Protein and energy metabolism in prepubertal children with sickle cell anemia

Pediatric Research
E K SalmanD Darmaun


We hypothesized that, in children with homozygous sickle cell anemia (HbSS), the shortened life-span of erythrocytes places an increased demand on protein stores, accelerates whole body protein turnover, and consequently, energy expenditure, as well as the rate of utilization of glutamine, a major fuel for reticulocytes. Eight (11.2 +/- 0.4 y old) children with HbSS who were free of infection of vaso-occlusive disease, and seven (11.3 +/- 0.4 y old) healthy black children were therefore studied in the postabsorptive state. Each received a continuous 4-h infusion of L-[1-(13)C]leucine to determine the rate of leucine oxidation, leucine rate of appearance, and nonoxidative leucine disposal, indicators of whole body protein breakdown and synthesis, respectively. Infusion of L-[2-(15)N]glutamine was used to assess rates of glutamine utilization. Resting energy expenditure and cardiac output were measured using indirect calorimetry and echocardiography, respectively. Compared with control subjects, HbSS children had a 58 and 65% higher leucine rate of appearance and nonxidative leucine disposal, respectively (both p < 0.001), 47% higher rates of whole body glutamine utilization (p < 0.01), 19% higher resting energy expenditure (p < ...Continue Reading


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Related Concepts

Anemia, Sickle Cell
Basal Metabolic Rate
Calorimetry, Indirect
Energy Metabolism
Intravenous Infusion Procedures
Nested Case-Control Studies

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