Abstract
Resting energy expenditure (REE) is often used to estimate total energy needs. The Schofield equation based on weight and height has been reported to underestimate REE in female children with phenylketonuria (PKU). The objective of this observational, cross-sectional study was to evaluate the agreement of measured REE with predicted REE for female adolescents with PKU. A total of 36 females (aged 11.5 to 18.7 years) with PKU attending Emory University's Metabolic Camp (June 2002 to June 2008) underwent indirect calorimetry. Measured REE was compared to six predictive equations using paired Student's t tests, regression-based analysis, and assessment of clinical accuracy. The differences between measured and predicted REE were modeled against clinical parameters to determine whether a relationship existed. All six selected equations significantly under predicted measured REE (P<0.005). The Schofield equation based on weight had the greatest level of agreement, with the lowest mean prediction bias (144 kcal) and highest concordance correlation coefficient (0.626). However, the Schofield equation based on weight lacked clinical accuracy, predicting measured REE within +/-10% in only 14 of 36 participants. Clinical parameters were not associated with bias for any of the equations. Predictive equations underestimated measured REE in this group of female adolescents with PKU. Currently, there is no accurate and precise alternative for indirect calorimetry in this population.