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Departments of 1 Surgery and
2 Biochemistry,
Basal rates of whole body protein, glucose, and
lipid metabolism and resting energy expenditure (REE) were measured in
eight African-American sickle cell disease (SCD) patients and in six African-American controls. Catheters were placed
1) in an antecubital vein for stable
isotope infusion and 2) in a heated
hand vein for arterialized venous blood. Breath and blood were
collected during the last 30 min of the 2.5-h study, and REE was
measured by indirect calorimetry. REE [128 ± 5 vs. 111 ± 1 kJ · kg fat-free mass
(FFM)
1 · day
1;
P < 0.05 vs. controls] was
15% greater in the SCD patients. Whole body protein breakdown (5.0 ± 0.3 vs. 3.8 ± 0.2 mg · kg FFM
1 · min
1;
P < 0.05 vs. controls) and protein
synthesis (4.4 ± 0.3 vs. 3.2 ± 0.2 mg · kg
FFM
1 · min
1;P < 0.05 vs. controls) were 32 and 38% greater, respectively, in the
SCD patients, but whole body amino acid oxidation was similar (0.58 ± 0.03 vs. 0.66 ± 0.03 mg · kg
FFM
1 · min
1).
Measures of whole body glucose and lipid metabolism were not significantly different between the groups. The additional energy required for the greater rates of whole body protein breakdown and
synthesis caused by SCD contributes significantly to the observed increase in REE, suggesting that dietary energy and protein
requirements are enhanced in SCD patients.
sickle cell anemia; protein metabolism; carbohydrate metabolism; lipolysis; amino acids
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