European Journal of Pediatrics

, Volume 150, Issue 2, pp 80–85

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

  • J. P. Bonnefont
  • N. B. Specola
  • A. Vassault
  • A. Lombes
  • H. Ogier
  • J. B. C. de Klerk
  • A. Munnich
  • M. Coude
  • M. Paturneau-Jouas
  • J. -M. Saudubray
Review

DOI: 10.1007/BF02072043

Cite this article as:
Bonnefont, J.P., Specola, N.B., Vassault, A. et al. Eur J Pediatr (1990) 150: 80. doi:10.1007/BF02072043

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<0.001), plasma carnitine (P<0.005) and the amplitude of glycaemic response to glucagon at the end of the fast (P<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.

Key words

Fasting test Fatty acid oxidation Hypoketosis Hyperketosis 

Abbreviations

FFA

free fatty acids

KB

ketone bodies

Copyright information

© Springer-Verlag 1990

Authors and Affiliations

  • J. P. Bonnefont
    • 1
  • N. B. Specola
    • 1
  • A. Vassault
    • 2
  • A. Lombes
    • 1
  • H. Ogier
    • 1
  • J. B. C. de Klerk
    • 1
  • A. Munnich
    • 1
  • M. Coude
    • 1
  • M. Paturneau-Jouas
    • 3
  • J. -M. Saudubray
    • 1
  1. 1.Clinique de Génétique Médicale, Département de PédiatrieHôpital des Enfants-MaladesParis Cedex 15France
  2. 2.Laboratoire de Biochimie AInserm U-12 Hôpital des Enfants-MaladesFrance
  3. 3.Laboratoire de Neurochimie Inserm U-134Hôpital La SalpétrièreParisFrance