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Tolerance to fast: rational and practical evaluation in children with hypoketonaemia

  • SSIEM Symposium 2008
  • Published:
Journal of Inherited Metabolic Disease

Summary

Prolonged fasting in children with disorder of fat oxidation or ketone body synthesis can lead not only to hypoglycaemia but also to the accumulation of toxic metabolites. The length of time such patients can be safely fasted is important information for caregivers. Most children with MCAD deficiency when well can tolerate ‘normal’ periods without food, but in more severe disorders such as LCHAD deficiency even these may be associated with acute or chronic damage. Guidelines have been published for safe fasting periods in MCAD but not in other conditions. In the absence of such recommendations, a rational approach must be based on an understanding of the normal physiology of fasting in children of different ages and the pathophysiology associated with the child’s particular disorder. Intercurrent infections pose a particular risk and may significantly reduce fasting tolerance.

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Abbreviations

LCHAD:

long-chain hydroxyacyl-CoA dehydrogenase

MCAD:

medium-chain acyl-CoA dehydrogenase

MCT:

medium-chain triglycerides

VLCAD:

very long-chain acyl-CoA dehydrogenase

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Authors and Affiliations

  1. Willink Biochemical Genetics Unit, Royal Manchester Children’s Hospital, Hospital Road, Manchester, M27 4HA, UK

    J. H. Walter

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  1. J. H. Walter
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Correspondence to J. H. Walter.

Additional information

Communicating editor: Ertan Mayatepek

Competing interests: None declared

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Walter, J.H. Tolerance to fast: rational and practical evaluation in children with hypoketonaemia. J Inherit Metab Dis 32, 214–217 (2009). https://doi.org/10.1007/s10545-009-1087-y

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  • DOI: https://doi.org/10.1007/s10545-009-1087-y

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