Resting energy expenditure in disorders of propionate metabolism

François Feiltet, Olaf A F Bodamer, Marjorie A. Dixon, Silvia Sequeira, James V. Leonard

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

Objectives: During intercurrent illness children with methylmalonic acidemia were found to have increased resting energy expenditure (REE). We measured REE in children with disorders of propionate metabolism (methylmalonic and propionic acidemia) when they were well and compared the values with those predicted by the Schofield equation. Study design: Prospective study in tertiary care facility. REE was measured with open- circuit indirect calorimetry under standardized conditions. Predicted REE values were calculated with the Schofield equation. Fourteen subjects with propionic acidemia (n = 3) and methylmalonic acidemia (n = 11) were studied. Results: The median REE was 690 kcal/d (range 186 to 1687 kcal/d), which is significantly reduced, representing 80% ± 18% of that predicted by the Schofield height and weight equation (P < .01). REE was significantly lower in female compared with male patients for unknown reasons. There were no differences with age or neurologic state. REE was not further reduced in those with chronic renal failure. Conclusion: REE in patients with disorders of propionate metabolism is reduced when they are well.

Original languageEnglish
Pages (from-to)659-663
Number of pages5
JournalJournal of Pediatrics
Volume136
Issue number5
StatePublished - Jun 6 2000

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Propionates
Energy Metabolism
Propionic Acidemia
Indirect Calorimetry
Tertiary Healthcare
Nervous System
Chronic Kidney Failure
Prospective Studies
Weights and Measures

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Feiltet, F., Bodamer, O. A. F., Dixon, M. A., Sequeira, S., & Leonard, J. V. (2000). Resting energy expenditure in disorders of propionate metabolism. Journal of Pediatrics, 136(5), 659-663.

Resting energy expenditure in disorders of propionate metabolism. / Feiltet, François; Bodamer, Olaf A F; Dixon, Marjorie A.; Sequeira, Silvia; Leonard, James V.

In: Journal of Pediatrics, Vol. 136, No. 5, 06.06.2000, p. 659-663.

Research output: Contribution to journalArticle

Feiltet, F, Bodamer, OAF, Dixon, MA, Sequeira, S & Leonard, JV 2000, 'Resting energy expenditure in disorders of propionate metabolism', Journal of Pediatrics, vol. 136, no. 5, pp. 659-663.
Feiltet F, Bodamer OAF, Dixon MA, Sequeira S, Leonard JV. Resting energy expenditure in disorders of propionate metabolism. Journal of Pediatrics. 2000 Jun 6;136(5):659-663.
Feiltet, François ; Bodamer, Olaf A F ; Dixon, Marjorie A. ; Sequeira, Silvia ; Leonard, James V. / Resting energy expenditure in disorders of propionate metabolism. In: Journal of Pediatrics. 2000 ; Vol. 136, No. 5. pp. 659-663.
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