Impaired ketogenesis in fructose-1,6-bisphosphatase deficiency: a pitfall in the investigation of hypoglycaemia

Author:

Morris A. A. M.,Deshpande S.,Ward-Platt M. P.,Whitfield A. E.,Aynsley-Green A.,Leonard J. V.,Pourfarzam M.,Bartlett K.

Publisher

Wiley

Subject

Genetics (clinical),Genetics

Reference8 articles.

1. Bartlett K, Aynsley-Green A, Leonard JV, Turnbull DM (1991) Inherited disorders of mitochondrial ?-oxidation. In Schaub J, Van Hoof, Vis HC, eds.Inborn Errors of Metabolism, Nestl� Nutrition Workshop Series, vol. 24. New York: Raven Press, 19?41.

2. Harrison J, Hodson AW, Skillen AW, Stappenbeck R, Agius L, Alberti KGMM (1988) Blood glucose, lactate, pyruvate, glycerol, 3-hydroxybutyrate and acetoacetate measurements in man using a centrifugal analyser with a fluorimetric attachment.J Clin Chem Clin Biochem 26: 141?146.

3. Hawdon JM, Ward-Platt MP, Aynsley-Green A (1992) Patterns of metabolic adaptation for preterm and term infants in the first neonatal week.Arch Dis Child 67, 357?365.

4. Jackson S, Bartlett K, Land J et al (1991) Long chain 3-hydroxyacyl-CoA deficiency.Pediatr Res 29: 406?411.

5. McGarry JD, Foster DW (1980) Regulation of hepatic fatty acid oxidation and ketone body production.Annu Rev Biochem 49: 395?420.

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