Mitochondrial energy production correlates with the age-related BMI

Saskia B. Wortmann, Heidi Zweers Van Essen, Richard J.T. Rodenburg, Lambert P. Van Den Heuvel, Maaike C. De Vries, Ellen Rasmussen-Conrad, Jan A.M. Smeitink, Eva Morava-Kozicz

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Besides characteristic neurologic and musculoskeletal symptoms, children with mitochondrial dysfunction often present with feeding problems and failure to thrive. Substrate depletion for the respiratory chain has an effect on energy expenditure. Secondary mitochondrial dysfunction has been reported in severe chronic malnutrition. We evaluated the nutritional state, the growth parameters, and the metabolic condition in 172 children undergoing muscle biopsy for a suspected disorder of the oxidative phosphorylation system (OXPHOS). We performed dietary evaluation and initiated nutritional intervention when needed before the biopsy. Mitochondrial dysfunction was confirmed by detection of enzyme-complex deficiencies and/or by mutations in 83 children, in 14 patients no biochemical abnormalities were found. In the whole study group, and in the subgroup with enzyme-complex deficiency and/or mutation, a significant correlation was found between the mitochondrial production of adenosine triphosphate (ATP) and the age-related body mass index (BMI). Nutritional state and growth should be considered by interpreting the results of ATP-production in fresh muscle biopsy. Because of a positive correlation between the age-appropriate BMI and the ATP-production, we strongly advise optimizing the nutritional state preceding the muscle biopsy in children with a suspected OXPHOS-disorder. Dietary intervention remains although challenging because of frequent gastrointestinal problems and eating disorders.

Original languageEnglish (US)
Pages (from-to)103-108
Number of pages6
JournalPediatric Research
Volume65
Issue number1
DOIs
StatePublished - Jan 1 2009
Externally publishedYes

Fingerprint

Body Mass Index
Biopsy
Adenosine Triphosphate
Oxidative Phosphorylation
Muscles
Failure to Thrive
Mutation
Enzymes
Growth
Electron Transport
Neurologic Manifestations
Malnutrition
Energy Metabolism

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Wortmann, S. B., Van Essen, H. Z., Rodenburg, R. J. T., Van Den Heuvel, L. P., De Vries, M. C., Rasmussen-Conrad, E., ... Morava-Kozicz, E. (2009). Mitochondrial energy production correlates with the age-related BMI. Pediatric Research, 65(1), 103-108. https://doi.org/10.1203/PDR.0b013e31818d1c8a

Mitochondrial energy production correlates with the age-related BMI. / Wortmann, Saskia B.; Van Essen, Heidi Zweers; Rodenburg, Richard J.T.; Van Den Heuvel, Lambert P.; De Vries, Maaike C.; Rasmussen-Conrad, Ellen; Smeitink, Jan A.M.; Morava-Kozicz, Eva.

In: Pediatric Research, Vol. 65, No. 1, 01.01.2009, p. 103-108.

Research output: Contribution to journalArticle

Wortmann, SB, Van Essen, HZ, Rodenburg, RJT, Van Den Heuvel, LP, De Vries, MC, Rasmussen-Conrad, E, Smeitink, JAM & Morava-Kozicz, E 2009, 'Mitochondrial energy production correlates with the age-related BMI', Pediatric Research, vol. 65, no. 1, pp. 103-108. https://doi.org/10.1203/PDR.0b013e31818d1c8a
Wortmann SB, Van Essen HZ, Rodenburg RJT, Van Den Heuvel LP, De Vries MC, Rasmussen-Conrad E et al. Mitochondrial energy production correlates with the age-related BMI. Pediatric Research. 2009 Jan 1;65(1):103-108. https://doi.org/10.1203/PDR.0b013e31818d1c8a
Wortmann, Saskia B. ; Van Essen, Heidi Zweers ; Rodenburg, Richard J.T. ; Van Den Heuvel, Lambert P. ; De Vries, Maaike C. ; Rasmussen-Conrad, Ellen ; Smeitink, Jan A.M. ; Morava-Kozicz, Eva. / Mitochondrial energy production correlates with the age-related BMI. In: Pediatric Research. 2009 ; Vol. 65, No. 1. pp. 103-108.
@article{e2e044291bbf4acd8c4dedfab56591f0,
title = "Mitochondrial energy production correlates with the age-related BMI",
abstract = "Besides characteristic neurologic and musculoskeletal symptoms, children with mitochondrial dysfunction often present with feeding problems and failure to thrive. Substrate depletion for the respiratory chain has an effect on energy expenditure. Secondary mitochondrial dysfunction has been reported in severe chronic malnutrition. We evaluated the nutritional state, the growth parameters, and the metabolic condition in 172 children undergoing muscle biopsy for a suspected disorder of the oxidative phosphorylation system (OXPHOS). We performed dietary evaluation and initiated nutritional intervention when needed before the biopsy. Mitochondrial dysfunction was confirmed by detection of enzyme-complex deficiencies and/or by mutations in 83 children, in 14 patients no biochemical abnormalities were found. In the whole study group, and in the subgroup with enzyme-complex deficiency and/or mutation, a significant correlation was found between the mitochondrial production of adenosine triphosphate (ATP) and the age-related body mass index (BMI). Nutritional state and growth should be considered by interpreting the results of ATP-production in fresh muscle biopsy. Because of a positive correlation between the age-appropriate BMI and the ATP-production, we strongly advise optimizing the nutritional state preceding the muscle biopsy in children with a suspected OXPHOS-disorder. Dietary intervention remains although challenging because of frequent gastrointestinal problems and eating disorders.",
author = "Wortmann, {Saskia B.} and {Van Essen}, {Heidi Zweers} and Rodenburg, {Richard J.T.} and {Van Den Heuvel}, {Lambert P.} and {De Vries}, {Maaike C.} and Ellen Rasmussen-Conrad and Smeitink, {Jan A.M.} and Eva Morava-Kozicz",
year = "2009",
month = "1",
day = "1",
doi = "10.1203/PDR.0b013e31818d1c8a",
language = "English (US)",
volume = "65",
pages = "103--108",
journal = "Pediatric Research",
issn = "0031-3998",
publisher = "Lippincott Williams and Wilkins",
number = "1",

}

TY - JOUR

T1 - Mitochondrial energy production correlates with the age-related BMI

AU - Wortmann, Saskia B.

AU - Van Essen, Heidi Zweers

AU - Rodenburg, Richard J.T.

AU - Van Den Heuvel, Lambert P.

AU - De Vries, Maaike C.

AU - Rasmussen-Conrad, Ellen

AU - Smeitink, Jan A.M.

AU - Morava-Kozicz, Eva

PY - 2009/1/1

Y1 - 2009/1/1

N2 - Besides characteristic neurologic and musculoskeletal symptoms, children with mitochondrial dysfunction often present with feeding problems and failure to thrive. Substrate depletion for the respiratory chain has an effect on energy expenditure. Secondary mitochondrial dysfunction has been reported in severe chronic malnutrition. We evaluated the nutritional state, the growth parameters, and the metabolic condition in 172 children undergoing muscle biopsy for a suspected disorder of the oxidative phosphorylation system (OXPHOS). We performed dietary evaluation and initiated nutritional intervention when needed before the biopsy. Mitochondrial dysfunction was confirmed by detection of enzyme-complex deficiencies and/or by mutations in 83 children, in 14 patients no biochemical abnormalities were found. In the whole study group, and in the subgroup with enzyme-complex deficiency and/or mutation, a significant correlation was found between the mitochondrial production of adenosine triphosphate (ATP) and the age-related body mass index (BMI). Nutritional state and growth should be considered by interpreting the results of ATP-production in fresh muscle biopsy. Because of a positive correlation between the age-appropriate BMI and the ATP-production, we strongly advise optimizing the nutritional state preceding the muscle biopsy in children with a suspected OXPHOS-disorder. Dietary intervention remains although challenging because of frequent gastrointestinal problems and eating disorders.

AB - Besides characteristic neurologic and musculoskeletal symptoms, children with mitochondrial dysfunction often present with feeding problems and failure to thrive. Substrate depletion for the respiratory chain has an effect on energy expenditure. Secondary mitochondrial dysfunction has been reported in severe chronic malnutrition. We evaluated the nutritional state, the growth parameters, and the metabolic condition in 172 children undergoing muscle biopsy for a suspected disorder of the oxidative phosphorylation system (OXPHOS). We performed dietary evaluation and initiated nutritional intervention when needed before the biopsy. Mitochondrial dysfunction was confirmed by detection of enzyme-complex deficiencies and/or by mutations in 83 children, in 14 patients no biochemical abnormalities were found. In the whole study group, and in the subgroup with enzyme-complex deficiency and/or mutation, a significant correlation was found between the mitochondrial production of adenosine triphosphate (ATP) and the age-related body mass index (BMI). Nutritional state and growth should be considered by interpreting the results of ATP-production in fresh muscle biopsy. Because of a positive correlation between the age-appropriate BMI and the ATP-production, we strongly advise optimizing the nutritional state preceding the muscle biopsy in children with a suspected OXPHOS-disorder. Dietary intervention remains although challenging because of frequent gastrointestinal problems and eating disorders.

UR - http://www.scopus.com/inward/record.url?scp=58149376277&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=58149376277&partnerID=8YFLogxK

U2 - 10.1203/PDR.0b013e31818d1c8a

DO - 10.1203/PDR.0b013e31818d1c8a

M3 - Article

C2 - 19096353

AN - SCOPUS:58149376277

VL - 65

SP - 103

EP - 108

JO - Pediatric Research

JF - Pediatric Research

SN - 0031-3998

IS - 1

ER -