Insulin and glucagon secretion in the ketotic (idiopathic glucagon unresponsive) hypoglycemia of childhood
Five children affected by ketotic hypoglycemia (KH) and 13 control children were given the Colle and U Istrom ketogenic diet (KD). Intramuscular glucagon injection (100 μg/kg, max 1 mg) was carried out before and at the end of the KD. Arginine infusion (0.5g/kg/30 min) was administered to the KH children and to 20 control children. The KH children also underwent an oral glucose tolerance test (1.75 g/kg) (OGTT) and were compared with 12 controls. Blood glucose, plasma insulin and glucagon were assayed throughout the 48 h of KD, or until hypoglycemia developed, and during both the arginineand OGTT tests, Blood glucose was also determined during the two im glucagon tests. All 5 KH children developed hypoglycemia during the KD; during the first im glucagon test their blood glucose levels were lower than those of the controls, but showed a sluggish increase during the second test. Three control children showed hypoglycemic levels during the KD, but their blood glucose values increased during the second im glucagon test as did those of the other control children. An evident decrease in plasma insulin, and an increase in plasma glucagon levels were observed during the KD; there were no significant differences between the KH children and the controls. During the arginine test the KH children showed mean plasma insulin levels significantly lower at 30 min (p<0.05) and mean plasma glucagon levels significantly higher at 60 min (p<0.05), at 90 min (p<0.001 ) and 120 min (p<0.05) when compared with the controls. Also during the OGTT lower plasma insulin levels were observed in the KH children at 120 min (p<0.05). These data suggest that endocrine pancreas activity of KH children is different from that of controls and probably adapts itself to a reduced glucose output ability of the liver. The findings obtained in the KH children by the glucagon tests are in accordance with a poor hepatic glycogen storage.
Key-wordsInsulin glucagon ketotic hypoglycemia
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