w) of ascorbic acid in a Cu-adequate (150 mumol/kg) purified diet was studied in rats. After 6 wk, ascorbic acid had significantly reduced Cu concentrations in muscle and bone. The estimated whole body content of Cu in rats fed ascorbic acid was reduced by 20%. Within 1 d after oral administration of 64Cu, the recovery of the dose in feces was increased in rats fed ascorbic acid, suggesting that the vitamin depresses intestinal absorption of Cu. After intraperitoneal (ip) administration of 64Cu, the rate of loss of the dose from the body was decreased in rats fed ascorbic acid. This study suggests that the ascorbic acid induces a decreased efficiency of intestinal Cu absorption, which in turn triggers mechanisms to preserve Cu in the body stores. This is supported by the observation that the feeding of a Cu-deficient diet (5 mumol/kg) had similar effects, although more pronounced.