ASSESSMENT OF ENERGY-EXPENDITURE IN METABOLIC DISORDERS

The assessment of energy expenditure is valuable for the management of children with various conditions such as obesity and failure to thriv e. Total daily energy expenditure (TDEE) includes resting energy expen diture (REE), energy expenditure during physical activity, dietary the rmogenesis and growth. TDEE can be assessed by using the double-labell ed water technique, but it has complex pitfalls and potential sources of errors and is impractical for everyday use. As REE is a substantial part of TDEE (65%-70%) and computerised indirect calorimeters have be come recently available, this non-invasive, relatively cheap and easy to use technique is valuable for the assessment of short-term changes in energy metabolism. This can be used to assess REE of children with inborn errors of metabolism, whilst well and during episodes of metabo lic decompensation and therefore to accurately determine energy intake .