RESUMO
We investigated whether paediatric patients with sickle cell disease (9ñ4 years; 27 HbSS; 19 HbSC) have different folic acid status compared with age-, sex-and race-matched HbAA controls (n=20), and whether their folic acid status can be improved by folic acid supplementation. The patients were supplemented with vitamins B6 and B12 during one week and with folic acid during the next week. Circulating folic acid, homocysteine, vitamin B6 and vitamin B12 levels were measured at baseline (patients and controls), after 1 and 2 weeks (patients). The patients had similar folic acid, vitamin B6 and vitamin B12, but higher homocysteine levels, compared with HbAA controls (12.7ñ4.5 vs 10.9ñ3.5 mmol/l;p=0.04). Vitamin B6 and B12 supplementation did not change their homocysteine levels, but folic acid supplementation caused a 52 percent reduction (to 5.7ñ1.6). We conclude that patients with sickle cell disease have adequate vitamin B6 and B12 status, but suboptimal folic acid status. They may benefit from folic acid supplementation to reduce their high risk for endothelial damage.(AU)
Assuntos
Criança , Humanos , Anemia Falciforme/fisiopatologia , Deficiência de Ácido Fólico , Deficiência de Vitamina B 12 , Deficiência de Vitamina B 6 , Deficiência de RiboflavinaRESUMO
A high-performance liquid chromatography (HPLC) method for the screening of haemoglobins in cord blood was evaluated and the gene frequencies of the structural haemoglobin variants HbS and HbC and the synthesis variants O- and á+ -thalassaemia were studied in babies born on the Caribbean island of Curacao, the Netherlands Antilles. In 3 months, 67.2 percent of all (748) newborns were screened and 122 (24.3 percent) had an abnormal haemoglobin pattern, of which 53 (43.4 percent) had a haemoglobinopathy (HbS or HbC), 64(52.2 percent) O-thalassaemia (Hb Barts > 0.5 percent, corresponding with heterozygous or homozygous O-thalassaemia-2) and 5 (4.1 percent) a haemoglobinopathy plus O-thalassaemia. None of the newborns with heterozygous HbS and HbC had concomitant á+-thalassaemia. The population genotype frequency of heterozygous O-thalassaemia -2 remain undetected. The data are in excellent agreement with comparable published results. The HPLC method proved pre-eminently suitable for the screening of cord blood samples. (AU)