Oral citrulline as arginine precursor may be beneficial in sickle cell disease: early phase two results.

摘要: L-Arginine may be a conditionally essential amino acid in children and adolescents with sickle cell disease, particularly as required substrate the arginine-nitric oxide pathway for endogenous nitrovasodilation vasoprotection. Vasoprotection by arginine is mediated partly nitric oxide-induced inhibition of endothelial damage adhesion activation leukocytes. Activated leukocytes trigger many complications, including vasoocclusive events intimal hyperplasias. High blood leukocyte counts during steady states absence infection are significant laboratory risk factors adverse complications. L-Citrulline precursor was given orally twice daily doses approximately 0.1 g/kg pilot Phase II clinical trial four homozygous disease subjects one cell-hemoglobin C patient (ages 10-18). There soon resulted dramatic improvements symptoms well-being, raised plasma levels, reductions high total segmented neutrophil toward or to within normal limits. Continued L-citrulline supplementation compliant continued lessen symptomatology, maintain concentrations greater than control nearly counts. Side effects toxicity from citrulline were not experienced. Oral portend very useful palliative therapy disease. Placebo-controlled, long-term trials now indicated.