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This is the scenario:
A child came to the clinic with signs of fasting hypoglycaemia, elevated blood free fatty acids & beta-hydroxybutyrate. Oral glucose feeding resulted in an excessive rise in blood glucose & lactate. A liver biopsy revealed very low glycogen synthase activity, but normal levels of other liver metabolic enzymes. Blood levels of insulin were within the normal range. Cornstarch feeding prevented the occurrence of hypoglycaemia.
Why would oral glucose feeding result in a higher than expected rise in blood glucose & lactate? Is it because just prior to oral glucose feeding, gluconeogenesis (GNG) is taking place while the oral glucose adds to the blood [glucose], and GNG continues for a little longer just after refeeding; since glycogen synthase activity is low, instead of stocking up on liver glycogen, the glucose synthesised by liver gets released into the bloodstream. Hence, a "double dose" (GNG from liver + oral glucose) and the higher than expected rise in blood glucose? How to explain the increased lactate then? Cornstarch is used instead of glucose because it's a slow-release carbs?
A child came to the clinic with signs of fasting hypoglycaemia, elevated blood free fatty acids & beta-hydroxybutyrate. Oral glucose feeding resulted in an excessive rise in blood glucose & lactate. A liver biopsy revealed very low glycogen synthase activity, but normal levels of other liver metabolic enzymes. Blood levels of insulin were within the normal range. Cornstarch feeding prevented the occurrence of hypoglycaemia.
Why would oral glucose feeding result in a higher than expected rise in blood glucose & lactate? Is it because just prior to oral glucose feeding, gluconeogenesis (GNG) is taking place while the oral glucose adds to the blood [glucose], and GNG continues for a little longer just after refeeding; since glycogen synthase activity is low, instead of stocking up on liver glycogen, the glucose synthesised by liver gets released into the bloodstream. Hence, a "double dose" (GNG from liver + oral glucose) and the higher than expected rise in blood glucose? How to explain the increased lactate then? Cornstarch is used instead of glucose because it's a slow-release carbs?