Abstract
P.type I G and type I glyoogenosis (type I G) have identical clinical and biological features although the hepatic content of glucose 6 phosphatase is normal in P.type I G. Recently Hers et al showed that in this latter affection a shortening of the half life of glucose and an increase in the recycling of glucose were present (Biochem. Soc. Trans. 1975 P. 1051). Venous blood glucose, lactate, NEFA, pH, and plasma insulin levels were studied on several occasions before, 1, 2, 3, and 4 h after feeding in one case of type I G and one case of pseudo type I G, on basal conditions and after prolonged glucagon administration (1 to 2 mgIM every 8h. for several days to months). Both cases were severe forms with a fasting tolerance not exceeding 4 h. On basal condition the biological data were similar in both patients with low insulin levels (<7,5 to 40 μu/ml). Glucagon did not induce any significant changes in type I G and insulin levels were not modified ( <7,5 to 48 μu/ml). By contrast glucagon induced important increases in plasma insulin in P.type I G with post-feeding values > 200 μu/ml reaching on several occasions values > 1 000 μu/ml; parallel blood glucose levels varied from low to high normal (20 to 145 mg/100ml) This hyperinsulinism was still present (120 to 335 μu/ml) 2 months after discontinuation of 6 months glucagon injections. This finding suggest that in spite of normal basal insulin levels, an abnormal insulin/glucagon secretion ratio is present in P.typol G which may be involved in the abnormal glucose kinetics.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
David, L., Ruitton-Ugliengo, A. & Francois, R. 61: Contribution to the pathogenesis of pseudotype I glycogenosis (P. typeI G): prolonged hyperinsulinism induced by glucagon administration. Pediatr Res 10, 881 (1976). https://doi.org/10.1203/00006450-197610000-00058
Issue Date:
DOI: https://doi.org/10.1203/00006450-197610000-00058