GLUCONEOGENESIS IN INFANCY AND CHILDHOOD II. Studies on the Glucose Production from Alanine in Three Cases of Persistent Neonatal Hypoglycaemia

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Abstract
Gluconegenesis was studied in 3 cases of persistent neonatal hypoglycemia. In 2 of the cases the labeling of blood glucose after i.v. injection of 14C-alanine was reduced. In these 2 patients only 1.3-5% of the injected radioactivity was recovered in blood glucose, compared with 10% in normoglycemic patients. The labeling of blood glucose from 14C-glycerol, as studied in 1 case, was not reduced. In this patient the labeling of blood glucose from 15C-alanine was improved after subtotal resection of the pancrease and with increasing age. By the time of the isotope studies plasma insulin was normal in all patients, and no deficiency of glucagon secretion was detected after stimulation with an alanine load. A quantitative amino acid analysis of plasma revealed a moderate increase of some of the glucogenic amino acids. The results were interpreted as a deficiency of gluconeogenesis, probably at the phosphoenolypyruvate carboxykinase or pyruvate carboxylase step.