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Resting energy expenditure in disorders of propionate metabolism

F Feillet1, O A Bodamer, M A Dixon

  • 1Biochemistry, Endocrinology and Metabolism Unit, Institute of Child Health, London, United Kingdom.

The Journal of Pediatrics
|May 10, 2000
PubMed

Insights

Resting energy expenditure (REE) is reduced in children with propionate metabolism disorders when they are well, contrary to findings during illness. This study measured REE in well children with methylmalonic acidemia and propionic acidemia.

Area of Science:

  • Biochemistry
  • Pediatric Metabolism
  • Nutritional Science

Background:

  • Children with methylmalonic acidemia exhibit increased resting energy expenditure (REE) during illness.
  • Propionate metabolism disorders include methylmalonic acidemia and propionic acidemia.
  • Understanding baseline REE is crucial for managing these metabolic conditions.

Purpose of the Study:

  • To measure REE in well children with methylmalonic and propionic acidemia.
  • To compare measured REE with values predicted by the Schofield equation.
  • To investigate factors influencing REE in these patients.

Main Methods:

  • Prospective study utilizing open-circuit indirect calorimetry.
  • REE was measured under standardized conditions in a tertiary care facility.
  • Schofield equation used for calculating predicted REE.

Main Results:

  • Median REE was 690 kcal/d, representing 80% of predicted values (P <.01).
  • REE was significantly lower in female patients.
  • No significant differences in REE were observed based on age, neurologic state, or chronic renal failure.

Conclusions:

  • Resting energy expenditure is reduced in children with propionate metabolism disorders when they are metabolically stable.
  • This finding contrasts with elevated REE observed during intercurrent illnesses.
  • Further research may explore the underlying mechanisms for reduced REE in these patients.
Abstract

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