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      l-Glutamine for sickle cell anemia: more questions than answers

      Blood
      American Society of Hematology

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          Abstract

          In 2017, the Food and Drug Administration approved 2 medications for sickle cell anemia (SCA): hydroxyurea for children and l-glutamine for children and adults. The approval of hydroxyurea was long overdue, but the approval of l-glutamine was a surprise to many. Any effective new treatment for SCA is a welcome advance, but there are few published studies of l-glutamine as a specific treatment for SCA. Accordingly, there are many unanswered questions about its efficacy, safety, and role in current therapy.

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          Most cited references4

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          Role of aminotransferases in glutamate metabolism of human erythrocytes

          Human erythrocytes require a continual supply of glutamate to support glutathione synthesis, but are unable to transport this amino acid across their cell membrane. Consequently, erythrocytes rely on de novo glutamate biosynthesis from α-ketoglutarate and glutamine to maintain intracellular levels of glutamate. Erythrocytic glutamate biosynthesis is catalyzed by three enzymes, alanine aminotransferase (ALT), aspartate aminotransferase (AST), and glutamine aminohydrolase (GA). Although the presence of these enzymes in RBCs has been well documented, the relative contributions of each pathway have not been established. Understanding the relative contributions of each biosynthetic pathway is critical for designing effective therapies for sickle cell disease, hemolytic anemia, pulmonary hypertension, and other glutathione-related disorders. In this study, we use multidimensional 1H–13C nuclear magnetic resonance (NMR) spectroscopy and multiple reaction mode mass spectrometry (MRM-MS) to measure the kinetics of de novo glutamate biosynthesis via AST, ALT, and GA in intact cells and RBC lysates. We show that up to 89% of the erythrocyte glutamate pool can be derived from ALT and that ALT-derived glutamate is subsequently used for glutathione synthesis.
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            Oral glutamine supplementation decreases resting energy expenditure in children and adolescents with sickle cell anemia.

            To determine the effects of orally administered glutamine on the resting energy expenditure (REE) and nutritional status of children and adolescents with sickle cell anemia. Twenty-seven children and adolescents (13 boys, 14 girls), 5.2 to 17.9 years old (median 11.0 years), received orally administered glutamine (600 mg/kg per day) for 24 weeks. Measures of REE and other nutritional parameters were compared at baseline and 24 weeks. After 24 weeks, the patients' median REE (kcal/d) decreased by 6% (P = 0.053) as indicated by the Harris Benedict equations and by 5% (P = 0.049) as indicated by the modified equations. Patients with less than 90% ideal body weight had even greater declines in REE after 24 weeks (P < 0.03 and 0.02, respectively). Improvements in nutrition parameters and in two amino acids in the plasma were observed. After 24 weeks of orally administered glutamine, children and adolescents with sickle cell anemia had a decrease in REE and improvement in nutritional parameters. Those who were underweight had a greater decrease in REE than those of normal body weight. Lowering REE may be an effective way to improve the growth of these children and adolescents.
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              A comparison in normal individuals and sickle cell patients of reduced glutathione precursors and their transport between plasma and red cells

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                Author and article information

                Journal
                Blood
                Blood
                American Society of Hematology
                0006-4971
                1528-0020
                August 16 2018
                August 16 2018
                August 16 2018
                June 12 2018
                : 132
                : 7
                : 689-693
                Article
                10.1182/blood-2018-03-834440
                29895661
                3701930d-d81d-4223-be05-6b25a6fa9b65
                © 2018
                History

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