Abstract
The intravenous administration of L-alanine can be used to test gluconeogenesis. It is important that the glycogen stores of the liver are depleted before the test is performed. Only then can an increase of blood glucose be ascribed to gluconeogenesis from L-alanine, glycogenolysis being of minor importance. Therefore, we extended the fasting period previous to the test until the blood glucose concentration was 3 mmol/1 approximately. We performed the test on 3 children with ketotic hypoglycemia, 4 with hepatic glycogenosis and 1 with a deficiency of hepatic fructose-1,6-diphosphatase. The former two groups responded with a marked increase of blood glucose, whereas blood lactate hardly varied. The results pointed to unimpaired gluconeogenesis. In the patient with a fructose-1, 6-diphosphatase deficiency blood glucose did not increase, the elevated fasting lactate increased further and L-alanine elimination was delayed. These data pointed to impaired gluconeogenesis.
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