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.
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Abbreviations
- FFA:
-
free fatty acids
- KB:
-
ketone bodies
References
Bank WJ, Di Mauro S, Bonilla E, Capuzzi DM, Rowland LF (1975) A disorder of muscle lipid metabolism and myoglobinuria. Absence of carnitine palmitoyl transferase. N Engl J Med 292:443–449
Bougnères PF, Saudubray JM, Marsac C, Bernard O, Odièvre M, Girard J (1981) Fasting hopoglycemia resulting from hepatic carnitine palmitoyl transferase deficiency. J Pediatr 98:742–746
Chaussain JL, Georges P, Olive G, Job JC (1974) Glycemic response to 24-hour fast in normal children and children with ketotic hypoglycemia: II. Hormonal and metabolic changes. J Pediatr 85:776–781
Chaussain JL, Georges P, Calzada L, Job JC (1977) Glycemic response to 24-hour fast in normal children: III. Influence of age. J Pediatr 91:711–714
Demaugre F, Bonnefont JP, Mitchell G, Nguyen-Hoang N, Pelet A, Rimoldi M, DiDonato S, Saudubray JM (1988) Hepatic and muscular presentations of carnitine palmitoyl transferase deficiency: two distinct entities. Pediatr Res 24:308–311
Di Donato S, Castiglione A, Rimoldi M, Cornelio F, Vendemia F, Gardace C, Bertagnolio B (1981). Heterogeneity of carnitine palmitoyl transferase deficiency. J Neurol Sci 50:207–215
Duran M, Wadman SK (1987) Chemical diagnosis of inherited defects of fatty acid metabolism and ketogenesis. Enzyme 38:115–123
Fourre C, Rota M, Vassault A, Bonnefont JP, Nicolas A, Bailly M (1987) Miniaturisation et automatisation du dosage du glucose, du pyruvate, du lactate, de l'acétoacétate, du 3-hydroxybutyrate, des acides gras non estérifiés sanguins. Act Pharm Biol Clin 4:420–423
Frohlich J, Seccombe DW, Jahn P, Dodek P, Hynie I (1978) Effect of fasting on free and esterified carnitine levels in human serum and urine: correlation with serum levels of free fatty acids and 3-hydroxybutyrate. Metabolism 27:555–561
Gregersen N (1985) Acyl CoA dehydrogenation disorders. Scand J Clin Invest 45 [Suppl] 174:1–60
Haymond HW, Karl IE, Clarke WL, Pagliara AS, Santiago JV (1982) Differences in circulating gluconeogenic substrates during short-term fasting in men, women, and children. Metabolism 31:33–42
Kerr DS, Hansen IL, Levy MM (1983) Metabolic and hormonal responses of children and adolescents to fasting and 2-Deoxyglucose. Metabolism 32:951–959
Lamers KJB, Doesburg WH, Gabreëls FJM, Romson AC, Renier WO, Wevers RA, Lemmens WAIG (1985) The concentration of blood components related to fuel metabolism during prolonged fasting children. Clin Chim Acta 152:155–163
Mc Garry JD, Foster DW (1976) An improved and simplified radioisotope assay for determination of free and esterified carnitine. J Lipid Res 17:277–281
Middleton B, Day r, Lombes A, Saudubray JM (1987) Infantile ketoacidosis associated with decreased activity of succinyl-CoA: 3-ketoacid CoA transferase. J Inherited Metab Dis 10 [Suppl 2]:273–275
Morand P, Despert F, Carrier HN, Saudubray JM, Fardeau M, Romieux B, Fauchier C, Combe B (1979) Myopathie lipidique avec cardiomyopathie sévère par déficit généralisé en carnitine. Arch Mal Coeur 72:536–544
Saudubray JM, Marsac C, Limal JM, Dumurgier E, Charpentier C, Ogier H, Coudé FX (1981) Variation in plasma ketone bodies during a 24-hour fast in normal and hypoglycemic children: relationship to age. J Pediatr 98:904–908
Saudubray JM, Spécola N, Middleton B, Lombes A, Bonnefont JP, Jakobs C, Vassault A, Charpentier C, Day R (1987) Hyperketotic states due to inherited defects of ketolysis. Enzyme 38:80–90
Stanley CA (1987) New genetic defects in mitochondrial fatty acid oxidation and carnitine deficiency. Adv Pediatr 34:59–88
Stanley CA, Baker L (1976) Hyperinsulinism in infancy: diagnosis demonstration of abnormal response to fasting hypoglycemia. Pediatrics 57:702
Vianey-Liaud C, Divry P, Gregersen N, Mathieu M (1987) The inborn errors of mitochondrial fatty acid oxidation. J Inherited Metab Dis 10 [Suppl 1]:159–198
Wolfsdorf JI, Sadeghi-Nejad A, Senior B (1982) Fat-derived fuels during a 24-hour fast in children. Eur J Pediatr 138:141–144
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Bonnefont, J.P., Specola, N.B., Vassault, A. et al. The fasting test in paediatrics: Application to the diagnosis of pathological hypo- and hyperketotic states. Eur J Pediatr 150, 80–85 (1990). https://doi.org/10.1007/BF02072043
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DOI: https://doi.org/10.1007/BF02072043