Accuracy of equations for estimating resting energy expenditure in children and adolescents living with phenylketonuria

• Published in: Journal of human nutrition and dietetics 2023-10, Vol.36 (5), p.1661-1674
• Main Authors: Mezzomo, Thais R, Dias, Marcia Regina Messaggi G, Vilela, Regina M, Pereira, Rosana Marques
• Format: Article
• Language: English
• Subjects: Adolescents; Blood; Body composition; Body height; Body mass; Body mass index; Body size; Calorimetry; Children; Energy expenditure; Health care facilities; Heat measurement; Phenylalanine; Phenylketonuria; Teenagers; Weight
• ISSN: 0952-3871; 1365-277X
• DOI: 10.1111/jhn.13200

Measuring resting energy expenditure (REE) in individuals living with phenylketonuria (PKU) using indirect calorimetry (IC) is unusual in healthcare facilities because it requires specific protocols and expensive equipment. Considering that determining REE is crucial for devising nutritional strategies for the management of PKU, the aim of this study was to identify the predictive equations that provide the best estimates of REE in children and adolescents living with PKU and to propose a predictive equation for determining REE in this population. An REE concordance study was conducted with children and adolescents living with PKU. Anthropometric and body composition assessments using bioimpedance and REE assessment using IC were performed. The results were compared to 29 predictive equations. Fifty-four children and adolescents were evaluated. The REE obtained using IC differed from all estimated REE, except Henry's equation for male children (p = 0.058). Only this equation showed good agreement (0.900) with IC. Eight variables were associated with the REE obtained using IC with emphasis on fat-free mass (kg) (r = 0.786), weight (r = 0.775), height (r = 0.759) and blood phenylalanine (r = 0.503). With these variables, three REE equations were suggested, with R  = 0.660, 0.635 and 0.618, respectively, and the third equation, which involves weight and height, showed adequate sample size for a statistical power of 0.942. Most equations, not specific for individuals living with PKU, overestimate the REE of this population. We propose a predictive equation for assessing REE for children and adolescents living with PKU to be used in settings where IC is not available.