Metabolism
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Two boys with profound fasting hypoglycaemia and no ketone bodies
Objectives
By the end of this exercise you should be able to:
Describe the pathway of ketone body synthesis.
Explain why it is beneficial to reduce acetoacetate to beta-hydroxybutyrate before export from the liver.
Explain why a child who lacks one or other of the enzymes of ketone body synthesis becomes profoundly hypoglycaemia after a relatively prolonged fast, or when s/he has an infection.
Explain why infusion of beta-hydroxybutyrate will maintain the plasma concentration of glucose after an overnight fast in a child who lacks one or other of the enzymes of ketone body synthesis.