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Although the pathophysiology of sickle cell disease (SCD) is well known, the disorder has been resistant to most efforts to prevent or ameliorate painful crises. L-glutamine, a precursor of the antioxidant glutathione, was recently approved by the FDA for the management of sickle cell anemia. To evaluate the role of L-glutamine in SCD, investigators reviewed all published clinical trials reporting on the efficacy and safety of the drug.
Three small studies, encompassing a total of 45 patients, showed decreased cell adhesion to the endothelium and a decrease in energy expenditure by children but no change in total hemoglobin concentration. Two randomized, double-blind, placebo-controlled trials that administered L-glutamine in doses of 0.3 g/…