Safe and unsafe duration of fasting for children with MCAD deficiency.
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.


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.


CONCLUSIONS
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.
Derks
Terry G J
TG
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. t.g.j.derks@bkk.umcg.nl
van Spronsen
Francjan J
FJ
Rake
Jan Peter
JP
van der Hilst
Christian S
CS
Span
Mark M
MM
Smit
G Peter A
GP
D042964:Acyl-CoA Dehydrogenase / Q000172:deficiency*; D002675:Child, Preschool; D005215:Fasting; D006801:Humans; D007223:Infant; D008661:Metabolism, Inborn Errors / Q000628:therapy*; D009426:Netherlands; D013997:Time Factors
D042964:Acyl-CoA Dehydrogenase