RESTING ENERGY-EXPENDITURE IN CHILDREN WITH PHENYLKETONURIA

Reports have suggested that children with phenylketonuria (PKU) weigh more compared with reference data. We found lower body protein and bon e mineral density in children with PKU. These children may have a pred isposition to becoming overweight because of an alteration in body com position, which may lower resting energy expenditure (REE). REE was me asured in 30 (15 males, 15 females) children with PKU (aged 9.6 +/- 2. 9 y) and in 65 (23 males, 42 females) control children (aged 11.2 +/- 3.1 y). There was a comparable range in body fat within each group (co ntrol group: 11-34%; PKU group: 10-34%). The mean REE was similar betw een the male and female children with PKU (5300 +/- 757 and 4703 +/- 1 024 kJ/24 h, respectively) and the control subjects (5306 +/- 969 and 5164 +/- 701 kJ/24 h, respectively). The children with PKU had an REE similar to that predicted from control data (males 102.1 +/- 7.8% of p redicted and females 100.2 +/- 8.5% of predicted). This study found no evidence of a reduced REE or of increased weight in children with PKU .