Enhanced sVCAM-1 levels in paediatric patients with sickle cell disease

Recently, several reports have shown endothelial activation to play an important role in the pathophysiology of sickle cell vaso-occlusion. We measured serum soluble VCAM-1 and soluble ICAM-I levels in steady state paediatric sickle cell patients. TNF, an endothelial activating cytokine, was also measured. sVCAM-I levels were increased as compared to age, sex and race-matched controls (p=0.002), whereas sICAM-I levels were not significantly enhanced. TNF levels were also elevated in paediatric sickle cell patients as compared to controls (p=0.01). These results show that endothelial activation is already manifest at a very young age in sickle cell patients, probably resulting from enhanced endothelial activating cytokines, such as TNF.(AU)

Folic acid status of paediatric patients with sickle cell disease

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)