Cited by 168Metabolism and Genetic DisordersDiet and metabolism studiesLipid metabolism and disorders

The fasting test in paediatrics: application to the diagnosis of pathological hypo- and hyperketotic states

Bonnefont, J. P., Specola, N. B., Vassault, A., Lombes, A., Ogier, H., de Klerk, J. B. C., Munnich, A., Coude, M. +2 more·1990·European Journal of Pediatrics

Abstract

A 24-h fasting test was performed in 48 control children, in 9 hypoketotic patients with inherited defects of fatty acid oxidation and in 2 hyperketotic patients with inherited defects of ketolysis. The control group was then divided into three age groups on the basis of different adaptation to fasting. Concentrations of blood glucose, lactate, free fatty acids (FFA), 3-hydroxybutyrate, acetoacetate and carnitine were measured after 15 h, 20 h and 24 h of fasting. Significant negative correlations were found in the control group between plasma total ketone bodies (KB) and plasma glucose (P less than 0.001), plasma carnitine (P less than 0.005) and the amplitude of glycaemic response to glucagon at the end of the fast (P less than 0.01). FFA/KB ratio and the product of final fasting values of glucose and ketones were useful to differentiate between hypoketotic or hyperketotic patients and normal subjects. In children with a suspected or definite hyperketotic or hypoketotic disorder, a fasting test must only be performed in healthy patients, in good nutritional condition with non-diagnostic basal biochemical investigations. Carefully supervised fasting should be continued sufficiently to allow ketogenesis and ketolysis to become activated.

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Crossref · 10.1007/bf02072043

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Authors
Bonnefont, J. P., Specola, N. B., Vassault, A., Lombes, A., Ogier, H., de Klerk, J. B. C., Munnich, A., Coude, M., Paturneau-Jouas, M., & Saudubray, J. M.
Affiliations
Inserm (FR)
Venue
European Journal of Pediatrics
Published
1990-12
Volume
150
Issue
2
Pages
80-85
ISSN
  • 0340-6199 (ISSN-L)
  • 1432-1076
OpenAlex
W1998858187
Cited by
168
References
22
Citations by year
2026: 3 · 2025: 2 · 2024: 7 · 2023: 1 · 2022: 9 · 2021: 7 · 2020: 2 · 2019: 5 · 2018: 3 · 2017: 6 · 2016: 12 · 2015: 8 · 2014: 11 · 2013: 5 · 2012: 1
FWCI
2.61
Type
journal article
Language
en
Open access
closed
Locations
  • European Journal of Pediatrics (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Health Sciences › Medicine › Physiology › Diet and metabolism studies
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Lipid metabolism and disorders
Keywords
fasting test, inborn errors of fatty acid oxidation, ketone bodies, beta-hydroxybutyrate, acetoacetate, free fatty acids, plasma glucose, glucagon response, L-carnitine, children, ketogenesis, ketolysis
MeSH terms
Adolescent, Blood Glucose, Child, Child, Preschool, Fasting, Fatty Acids, Nonesterified, Female, Humans, Hydroxybutyrates, Hypoglycemia, Infant, Ketone Bodies, Lipid Metabolism, Inborn Errors, Male, 3-Hydroxybutyric Acid

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