Total and resting energy expenditure in children with sickle cell disease

Objective: To investigate energy balance in children with sickle cell disease (SCD) as the possible cause of impaired growth and undernutrition. Study design: Growth, resting (REE), total (TEE), and activity-related (AEE) energy expenditure and dietary intake were examined in 36 African American children with SCD (20 girls and 16 boys) and 30 control subjects (15 girls and 15 boys) of similar age (mean, 11.2 years) and ethnicity. TEE was measured by means of the doubly labeled water technique and REE by indirect calorimetry. AEE was calculated as TEE minus REE. Fat free mass (FFM) was calculated from skinfold prediction equations. Results: REE was significantly increased (131 kcal/d) in children with SCD (P = .001), after adjusting for sex and FFM. Children with SCD tended to have lower TEE (214 kcal/d) than control subjects, but there was no difference after adjusting for FFM and sex (P = .57). Children with SCD had significantly (P = .025) lower AEE (268 kcal/d) but only marginally (P = .08) lower AEE after adjusting for FFM and sex. Conclusions: The elevated REE and lower AEE, in combination with poor growth status, indicate chronic energy deficiency in children with SCD. Further studies are needed to determine the best approaches to the treatment and prevention of undernutrition in children with SCD. (J Pediatr 2000;136:73-9).