Whole Body Protein Turnover and Resting Metabolic Rate in Homozygous Sickle Cell Disease

1. Whole body protein turnover and resting metabolic rate were measured in six adults with homozygous sickle cell disease (genotype HbSS) and in six normal adults (genotype HbAA) of similar age.

2. Turnover was measured with prime/intermittent oral doses of [¹⁵N]glycine over 18 h and resting energy expenditure was measured by indirect calorimetry.

3. In HbSS, nitrogen flux (0.9 ± 0.08 g day⁻¹ kg⁻¹), protein synthesis (6.0 ± 0.5 g day⁻¹ kg⁻¹) and protein degradation (5.6 ± 0.5 g day⁻¹ kg⁻¹) were significantly increased compared with HbAA nitrogen (flux 0.5 ± 0.02 g day⁻¹ kg⁻¹, protein synthesis 3.2 ± 0.2 g day⁻¹ kg⁻¹ and protein degradation 2.8 ± 0.2 g day⁻¹ kg⁻¹).

4. Resting energy expenditure was significantly higher in HbSS compared with HbAA when expressed per unit of body weight (115 ± 3 and 94 ± 4 kJ day⁻¹ kg⁻¹, respectively) or weight ^0.75 (317 ± 6 and 269 ± 8 kJ day⁻¹ kg^−0.75, respectively).

5. The increase in protein turnover and energy expenditure suggest that patients with HbSS exist in a hypermetabolic state that requires greater dietary energy compared with HbAA.