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Resting energy expenditure in children with phenylketonuria
J R Allen1, J C McCauley, D L Waters
1James Fairfax Institute of Paediatric Clinical Nutrition, Royal Alexandra Hospital for Children, Camperdown, NSW, Australia.
Insights
Children with phenylketonuria (PKU) do not have lower resting energy expenditure (REE) or increased weight. This study found comparable REE in children with PKU versus controls, challenging prior assumptions about their body composition and metabolism.
Area of Science:
- Metabolic disorders
- Pediatric nutrition
- Body composition analysis
Background:
- Phenylketonuria (PKU) is a genetic disorder affecting protein metabolism.
- Previous reports suggested children with PKU may be overweight.
- Altered body composition in PKU might influence resting energy expenditure (REE).
Purpose of the Study:
- To investigate resting energy expenditure (REE) in children with phenylketonuria (PKU).
- To compare body composition and REE between children with PKU and healthy controls.
- To assess potential links between body composition, REE, and weight in pediatric PKU.
Main Methods:
- Measured REE in 30 children with PKU and 65 control children.
- Analyzed body composition, including body protein and bone mineral density.
- Compared REE values against predicted values based on control data.
Main Results:
- Children with PKU exhibited lower body protein and bone mineral density compared to controls.
- No significant difference in mean REE was observed between PKU and control groups.
- REE in children with PKU was similar to predicted values from control data.
- Body fat percentage ranges were comparable between the PKU and control groups.
Conclusions:
- This study found no evidence of reduced REE in children with PKU.
- Findings do not support the hypothesis that children with PKU have increased weight due to lower REE.
- Lower body protein and bone density in PKU warrant further investigation regarding long-term health implications.
Abstract:
Reports have suggested that children with phenylketonuria (PKU) weigh more compared with reference data. We found lower body protein and bone mineral density in children with PKU. These children may have a predisposition becoming overweight because of an alteration in body composition, which may lower resting energy expenditure (REE). REE was measured 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 (control group: 11-34%; PKU group: 10-34%). The mean REE was similar between the male and female children with PKU (5300 +/- 757 and 4703 +/- 1024 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 predicted 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.