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GLUCONEOGENESIS IN INFANCY AND CHILDHOOD II. Studies on the Glucose Production from Alanine in Three Cases of Persistent Neonatal Hypoglycaemia
Author(s) -
VIDNES JOSTEIN,
SØVIK ODDMUND
Publication year - 1976
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1976.tb04889.x
Subject(s) - gluconeogenesis , glucagon , medicine , hypoglycemia , endocrinology , alanine , labelling , phosphoenolpyruvate carboxykinase , insulin , pyruvate carboxylase , amino acid , metabolism , biochemistry , biology , enzyme
. Gluconeogenesis was studied in 3 cases of persistent neonatal hypoglycaemia. In 2 of the cases the labelling of blood glucose after i.v. injection of 14 C‐alanine was reduced. In these 2 patients only 1.3‐5% of the injected radioactivity was recovered in blood glucose, compared with 10% in normoglycaemic patients. The labelling of glucose from 14 C‐glycerol, as studied in one case, was not reduced. In this patient the labelling of blood glucose from 15 C‐alanine was improved after subtotal resection of the pancreas, and with increasing age. By the time of the isotope studies the plasma insulin was normal in all patients, and no deficiency of glucagon secretion could be 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 phosphoenolpyruvate carboxykinase or pyruvate carboxylase step.