Blood: Effects of arginine therapy on mitochondrial function during vascular closed pain in sickle cell disease in children

The mitochondrial function of sickle cell disease (SCD) changes, in part because of the low bioavailability of nitric oxide (NO)Arginine is the substrate that produces NO and becomes severely deficient in SCD patients associated with vascular ablation pain events (VOE)to determine whether arginine can improve mitochondria function, Morris et alrandomly divided 12 sCD patients hospitalized with VOE (age 13.6 to 3 years, men 67%, HbSS 75%) into 3 groups (4 people per group) and received different doses of arginine therapy:1) 100 mg/kg intravenous drip, 3 times/day;2) load dose 200 mg/kg, then 100 mg/kg, 3 times/day,3) load dose 200 mg/kg, and then continue to intake 300 mg/kg/day until discharged from the hospitaltested the blood platelet-rich mitochondrial activity, protein expression and protein carinatine in children who were enrolled in the group and discharged from hospital, respectivelyresults showed that the activity of the compound V in all CHILDREN with VOE was significantly reduced when they entered the group compared to the stable queueIn addition, the activity of the complex V of the three arginine dose therapy increased significantly at discharge from the hospital, and the increase was greatest when the load dose was usedAlthough the activity of platelet complex IV and citrate synthase in children with VOE was similar to that of stable state, the enzyme activity increased significantly in children with VOE after the arginine load dose therapyIn addition, arginine therapy also lowered the level of protein in all children, indicating a reduction in oxidative stress, this study shows that arginine therapy can enhance mitochondrial activity in Children with SCD/VOE and reduce oxidative stress