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Safe and unsafe duration of fasting for children with MCAD deficiency

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Abstract

Objective

To study the safe and unsafe duration of fasting in children with medium chain acyl-Coenzyme A dehydrogenase (MCAD) deficiency, the literature and the database on Dutch MCAD-deficient patients were searched for data on fasting studies in patients with MCAD deficiency.

Materials and methods

These data were extended with information on fasting studies performed on our patients with MCAD deficiency known in the Beatrix Children’s Hospital, UMC Groningen, The Netherlands. The data reflect considerable inter-individual variation and overlap between safe and unsafe duration of fasting.

Results

In six out of 35 fasting tests, symptoms were reported before hypoglycaemia was observed. Until 1 year of age, the median safe and unsafe duration of fasting was 12 hours (n=7, range 8–19 hours) and 18 hours (n=5, range 15–20 hours), respectively. After the first year of life, the median safe and unsafe duration of fasting was 18 hours (n=17, range 10–24 hours) and 20 hours (n=9, range 13–32 hours), respectively.

Conclusion

Therefore, to conclude, we recommend a maximum duration of fasting in children with MCAD deficiency of 8 hours between 6 months and 1 year of age, 10 hours in the second year of life and 12 hours thereafter. From this study, no conclusions can be drawn on the duration of fasting during situations of intercurrent illness, especially with fever.

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Abbreviations

MCAD:

Medium chain acyl-Coenzyme A dehydrogenase

mFAO:

Mitochondrial fatty acid oxidation

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Acknowledgements

From the database, fasting tests were included originating from the patients of M.P.J.M. Cuppen, MD, J.B.C. de Klerk, MD, T.J. de Koning, MD PhD, B.J.H.M. Poorthuis, PhD. We thank M. van Rijn for the fruitful discussions. Moreover, we thank the Data Management Bureau of the UMC Groningen for their technical support. ZonMw supports the work of T.G.J. Derks.

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Authors and Affiliations

  1. Section of Metabolic Diseases, Beatrix Children’s Hospital, University Medical Center Groningen, University of Groningen, P.O. Box 30 001, 9700 RB, Groningen, The Netherlands

    Terry G. J. Derks, Francjan J. van Spronsen & G. Peter A. Smit

  2. Department of Pediatrics, Martini Hospital, Groningen, The Netherlands

    Jan Peter Rake

  3. Office for Medical Technology Assessment, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

    Christian S. van der Hilst & Mark M. Span

Authors
  1. Terry G. J. Derks
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  2. Francjan J. van Spronsen
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  3. Jan Peter Rake
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  4. Christian S. van der Hilst
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  5. Mark M. Span
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  6. G. Peter A. Smit
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Correspondence to Terry G. J. Derks.

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Derks, T.G.J., van Spronsen, F.J., Rake, J.P. et al. Safe and unsafe duration of fasting for children with MCAD deficiency. Eur J Pediatr 166, 5–11 (2007). https://doi.org/10.1007/s00431-006-0186-0

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  • DOI: https://doi.org/10.1007/s00431-006-0186-0

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