Resting metabolic rate in homozygous sickle-cell disease - abstract

ABSTRACT

Erythropoietic hyperplasia and a hyperdynamic circulation might be expected to increase the resting metabolic rate (RMR) of patients with homozygous sickle-cell (SS) disease. Therefore we used the ventilated hood technique to determine the RMRs of the representative sample of adolescents with SS disease and controls with a normal haemoglobin (AA) genotype matched for age, sex, and pubertal stage. The RMR in 19 pairs was corrected for the lean body mass which was derived from the measurement of total body water. The RMR of SS subjects expressed as kcal/kg LBM was greater than that of AA controls (median, range 38.6, 30.7 - 45.4 and 33.2, 25.4 - 35.7, respectively, z = 3.1, p = 0.002). We postulate that the increased RMR in SS disease is not compensated for by a reduction in physical activity or increased energy intake and that this leads to a marginal nutritional state which may contribute to the abnormal growth of children with SS disease(AU)