080^120^160^2000 2400 0400 0800 hrs Meals

Diurnal metabolite profile Figure 8 Diurnal blood metabolite profile of a child with glycogen synthetase deficiency. Values taken from Aynsley-Green A, Williamson DH and Gitzelmann R (1977) Archives of Disease in Childhood 52: 573-579. (With permission from BMJ Publishing Group.)

ketogenesis is switched on. On consuming a meal the pattern is reversed until the blood glucose falls again. This case illustrates the importance of hepatic glycogen (and its mobilization) in the smooth transition of substrate supply from the fed to the fasted state. Treatment in this case was to recommend the consumption of more frequent high-carbohydrate snacks. It is of interest that this particular child suffered no ill effects from the daily exposure to high concentrations of ketone bodies, underlining their role as normal substrates for the brain when available.

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